40965358 Underground Clinical Vignettes Pa Tho Physiology III

BLACKWELL'S

I

VI[KAS BWSHAN, MD University of California, San F r a n c i ~ o , Class nf 1991 Srrim Editor, D ia~~ los r i c Radiologist

VTSHAL PALL, MBBS Governtnent Medical College, Chandigal-11, India, CIacs of 1996 Series Editor, 1:. of Texas. I;alveston, Recident in Intrrnal Meclicine & Preventive Medicine

TAO LE, MD Universitv or Clalifornia, San Francisco. C l a ~ s of 1996

A s m ZAMAN, MBBS Kctv Drlhi, India

Blackwell Science

CONTRIBUTORS Robert Nason Universitv of fcxaq Medical Branch. C1x .~ of 2003

Kristen Lem Mygdal, MD L'niwrsity of Kansas School of Medicine, Re~iden t in Radir~logy

Wpih Shah, PA-C tlt l ive~ritv of Texas M ~ d i c i l l Rmnrh, C;alv~ston, Clxs or 2M0

Fadi Abu Shahin, MD Univemitv or Da~narcu~, Syria, Clasq nf 1 YO9

Hoang Nguyen, MD. MBA northwest en^ Llniversity, Class of 21301

Sonal Shah, MD Ross Univenitv, Class of 2000

B 2002 hy Blackrvell Science, lnc.

Editorial Ofices: Commerce P l ~ c e , 950 Main Street. Malden,

Masswhusetw O"I4X. USA Omey Mead, Oxford CTX:! OEL, Engla~ld 25Jnhn Street, I-ondan WCLN "S. F.ngland 25 Ainslle Place, Ed~nburgh EH3 GAJ, .%orland 54 Universiv Street. Carlton. Victoria 3055.

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Interiol. deqign by Sl~awn Gir~beqer Typeset by TechBoub I'rint~rI and b o i ~ n d hv Cap~tal Citv Prrr;a

Blackwell's Underground Clinical Wpettes:

Pathophysiology m, 3e ISBN 0-63Y-1145554

Printed 1 1 the United Srates of America 020304055432 1

The Blackwell Sripnce logo is a tradr mark ol' Blackw~ll Science Ltd., registered at the Unitrd Kingdom Tmde M a r k Regs-

Library of Congress Cadoh-in-Publicarion Data Bhuuhan, Vikas. Btaclnvell's underground clinical vignettes. Palhophpiolop / author, Vi'ikau Rhushan. - 3rd rd .

p. : cm. - (Undergrof~nd clinical vignettes)

k r . 4. of: PatIlophvsiologv / T ' i h EBl~tishan. 2nd ed. clYY9.15RN (kf3204551-3 (pbk.) 1 . Phwinloffl. Patl~ologicat - Case studies.

2. Phyacians - Licenser - United Status - Examinatinn~ - Study guides.

[DNLU 1 Clinical Mtdicinr - Case Repurt. 2. Clinical Medicine - ProM~ms and Excrciseq. WR 18.2 B573hb 20021 1. Ti&: Cndergzaund clinical vipettes. Fathnph?.;iology. 11. Fathoph!~inln~. 111. T~tle. IV, Serieq.

RBI 1'3 .I3459 2002 FIG.O7'C17ttdc21

All rights rcsrrved. Nu part ol thic hook may 'he

rcprotl~~ccd In any rorm nr hv any electmnir 01. rnrchanicaf means, including information storage and rcrricwl svstemy. rulthout permiasirln in uriting Frnm the I>uhlichrr, Pxcept Iw a rerieuwr rvho mav guoie brief passages in a review.

Notice 'l'hr ;IUI llors nT rhis t,.rliirnr h:wr takrn c;lrt. ~hak the ir~f?,~.rnatinn contained herrin is accurate ant1 compti-

hlr with thcl .;ta~ltl;~rrlc genrnillv itcccplctl at tlir Iinw or pi1,licadon. Newrtliclcsc, i l is r l i l l i c ~ ~ l t tn rnsure that :111 I ~ P infornlatiun given is entirclv acri~~.atc 1,r all tircumsrance~. The pr~hlisl~er and authors da not

,ymranLcc the C O ~ I C I ~ I S ol' this hook ant1 dicclaim anv liafiilitv, Ions. o r dar~lagc incrirrcd as a conneqrrence, directlv or intlirerlly, onf the use arid :lpplic;ttion ot'nny r)f the rnnrrnts nf thiq vol~irne.

Acknowledgments x Preface t o the 3rd Edition xiii How to Use This Book xvi Abbreviations xvii

Amyotrophic Lateral Sclerosis (ALS) !@! k" X "

Aphasia-Broca's 1

I c> C1 Spinal Cord Injury Cauda Equina Syndrome J

Cerebral Aneurysm T' Ctaniop haryngioma ic 'I

Creutzfeldt-Jakob Disease !< CVA, Capsular Infarct I A

CVA, Hypertensive ' 9

Epidural Bematoma "" ' 9 Friedreich's Ataxia 1

I 7

Glioblastorna Multiforme Guillain-Barre Syndrome I

x y , He miballismus Huntington's Chorea Internuclear Ophthalmoplegia 7

* '1 Kliiver-Bu y Syndrome Medulloblastoma I

M6niGreS Disease I . '

Meningioma Metastatic Brain Tumor " I

Migraine r >:

Multiple Sclerosis Myasthenia Gravis Myotonic Dystrophy I

S?+ , Neuroblastorna Normal Pressure Hydrocephalus OLigodendrogboma ! ^"

Peripheral Neuropathy-Diabetic 1 A " > ' P

Pseudobulbar Palsy ) -

Subarachnoid Hemosrhage 1' *

Subdural Hematoma + ,

Syringomyelia Temporal Arteritis (Giant Cell Arteritis) von Hippel-Lindau Disease * " ' 4

Bre-ast-Cystosarcoma Phyllodes I Breast-Fat Necrosis p., ! :I

Orthopedics

Breast-Fibrocystie Disease Breast -1nf ammatory Carcinoma Breast-Intraductal Papilloma Breast-Lobular Carcinoma Breast-Paget's Disease Breast Carcinoma Breast Fibroadenoma Cervical Carcinoma (In Situ) Choriacarcinoma Desmoid Tumor Dysmenorrhea Endometrial Carcinoma Endometriosis Hydatidiform Mole Menopause Ovarian Cancer Ovarian Cyst-Follicular Ovarian Teratorna Polycystic Ovarian Syndrome Prima y Amenorrhea-Taarnefs Syndrome Uterine fibroids Uterine Leiomyosarcoma Vulvar Carcinoma Vulvar Le&oplakia Vulvar Malignant Melanoma Ectopic Pregnancy Postpartum Hemorrhage Postpartum Thrombophlebitis Sheehan's Syndrome Toxemia of Pregnancy-Pxeeclampsia Ewing's Sarcoma Hand-Dupuytren's Contracture Hip-Avascular Necrosis of Femaral Head Hip-SSipped Capital Femoral Epiphysis Knee-Anterior Cruciate ligament Injury Osteaarthri'cis Osteogenic Sarcoma Paget's Disease of Bone Septic Arthritis-Staphylococcal Spine-CervTcal Spondylosis Alcoholism Ankylosing Spondylitis Decompression Sickness Dermatomyositis

Rheurnotology Toxico logy

Juvenile Rheumatoid Arthritis Mixed Connective Tissue Disorder Osteopetrosis Osteoporosis Polyarteritis Nodasa PolymyaIgia Hheumatica Polymyositis Progressive Systemic Sclerosis (Scleroderma) Pseudogout Raynaud's Disease Reiter's Syndrome Rheumatoid Arthritis Sj o gren's Syndrome Systemic Lupus Erythematosus (SLE) Wegener's Granulomatosis Carbon Dioxide Narcosis

Thrcxighour the pi+od~~ction of t h i ~ hook. we Iiave had the sup- pcll-1 ol rnan!tfrietlcls arid co1lea~uc.s. Special tfl;ink$ to mu- w ~ p - port trarrz incl~~tliiig AIXI~ Gtipta. ; l t ~ r l ~ - e a Fellows, .Anastasia ;2nderuorl. Srishti C;itpra. Mona Pall. Jona~han Kirsch nnd Chitag ,211ii11. FOI- p i o r c.oi~trihuiions WP ~li:l~ik C;iatllli Le Nguyen. Tanu l Mar l i t r r ; Alex Grimin. Sonia Snn to5 imrl Eli~:thettl ,Satlrlel-<.

Mi. ha\r cr!juyc.d working with a t~o~.ld-claw i nter-national p ~ t h liut~iilg gro\lp a t Rlxrkwrll Science. inrlucIing Lanul-a DeYoung, Anlv Ni ~ t t bt-ock, Liva Flanagan. Sha~vn Girshrrgcr, Lurna Hind and Gclt-dun Tihhitts. Fur hclp wit11 .;ucuririz irr1age:e9 h r . t he cntirc scries ~ v c also thank Lre Mar-tin. ki.;1uphc1- J o r l e ~ . Tina P a n i ~ / i a n d Prtrr Ander.soii at tht5 L!ni~.er.sity of' Alnhania, the ,L\rmed Fnrces Institute or Parholop, and m:my of our fcllo~v Rlack~rcll Scicncr a ~ ~ t h o i s .

For suhnii tting comments, corrections. cdiiir~g, proolreading. ant1 ;trsi$rat~re i l c1 .1 )~~ all o f (he v i g n ~ t r ~ t i t l e i r ~ all editions. ~ v c

collcctivcIy thank:

%I;I ~\ct,rniovich, C:i~r.rllvr~ d%lexantler. liris .Ildrn. P Ien t~ E. ,krva~l. Cynnran Bacolor, Natnlie k~rtcncva, Dcan Bar+rholomew, Det>ashisll Hel>rra. Sumir Rhntia, Sanjay Binrlta, I>a~~\lr Ilrinton,

Jt~lianne Bt.n~vt~. ,2Pexat1rlrr I<ro\~,nie. 'Tanral-a. Callal~an, Darid C:anes, 1h-y;tn Caw?: h a r m Ca~ighev, k I ~ h e r ~ Chen. Sanatl~an CEle~ig, R~nnld Cheung, :lul-~~old Chin. Silnion Chiosca. Yv'oun C:ho, Sitrnriel Cl~ting, C:retrhen I:r)ii;ln~, \laclimit- Cnric. Christ npher C;n~grnve, Rnll.tlrl Cswa~i. LIT-c kin R. Cunningham, A. Scan Dallcy Rama Dandaln~tdi. Sunit Qas, Ryan i-brnando D;r\-c.J~ht? David. Emrnanud c l r 1;1 C ~ u z , Rolx i - t Duhlello, Navtieet llhillon. SIiattnila. Uissanaike, Daritl Donson. Adolr Etclicjiarav. N r a Euschio. Priscilla A. Frasc. Daricl Fr-cbnj-, kis l in C ~ I I I ~ P I ; Y<lh;~r~nes C1~l,l-ecg/iabhel; AniF Gehi, Tonv George. 1 . .M. Gninncn. Pat11F <;oval, Ales Gritnt~i, Rjeev Gtpta. Ahmad I-lalim, Sue I-Iall, Davicl IIx~sell~aclicl; Ta1m-a Ilcimrrt. Michelle Higlev, Dan Hoi 1. EricJ;icksr~n. Tirri J;~cLson, Stmdar Jayararnati. Pei-Ni+Jnne. Aal-rlian+loshi. Ki-7jni K.+Jtltla. Fail~az liap;~di. Seth I h p , liaron S. Ke~serheim, Sans Jdihan, .h~rlrcw Pin-wci KO. Francis k n g . Paul Knnitzkr: 1lT;t;lrrc-n S. Eirackuv. Benjanii11 H.S. 1,311. 1111 t i 1 JC:;ISCP. COII n it= I ,ee, Srn~t I .cp. Gt~iIlermo Lel~inar~n. Iicvin Lcung. Paul Lcvctt, M'arrcil Lc~inson . Eric Ley. FFien Lin,

Pave1 L0hano.r; J. Mark Maddox, Aram hlardian, Sarnir Mehta, Gil MclmecI, Joe Messina, Rohert Mmca, hfichacl Murphy, Vivek Vandkarni, Sim Nan>-an. Car,vell Nguyen, Linh Nguyen, Deanna Noblcsa. Craig Nodurrr, Georgr Norimi, Darin T. Okuda. Adam L. Palan re, Paul Pamphrus, Jin ha Park. Sunny Patr.1, Ricardo Pietrnhm~, R i m L. h h ! , Aaqhita hnderia, hchan Reddy Beatriu Rcjg. Marilou Rrves,Jercmv Richmon, Tai Roe, Rick Roller, Rajiv Roy, Uiegn R~i iz , , r lntho~~y Russell, Smi jay Sahgal. LT~rnilnala Sal-kar. John Schilling. lsahe! l Schmitt, Daren Schuhmi~chrr; Sonal Shah, Facli A b r ~ Shahin, Mae Sheikh- ~'lli. Edic Shen. Justin Smith, John Str~lak, F .iIlian Su, Julie Sur~rl; trar~~. Rita Suri, Seth Swe~tscr, i 2 ~ ~ ~ o n i o Tahvero, Merita Tan, Mark Tanaka, Eric Taylor, Jess Thonipsoil. lndi Tt-ehan, I<aymoi~d Tin-ncr, Okafo Uchenna, Eric Uypanco , Kicha Varma. John M7iiges. Alan Wang, Eunicc Wing, ~ZntlIy Weiss, Amy Williams. Rrian k-ang, I-Ian): Zaky, hrhraf Zaman and Da\;id Zipf.

For generously confsibrlzing images to the entire Lhdmpund Uinzrol L ' i p ~ t t r Step I serieq, we collectively thank the staff at IElarku,ell Scicncc in OxFn~-rl, Uoston, ;tncl Berlin as we11 as:

~.L~rorcl, J. Mede'cin~. [Oqney Mead: Rlackwrll Science Ltd, 1996. Figures2.14,2.15,2.ltj,2.2T,2.2P,2..11,2.35.2.36.2.38,2.49. 2 . 6 5 ~ . 2.65h, 2.65c, 2.103b, 2.1051>, 3.20t>, 3.21, 8.27, 8.27b, S.77h, 8.7'7~. I0.81h. I0.9hi, 12.283. 14.6, 14.16, 14.50,

Balln ister b, EEegg N, Gillcspie S. ~ I ~ / P I . ! ~ o I ~ , F Ilzs~nse. 2"Tditimn. Osnry Mead: Black~vcll Sr ie i~ce I,crl, 2000. Fip~r.es 2.8, 3.4. 5.28, 18.10,'Clr:i.32, W5.6.

* C~~sc l~ ie r i 12. HennessyTPJ, G r e e n l ~ ~ ~ l g h RM. RowlqDrZ, Grace PA. CliniroI . I"rrrg~~. Osnev Mead: Elackwell Science Ltd. 1996. Figt~res 13.19, 18.22. 18.33.

Gillcspir SH, Ram fn t-rl R i l fd i cd ,~4;mhiolt!g nnrl In fkrf irm nl a

Glmrr. (3~11ey Mead: Blachvrll Scjrnce I.rrl, 2000. Figures 20,23.

Ginshrrg L.. 1,~rflirr Notps on A;rrrmlogp, T"'I;:diiion. Osney Mearl:

Rlackwel! Scicncc Ld , 1!)99. Figures 12.3. 18.3, 18.3h. - Klliatt T, I-Iatings M, Dessel het-ger U. L.~rium I\lut~s on ~Werlirnl iMirrohinir~gy, 3"'Eififio?r. O s n q Mrad: Rlackwell Science Lrd, 1997.Fiprscs2,5,7.8,9, 1 1 , 12, 14, 15, 16, 17,1I~,20,%,26, 27. 29, 30, 34.35.52.

Mehta ,a. Hoffbrand AV. Iic1mnotology~f rt G/nnw. 00~nev Mead: Rlackwe?l Science Ltd, 2000. F i ~ w e s =.I, 22.2,22.3.

Pleasc let us know if your name has heen missed or rnisqpeller3 and w t will he happy tu make the update i11 the next edition.

PREFACE TO THE 380 EDITION v

We wcre very pleased with the overwhelmingly positive student feedback Tor the 2nd edition of our U ~ t d p ~ p ~ d n d Clinical TGp~tles series. M1elf over 100,000 copies of the UC?J honks are in print and have been used by students all over the world.

Over the Fast two years we have accumulated and incorporated over a thousand "updates" and improvements suggested by you, our readers, including:

many additions of specific boards and wards testable content

ddetians of redundant and overtapping cases

reordering and reorganization of all cases in both series

a new master index by case name in each Atlas

correction of a few bctual errors

diagno'is and treatment updates

addition of .%20 new cases in every hook

and the addition of clinical exam photographs within UCV- Anatomy

And most important of all, the third edition sets now include two brand new COLOR ATLAS supplements, one for each Clinical Vignette rerim.

* The UW-Bnsir Sr.in,cp C'oEm Atlas f Sfq 1) includes over 2.50 color plates, divided into gross pathology, microscopic pathology (histology), hematology, and microbiology (smears).

The UW-Cliinlral Science Colw Arlas (St$ 2) hnq over 1 23 colol-

plates, including patirnt images, clermatology, and funduv copy.

Each atkas image is descriptiveIv captioned and linked to its cor- responding Step I case, Step 2 case, and/or Step 2 Minicase.

How Atlas Links Work:

Step 1 Book Codes are: A = .X~~a~unrv Step 2 Book Codes are:

R5 = Rehauinr~l Scicrlct KC: = R i n c h e m i s ~ r ~

W t = k,Ticrohinlnp3 Val. I >I!! = h~$ic~~ol>iolop-. l'nl. I1 1 P 1 1 1 1 l \'<>I. I r'l = I'atlirrphvsir~lr~fiv. Vnl. 11

F3 = P : i l h c r p h ~ ~ i n l o ~ , Val. I11

ER = lirnrrjienc? Medicine IhI1 = lnrrrnal MrrIirine. Vol 1 IM2 = lrutcrr~al lqedicine, K ~ I . I1 \TI I = Neu l . u I u ~

OR = O R / m I'lCD = Petli;itnr%

SCR = S u r ~ c - r b

PSI.'= F~vrli~arry M(: = \{iniCiqr

r:lrl IMP3-032A ER-0356, ER-035B

/ Indicates Type of Image: M = I - I ~ m a t c ~ l o ~ 7 Indicates UCVl or UCY2 Series

11 = blic rnt,icjlokq

I'C; = Crrrs5 Patlinlrqv PM = hTrcrn.;copir Pathnlrp

* II'thr Casc numher (032, 03.5, ctc.) is rlcll fr)llnwed by a letter.

thcn zhur-t- i s oitly one image. Othcmjse ,I, E, C . D inclica~e up to 4 itnages.

Bold Faced Links: I n or-rter to g v c you access to the largest number of inlages possible. wc have chosen to cross link the Step J a n d 2 series.

IT the l ink i s bdd-racer1 this inclicatcs tl~al [he link is direct (i .e.. Step 1 Gasc with [he Basic S~ience Step 1 Atlas l ink).

Tf llle l ink is not halbfilccd tltix indica~es that the Tink is indi- rect (Stcp 1 caw %it11 CEinical Science Step 2 AtIxq link or vice

\rema).

1t7e h a ~ v alsu implemented a FPW s t r n ~ ~ ~ ~ i l l C ~ ~ T I ~ P S 11p0n your request:

Each current and firtur-e eriitinn or our popular Rrvr ,.lirItnr

t h ~ IcSIViL,F S / P ~ I (Appletoil & Langr/McC:rxu,-Hill) ancl Fzrst Aid/?). I ~ P I !$il/ll,E': Stqh 2 (Appleton X. Laiige/MrGmm,-Hill) honk will he Einkcd lo the curl-rspnnrling IICV case.

Wc eliminatetl LTCX -+ First Aid links as the). f i e q ~ i ~ n t l v hecotne out ol'datc, as thc Firsi Airi bunks are revired ycasly

Tl~e C:nlol- Atla< is also specially cl~signed for- quizring- captions a m descriptive and d o riot give away thc case name clirec~~ly.

'Mir hope the updatcd UrnT \erie? will rcmaill a I I I I ~ ~ ~ I P and wcll- integrated st~lclv roo1 that prorides conlpact cliniral corrclations to hasic ~ c i e n c ~ information. Tlte), are deqignecl to hc easv and l i ~ n (comparatively) rn rrad. and I~elpfi~l for h n h Eice~~sing exam% a n d thc wards.

Clrt: irlvitr: yorlr corrections and sriggestinns for thc fourth edi- lint1 or hese hooks. Fur the fir<! S I I ~ I ~ ~ S F ~ O I ~ of caol~ factual cor- reccion or t ~ ~ v vipetre that is selectecl for incluhion in the

fourth edition. yozl will reccivc a per5orlal acknc>wledgrnerit i r l

tlw revised l>r)r>k. 1 C inu submit ovcr 20 high-quality corructions, additiorls 01- new ~igncttcs we will alco consirler inviting you to

become a "Contrib~itor" on the 'book of your choice. If vou arc interestccl i r ~ becnrning a poteiltial ' 'Con~i ih~~tnr" o r ".4uthor" on a f i ~ i i t ~ LICV hook, 01- wo~-ki~lg ~ ~ i t l i OUT team i l l developitlg additional tlooks. plensc alsu e--lllaiI 115 y011r C,Y/rcsume.

Fl.TF prcfrr ihat VOII s~rh in i~ corrections or stiggertinns ria rlectronic [nail to [email protected]. Plea~c include "Llndergrotlnrt Vi~,mctte<" as tlie s~~l?jec t of yorlr n~ttssage. H' you dn not h a ~ c accms 10 e-111ai1, uqe the following mailing addresq: Black~vcll Fl~l~lisllfng, httn: UC,lT Editors. 350 Main Street, Maldun. M:"IO"E 48, USA.

HOW TO USE THIS BOOK

T h i s series was originally developed lo artdress rhe increasing number of clinical vignette questions on medical examinations, incl~lding the LrSMLE Step 1 and Step 2. TI. is also deqiped to supplement and cornpl~mei~t the popular Frrrt Aid fm t h ~ ITSh.IIx St? 1 (Applcton 8: L.angc/ McGraw Hill) and Firs/ Aid frlr I ~ P IL~IVIJ: St$ 2 (Appleton Lange/McGraw Idill).

Each LlCV 1 hook uses a series of approximately 100 "supra- prototypid" cases as a way to condense testable hcts and associations. The clinical vig-nettes in this series arc desitped to incorporate as many testable fact5 as possible into a cohesi\.e and memorablc clinical picture. The t+iflettr% represent compi>sitrs d~awn from geneml and ~ p r c i a l ~ textbooks, reference hnoks, thousands of USMLE stdc qr~cstiorls and the personal experience nf the authors and reviewers.

Al~hough each case tends to present all the signs, symptoms, and diagnostic findings for a particular illness, patients generally will not present with such a "complete" picture either clini- cally or on a medical examination. Cases are not meant to simirlnle a potential real patient or an exan1 vipette. All the boldfaced "buzzwordsv are for learning purposes and are not necewarilv expected to he found in any one patient with the disease.

Definitions of' selected impnrtanr terms are placed within the vignettes in (S~ZZLL CAPS) in parentl~eses. Other parenthetical remarks often refer to the pathopl~y~iologv or mechanism of disease. The forrnat shnulrl also help s t~rr t en~~ learn to present cases succinctlv during oral "hullct" presentations on clinical roulrionr. The cases are meant to serve as a condenser1 review, n o t as a primary reference. The information provided in this book has heen prepared with a great dc-nl of tlloughr and care- f ~ 1 research. This book should not, hc~wever, he considered as yolrr ole source of information. Corrections. suggestions and submissions of ncw cascs are c*ncouraged anrl will be acknowl- rdged and incorporated when appropriate in r r ~ h ~ r e editions.

5-MA ABGq AB\m ACE ACTTI-1 ADI.1 AFP tu AInS ALL ALT AWL ANA m s ASD AS0 AST AV RE BP RCIN TAD CALL4 CRC: CI-IF CK CI,L CML cm3 CNS COPD CPK CSF CT CTA CXR DIC DIP DIU DM DTR.; DXT

5-aminosaliytic acid arterial hluod gases adriamvcin/blr.ornyci~~ /\incristine/dacarbazii~e angiutensin-converting enn;me arlrenocorticatropic hormone antidiuretic hormone alpha fetal protein aortic insufficiency acquired immunodeficiency s ~ d r n r n e acute lvrnphocy~ic leukemia alanine rransarn i nase acute myelogennz~q leukemia antinuclear antibody add r respiratory distress syndronle atrial septa1 defeci anti-stseptolysin 0 aspartate transaminaqe arterioven 011s barium enema blood pressure bloud urea nitrogen coronary artery disease common acute lyrnphublastic leukemia. antigen complete blood coullt

congestive heart failure cr~atine kinase chronic lymphocytic lelrkemia chronic rn).elogenous leukemia cytnrnegalovirus central nen70us sJIstem chronic obstn~csive pulrnonars disease creatine phnsphokinasr cerebrospinal fluid computed tomography cerebrovascular accident chest x-ray disseminated in tramscular coagulation distal interphaIangea1 diabetic ketoacidosis diabetes mcllitus deep tendon reflexes drep ven t ~ i is th rnrnhosis

EBY ECG Erhcl EF EC;D EM[; E RCP ESR m'iT

FN.4 ETA-tZBS nrC: CiFR GH r a GM-CSF

CU H .4v hrC; HEEAT FI lV ITL4 I-IPI HR tIKlG 1-1 s ID/GG TnnM I?7 TGF IM

.FT LLTR 1,lIM LES 1J-r~ I,P I .v LXW I,vt es MCHC MCY MEN

Epstein-Barr vil-ur clcc trocardiok~aplly r ~ l ~ o c a r - ~ l i o ~ r ; ? p l ~ ~ ~ qjrc~inn fixction eso~~l~agoj in~t rod~ior l c'no~cvpy cleccromvography cndowopic retrc>~prlc rhr)latrgc)pancrea~ography ery01rncl;te ~ctlirr~en t;tlion 11 tr

fin-retl rxpi1alol-y vcllun~e fine needle aspiratio11 fl~iore.;ccnt trrpol~clrlal antihr~dv ahuo~-ption fbi-crrl vili11 rapacily g l n m e r ~ ~ l i ~ r JiErrarion rare grnvth horn~ollc

pstrt)intcstiri;~l g ~ ~ n u I a c ~ t e ~~~acrlc>phage colnnv stiinulating l ~ l c ~ ~ ~ ~ - geni touriui~r\ hrpa~itis A \inis h ~ u n a n choriorlic goriadoti-r qlh it1

head, e\fe.r. cam, HOSP. :111d tllro:lt 11 tunan imrn~~n(~c l r f i c i rnc~ vi n I s

human 1ri1koc)rrcr a i i ~ i ~ r t ~ Iiiszoc o[ present illness 11~;ll.t rate hi~rnan n h i r s i ~ n i n ~ ~ n e gloh~tlirr hcreditiir?l S ~ I I P ~ C ~ C V I O F ~ S irlrnrificalion nnrI rhief rnnrplaint infi~lin-dc-pendent rlialnctcs 1ncl1itu'i immunoglahulin insulin-like growl h I:lrtor i t ~ t r a ~ n ~ ~ ~ c u l a r ju~qilar vcnous pr*c.ss~x~-c. kirlt~evu/ur~1er/hlacIrlct' Ixclate drl7vrlrogcna~e lower esopl~ i~se i~ l s p l i i ~ l r t ~ r I~\'L'I- fltnctio13 PSIS

I~rrnhar pzinct~lre left ventricu'tar letr v ~ n I rir~ilal- liyperti.nl~lly elertl-olvtes tnean corp~rscular I~cmt~~ l : l o~> i r~ conceniration Itlean ror.p~wc111ar r.r)?~ime rnriltiplc endocrine nrcl1d:rsi;l

MHC MI MOPP

MR NH1, WDDM NYC3 NSAlI) P.4 PTP P RS PE FFT.5 PMI PbIN PT PTCA PTI-I PTT PI! D RBC RPR RR R! RV R\W SRFT SMI>H SLE. ST13 TFTs (PA TSlH TfBC TIPS TPO 'TSH TTP U-A UGI LT S

tnanoclol-tal gatnmopathy nf undetermined signihrance tnaic~r I~istnronipatihili~y complex rnyocarclial inCarc~inn incchloretharnine/~~i~~cristi~~~ (Oncworin) / procarhazinr/prudnisvnc ma~netic resonance {imaging) non-H(~cljikin's lymph oma non-ins111 ii14epencleti t diabetes tnrlf i t us nil per us (nothing by mouth) nonsleroidal an ti-inflanlmutov dnlg postercranlerior proximal in tel-phaIa1igeal peripheral bloocl smear pliy~ical exam pulnionaw funct io~r tests p o i u ~ of maximal inteusitv polyrnorp honuclear 1 ~ 1 t kocvte

prothrombin tinlu percntanrnus trandarninal ;itlgioplasly parath~.roicl hot-]none r~artial thrumhoplastin time pep1 ic lilcer disease red ldoocl cell rapid plasma reagin I-uspiratory rate Keed-S~erttbrrg (cell) right ventrirular sight ventricular hypertropt~y small ho~vcl follo-rv-th l-ough ~ycndrome of iizippropriatr sucrction of ADI-I sy-stcmic ! E ~ ) ~ I L S e l -y th~n~aco~u~ ~exu;tlly ~rai-i~mittcd diseasc thyroirl r~lttction tests tissue plasmii~ogeii art i r a ~ o r Ihvr-oirl-sl i~rlzilating l lomonc total iron-binding capaciq uansjup~lar intnhepatir portoqstemic shunt thyroicl peroxirlase rhyroid-sti ~nulating hurmonr thrombotic tflrom I2ocvtopenic puvut;l t11-inalysis upper GI ul trasouncl

VIIRI, Venereal nisexse Re~earch Laboratory VS rimI s i p s \rT i~c~>tricular tachvcardia MBC white hloorl cell MTIV Wow-Parkinson-U'hi te (syn dmme ) XR x-ray

ID/CC h 49-vvaruld maIe immigrant who is a nativr of Guam ha? sern three doctors in his country ancl tried d f i r e n t therapies for marked. progressive weakness of his hands and arms, difficulty speaking. muscle wasting in both hands, and troul~lesonke involuntary rnusck conhactions (wscrc~lr ,~TIONS).

HPL He has no history of sensory symptoms, hlaclder or howel dysfimction, fever, exanthem, dog bites. vaccinations, o r spinal or cranial trauma.

PE Lawer motor neuron signs: bilateral wasting of hands, deep rendon reflexes absent in upper limhs, rntlscle weakness, Eascidations; tipper motor neuron signs: poqitive Bahinski's ~ i g n , stiffness and spasticity of upper limhs: normal fundus, sensorv system, and cranial nerves.

Labs LP: CSF normal. Slightly elevated CK; norrrlal TSH, T2, and T, levels; normal sertxnl calcium and glucose. EMG: partial inner- vacion with abnormal spontaneous activity in resting muscle and reduction in motor units under voluntary concrol.

Imaging CT/MR, brain: brain normal.

Micro hthalogy Nonspecific atrophy on muscIe biopsy.

Treatment Lartrgelv supportive; disease is proq-sessive and faul.

Discussion N s n known aq Lou Gehrig's disease, amyotrophic lateral sclerosis (ALS) is a slowly progressive, generalized motor muscle paralvsis

AMYOTROPHIC LATERAL SCLEROSIS (ALS) r : !

ID/CC A 60-year-old nlillc. presents rvit11 speech difficulties.

HPI The patient devclclprd this difticuliy rc~llnwit~g a left-sided stroke from wllich he is currentlv recovering. IIc is a diabetic whcl hits t l ee~ i o n insulin Tor I0 ?leai-s. and hc is alsr) a chronic smoker.

PE S p w c l ~ lacks fluency; patirnt 11:~ rlif'ficult~~ finding certain words and rornerilnes produces wrong word; comprehension is well preserved, as i l T r Fiiglier mentill I*~tnrtinns; ahiljtv tn reprnt is 11rlte1- I hat] spn nlalienw ~peech; assnciatrd rucovcrin# 1-iaht- sided aphasia noted: motor wcakr~rss of r igh~ upper anrl lawer Iim hs with rxaggeratcci tlcup tclulon i.efexes a~tr i right-~idecl Bahinski's rt.flcx.

Labs Elrv;~tt.rl hloc~rl glllcosr: retnaitirl~l- ol' tests ~~orn la l .

Imaging CT': infarct in region of left frontopanetal cortex.

Treatment Speech therapy in addific~u t o phvuic I t herap? for qtrt~ke; Inng- tei.rr1 lo\+*-clor;e aqpirin.

Discussion T h i ~ p;~ticnr has, RI-1 u ' s dyspha~ii~ (exl>l.e~~ive, nonfluen t) with an aswciated right l~emiple~ia . 'Tl-ie hrnii~ rlnmage causing this rtmrlitinn ir he1 i~verl 1 0 involve the dominant inferior frontal g y m s tl31inr:i's AREA). i n contrast to patirrbls with M'rrnicke'q iiphasiil. piltie111s will1 Broca's nphasia have insight into thuir

HPI Me [ell wrlically so that his heact hit the gmund first. Despite the injury. 11c is corlscio~~s and does not report any neurologic deficit, only ever-e pain in his neck.

PE No neurologic deficit found oil clinical exarnir~aticln

Imaging XR, cervical spine: burst fracture of atlas (JEETEWON FRACTURE) 0 C-I

with ring broken into four pieces. <

Treatment I r t heren LIT uns~ahle fractm-e requilir~g halo jacket immobilization and Fl~sion if nnnunior~ occnrs.

Discussion The most crjmmun mechanism of injury in patients with

JeTfersnn fracture is axial luacling. Othus cervical spine injuries include atlantoaxial fracture dislocation, more frequcndy associ- atrcl 14th tle~~rolngic clefici t ; rliqplacernen t is conirnonl~ antcriur, and treatment consist5 ol' skull traction followed by immobilization. I\ violent flexioncompression foi-ce may result in a sudden prolapse of h e nucIeus pulposus of the cer\-icaF disk into the vertebral canal, prorlucing quadriplegia: here an early decompression i s rrq~~irm.

F"/ C 1 SPINAL CORD INJURY

ID/CC A 70-yearslid male presents with dull, aching pain in both calves after moderate exercise.

HPI The svinptoms starter1 a few month5 ago, h.picallv developi~~g after the pa tier^^ wal k ~ d 300 to 400 yarrls; symptoms were relieved after a few minutes' rest or when the patient sat down and stooped Forward (pseudoclaudication) . In addition ro the pain, the patient has exprrie~iced r ~ ~ l m h r ~ e s s in his thighs. He ha? had 1 1 0 sphincter disturbance but has had low back pain for many years.

PE Spii-tal exam reveals loss of lumbar lotdosis and reduced flexion and extension of lumbar spine; tone, power, and coordination in lowcr limbs norma[; reflexes in lower limbs symmetrical but reduced compared to upper limbs; plantar rcfleses flexor; peripheral arterial pulses present both at re91 and after exercise.

Labs Lab parameters normal.

Imaging XR, lunihar pine: lumbar ~ponclylosis with masked osteophpe formation. CT, spinc: Itunbar spinal canaI stenosis confirmed.

Treatment Surgery requiring laminec~omy at varin~is 1eveIs.

Discussion A number of mcchallism~ ma?7 lead to lumbar canal stenosis, including osteoarthritis wilh hlrpertrophy of the fhcer joints, disk prolapse, surgerv, spondyfnli~t llesis, Paget's disease, neoplasia, and infectinn; any of thcsr conditions m:tv be st~perimposed on a congenitallv ~ ~ a n - o ~ v cpinal canal. The anternposterior diameter n l the cord i s narrowed dliring extension, which tcnds to

cornprnmise the hloorl supply of the cord. resulting in the devel- opmrn t ol' symptoms; stooping forward docs the reverw and

* CAUDA EQUINA SYNDROME

ID/CC A 40-year-old male complains of the 'Lworst headache of his Iife" and double vision.

HPI He has been projectile vomiting. He has no history of fever or neck stiffn~ss.

PE Papilledema on funduscopic exam; right eye deviated IatexdIy z- m

and downward (due to right third crarlial nerve palsy); other t FU

cranial nerves normal: no mening~al signs noted; motor system ? 0

examination normal. 0 <

Labs Routine laboratory rests normal.

f rnaging Angio, cerebral: posterior communicating (PCOM) artery aneurysm. CT, head: enhancing mass impinging over right third nerve.

Treatment Endovascular or new-osurgical clipping of anet~rysm.

Discussion Congenital berry aneurysms are associated with polycystic kidney disease and arteriavenous malformation; tlzep may rupture (during sexual activity, weight lifting, straining) and cause subarachnoid hemorrhage.

CEREBRAL ANEURYSM

ID/CC A I 4vcar-olcl w l ~ i te male coines into the e m e r g e n c y room hecxuse of projectile vomiting and a vevere headache.

HPI HP ha\ a hislory of ~inexplaincd short stahre and polyuria.

PE Papilledema and optic disk swelling (cluc to incruasccl i~ltracranial pr.c.ssurch) on ii~r~dttwnpic esarn: con h~ston: visilal licId testing reveal5 biternporal hernianopia; no other fr~c;~l rlr~~r-ologic signs: no ileuroclltaneous n~arkel-5 or meningeal signs.

Imaging S R , sklrll: enlarged sella turcica. CT/MR: enhancing. cystic, 111111 I ilohulat ~ ( 1 supraqellar mass with ring calcification: h~~dl-ocephaltlq (due t o oh~truction of fommcn of Monro and aqucdr~ct of Sylvivius).

Gross Pathology Cystic mas.; with concen~ric arras of'calcificatinn.

Micro Pathology Mixture of squamnris epjrlielial e l e ~ r l r n ~ r ; I I I ~ ~lelicate ret i~ l l lar s vomi~: gliosis seen ;II per.iphrry; cho1este1-01-rich cwtic fluid.

Treatment Slu-gical removal; mdi otlierapy.

Discussion C:rilitiophar~giclma is he most common supratentorial brain tumor in children and i s rrnhr-yologically cIeri~ccl from Rathke's pouch remnants. Ir is a cominon cause oTgrowth srtarclalion, rlinl~ccc~ insipidus (compression of pi tt~imry), biiemporal ltrrn ianrlpia (complrssion ol' optic rhiaqm). m d I~cadaclir (nhqtr~rrtive h~~risnccphal~~s) . It shows ;I birnoclal age diqtritnt tion with a scconrl peak in lhc fifth decnriu.

Atlas Link n-mI PEP3-006

FCI CRANIOPHARYNGIOMA

ID/CC A 62-year-old man is brought to his family doctor beca~~se of rapidly prop-essive loss of cognitive function (DEMENTM) and excessive somnolence.

MPI Five Years ago, he received a corneal mansplant. His wife slates that she ha? seen a definite change in his personality over the past year,

PE Dementia; myoclonic fascidations; normal frinduscopic exam; no other focal neurologic signs.

Labs LP: normal CSF profile. EEG: bursts of high-voltage slow-wave activity and slow background.

Imaging CT, head: ventricular enIargcmcut and cerrhral atrophy. MR, lxain: increawd signal itltrnsity in affected areas. PET, brain: areas of diminished glucose metabolisn~.

Micro Pathology Brain biopsy shows amyIoid depositio 11, spongiform degeneration, decrease in ner~ro~ls of cerebral cortex, and assrocytir proliferation; no inflammatory changcs sccn.

Treatment llsl,~ally fatal; vidarahine and amantarline are being tried.

Discussian A subacute spongiform cncephalopathy with a very long incuba- tion period, Cruntzfeldt-Jakoh disaase is presumably caused b y a slow virus or prion and transtnitted via coi-neal transplants, dura mater allografts, contanlinated cadaveric growth llnrmonu, or

neurosurgical contamination. Lithium overdose mav mimic signs and symptoms.

CREUTZFELDT-JAKOB DISEASE

ID/CC A 35-year-old woman known to have rheumatic mitral stenosis awakens in the morning to find the right side of her body paralyzed.

HPI The pasient also complains of palpitations. She has no history of fever. neck stiffness, vomiting. Iteadacl?c, or transient ischemic attacks (TLAs).

PE VS: na fever; irrrgularlv irregular pulse. PE: dcnse right-sided hemiplegia; brisk reflexes on right side; right-sided Babinski (FYITNSOR PL.Z~T.AR RESPOYS~) present: f tindtis normal; loud S1; apical micldiaqtolic mnrmur ant1 opening snap.

tabs ECG: prcsence or atrial fibrillation confirmed in addition to P-mi~rale. Blood culture sterile; roil tinr lab lest5 normal; riotting tinre. bleeding time, and PT normal.

Imaging Echo: left atrial thromh~is. CT: scan perrol-med arter 24 hours reveals infarct in posterior limb of left internal capsule.

Treatment Start heparin after foI10w-up; therapv guidcd with PT, digoxin for management of a~rial fibrillation; valvuloplasty or valve rpplacemen t after resolution of Icfr atrial thrombus.

Discussion Mitrat stenosis with atrial fibrillation predisposes to thromboern- holism.

CVA, CAPSULAR INFARCT

ID/CC A 65-year-old white male develops sudden severe headache and right-sided hemiplegia.

HPI The patient is a hown hypertensive and takes his medication irregularly; he now has both urinary and f e d incontinence.

z 2"- PE VS: severe hypertension (BP 21 0/ 180); no revel-. PE: dense m

right-sided hemiplegia; funduscopic exam reveals presence of C ;o 0 papilledema in addition to hypertensive retinapathy; right-sided

Babinski; eves deviated toward left; no meningeal s i p s present. 0 G, 4

Labs Routine labs normal; LP not done, since intracranial pressure {ICP) raised.

Imaging C T , head: facd hemorrhage in left putamen region of basal g;inglia.

Gross Pathology Autopsy: mass of bIood dissecting through parenchyma inlo deep structures of brain and ventricles.

Micro Pathology Hvpertensive change., seen in adclitioil to putmenal hemorrhage; hyaline arteriolosclerosis: lipohyaliaosis; Charcot- Rouchard aneurysms.

I Treatment Supportivc management to reduce ICP and blood p r e m .

Discussion Bleeding is niost often caused by hyperfension. In the preserlce of moderate to severe hypertension. d penetrating arterioles may rupture deep within the hrain, causing a hematoma that displace? hrain slructurps. Common sites are the putamen, thalamus, pons, and cerebellum.

CVA, HYPERTENSIVE

ID/CC: h I!+=-ar-old remalr Olvmpic it~orseback rider is brought into the emergeucv room with headache, codusion, weakness o f the left side of her body, blurred *ion, and projectile vomiting.

HP3 Three Iinltrr ago, 411e hit thc right side nf her head when shc fell frnrn a horse during a training exercise. SIle lost consciousness For I minute ancl then appeared to havr recovered completely before prewnting with the syrnptomatolop ((crrcrn ~ N ' F . R V ~ I . ) .

PE VS: RP mildly tlevatud; bradycardia. PE: papilledema: right- ~iderl mydriasis; dfel-en t pupillary reflex a h n o r m a l i ~ on right side; deviation of right eyeball outward and downward (RIGHT

CN 111 PAWY); left-sidcd weakness; brisk reflexes on left side; extensor plantar reresponse on left side.

Imaging CT/MR, head: r i ~ l ~ t trmpnra'al hone fracture; right-sided lens-shaped (convex) hyperdense extra-axial fluid collection.

Gross Pathotogy CoIlection of blood hetween dura mater mi3 skull wihrith mass

effect.

Treatment Emrrgcn t surgical ctacuation.

Discussion The results of arterial bleeding (rupturc of middle meningeal artery) are ~~siially assnciated v.5 th skull fracture. Classically. the patient loses consciousness immediately aftel- Ileati irljur): but rejiains consciousness and remains asvnlptomatic for a vasiablr perinct of time before qmlptoms worsen.

Atlas Link IG-P3-010

? EPIDURAL HEMATOMA

ID/CC A 19-ycnr-old male visit-5 his orthopediqt hecalrsc of a wide-based gait (ATAXIC: GAIT), congenitdl clubfoot. anrl abnormal lateral curvature of the spine (st:o~.rosrs).

HPI For the past scvrral years, lie has l ~ a d increarring difficulty walking: the procefs lxgan snhtly, hut Ile is now incapable of participating in sports. I-Te also complains of a proLgressive zW m diminution of vision. C

;E1

52 PE ITS: arrhythmia: tachycardia. PF.: hi131 era1 concentric cot~traction b

m of\.isuill f:elds on viwal field testing; findings slrggestivc of retinitis

<

pigmentom r ln retinal exam; scoIiosis of thoracic spinc; pcs can is

def(3rmity of righ ht foot; dirninish~cl sensatioil in stocking-glove distribution; proprioceptive sensory loss; areflexia; ataxia of li rn bq: Rabinski's sign: forceful pulse; prominent JVP; slutained apical impulse; S4 ( r l ~ ~ e to hypertrophic cardiunlyopathy).

Labs ECG: leCt ventriclrlar hypertrophy: invrsled T waves.

Imaging Echo: evidence of' hypertrophic ol7strr1ctivc rcadiomvopathy.

Micro Pathology Marked 105s of cells in posterior root ~ a n g l i a and degeneration of puripheml sensory Fibem: po5rerir)r atlrl lacel-al colun-ins of CNS a l w affected.

Treatment En treatment available.

Discussion Tile mc)st cominon hel-edi cary ataxia. Friedrcich's ataxia is an autosomal-recessive disorder d ~ c to a drfective gene on chromusomc I),

I D JCC A 60-year-old white male complains of headache that is worse in the mornirq along with occasional nausea and vomiting for 6 weeks.

HPI One day prior to presentation, he had an isolated grand ma1 seizure.

PE Bilateral papilledema: loss of recen r memory: brisk deep tendon reflexes on right side: Babinski on right side.

Imaging CT/blR: irregular enhancing leftsided mass with necrotic

center; mass effect and surrounding edema.

Gross Pathology Hemorrhagic and necrntic tumor mass in fillrating left parietal lobe.

Micro Pathology Biopsv reveals presence of anaplaqtic cells with pleomorphism and endothelial proliferation: foci of necrosis surroltnded bv palisading.

Treatment Surgical resection: chemotherapy: radiotherapy.

Discussion Astrocytomas are graded accnrdin~ to differentiation; the highest grade (grade IV) is glioblastoma multiforme. I t carries a. poor prognosis.

Attas Links = PPEP3-012, PM-P2-012

GLIOBLASTOMA MULTIFORME

ID/CC A SRyear-nld male visits his family doctor complaining of syrnmeb-ic muscle weakness that started diszally in his legs and ascended gradually, now involving the trunk and arms.

HPI One week ago he suffered frotn diarrhea and fever and was diagnosed wit11 and treated for UnmpyIobcart~r enteritis.

PE Syrnmebricd proxima1 muscle weakness and flaccidity in lower limbs; ahsen t deep tendon reflexes; normal sensory exam; normal cranial nerves.

Labs Elevated gamma globulin. LP: increased CSF protein concentration without cellular increase: normal glucose. Nonreactive VDRL: decreased nerve conduction velocity indicative of demyelination on electrop hvsiologic studies.

Treatment Flasmapheresis; intensive care and respiratory suppnrt.

Discussion Gnillain-Barre syndrome i s a common causc of palyneuropathy in adr~lw that is usr~ally preceded by GI nr respiratory infection or by specific illnesses such as Epstein-Barr, Chmmfllohnrfm enteritis, and cytomegalovirt~s i~lfection. Respiratory paralysis

GUILLAIN-BARRE SYNDROME

- -

ID/CC h 50-year-old male preseu FS to tllc eincrgcncy room with wild, flinging movements of his left arm and leg.

HPI He has b ~ e n rliagnnxrrl with diabetes and hypertension but has taken his inrrlicarions nrllv i rreg~~lar ly . I-Ie i s also a chronic smoker.

PE L'nconunllecl, violcnt, rapid flinging movements of Ieft arm ancF leg; remainder of neurc>logic exatrt nonnai.

Labs L.ab t m t s reveal ~Ievated l>loocl g l u r o ~ ~ .

Imaging (T (dnne at 48 hour=,): infa]-ct in r-ight subthalamic nucleu*.

Treatment Phcnothiuincs and dopamine antagonists such as sulpiridc and tr.lriibunazine mil)' ke of l i ~ lp .

Discussion I-IumihaPlismr~s is ch;~racteri;.ed by force f~ l l , flinging., and violent inrwements. primarily ol' the proximal parts of thc. limbs of one .;irlr of I he body. rliat disappear cli~ring s1cc.p. The mosi corn- moil e t in lop i s that or a vnscdar rrTcnt in thr contralater.al srihthalamic ntlclc~~s: nther C~ZIISPS include an expancling arteric- venouq rnalfi~i~ma~ ion, I rail tna, nttnnr, and mu1 ti pie sclcrusis. tlosl cases resofve spnntaneouslv within 6 to 8 week$; howcber, $111-gerv Inay he inrlicatecI in caws nf in~ractahlr involuntary

* HEMIBALLISMUS

ID/CC sZ 42-year-old tnale presen rq with depression, pnor memory. and jerking movements of the limhs and fingers.

HPI: His father died of a similar rnnilitiml in which thc symptoms progressively worsened, proceeding to demen ti3 t ~ n ti1 h i 5 death at the age of 50.

PE Chorea; psychiatric emluation rrveals cognitive impairment (i~~arrention and poor concentration ~vichorit memory loss) and depression; no other focal neurolo.gic deficir Fo~~nd.

Imaging MR, hmin: degeneration nf caudate n~~cleus. CT, brain: cci-cbral atrophv.

Gross Pathology I,oss nf lxa in mass wit11 striking atrophy nf caudate nuclel~s a ~ d , less stt-ikitlgly, plltarnen: secatldarp 105s of neurons in gIohus pallidus; cortical atrophy moqt commonlv occurs in Frontal lobe.

Micro Pathology Degeneration oF piny GABAergic neurons in thc striaturn lcads to a net loss of inhihiton7 signals from the striaturn.

Treatment No specific trP;Itmen 1 wailahle: stipportive and ytnptomarir treatment; genetic cnunseling with regard to ~ I ~ L I I - c offspring.

Disc~ssion Huntington's chorea is an a~itosomal-dominant disease whocc gene Incus is nn clirotnasorne 4. I t is ratwed hy expansion of a

trinlicleotide repeat (TAG) within thc Huntington genu; cxpan- sion of the trinucluotidc repcat leads to greater frecjl~ency of disease in s~lccernh~e generarims (C;FNPI-IT: ANT~CIFJITIOV). T h r nnset arthe disease is typicallv hetween 30 atlcl50 years of age, pm,qressing to dealh tvi thin 1 5 to 20 years.

HUNTINGTON'S CHOREA

Labs

Imaging

Gross Pathology

Micm Pathology

Treatment

Discussion

A 25-vear-old male is hrought to a neurologist with compIaints of inability to see on one side.

Two months ago he suffered right eye optic neuritis, hut his vision has s i ~ i f i c a n t l y improver1 since then, aIthough it is not completelv ~lormaE.

O n lateral Kaze i t3 either direction, one eye does not adduct and the other has nyshgmus on abduction (finding characteristic of bilateral internuclear ophthalmoplegia) ; Cunduscopy reveals temporal pallor s f right disk (due to atrophy of papillomacular fibers); visual field testing reveals righr par-acentral scotnma: flexion of neck produces an elecbical sensation that runs down back and into legs ( I , P ~ E R M I ~ ' S SIGN: snggest? intramedulkary disease OF cervical cord).

LP: specific increase in CSF IgG concentration. Aprose electrophoresis reveab oligoclonal hands in IgG region OF CSF. Evoked-poten tial ~tuclies of visual, auditory. and somatosensory pathways indicate impaired responses.

MR, brain (T2W): investigation of choice: reveals multiple, discrete, whfte-matter plaques.

Sharplv defined area5 of grav discolol-ation ( I S L ~ Q ~ E S ) of white matter that occur particularly frequently around the ventricles and in the corpus caIlosum.

Activr plaques show evidence oC myelin breakdown, lipid-laden inncrophages, loss of oligodendrocytes, and relative preservation of xxons; ljmphocytes and mononuclear cells prominent at edges of plaq~ies.

Beta-in trrfPron; immzine~uppression (corticosteroids. azathioprine. cyclnspnrine) , hi1 t success has heen modest.

The following are suggestive of mi11 tiple sderosis: (1 ) optic neliritis, whose early s i p q include diminished visual acuity,

central or paracentral scotoma. hyperernia and edema of thv optic disk. and a defective pupillary reaction to light: (2) inter- 11uc1ear ophthalmoplegia ( d l ~ e to dernvelination of the ~nedial longit~~dinal Fasciculus); and (3) Lhermitte's sign.

INTERNUCLEAR OPHTHALMOPLEGIA

ID/CC A 13-year-old male is brought to a physician hy his parents for an evaFuation of recen tlv ohserv~d overkddgence in sexual activities.

HPI The parents also report that the patient's behavior has recently changed markedly from aggressive to extremely placid; directed questioning reveals that he has now started exploring things

z rn " PP'

c orally and has dev~loped a voraciolls appetite. He sufferer! from herpes simplex encephalitis a Tetv months ago. There is no o r

c' history of prier psychiatric illness in the patient or in the family. a <

PE Patient is in exceIlent health and i s apparently unconcerned about his illness. displaying no reaction to parentq' complain t-7;

when physician attempts to shake his hand. patient begins to

orally explore it; on ~ee ing a nurse in doctor's room, he starts to masturbate.

Imaging MR/CT, head: bilateral temporal lobe and arleygdala darnage.

Treatment No specific treatment available.

Discussion Kli~ver-Bttcv syndrumc is a syndrome of hyperphqia, hyper- sexuality, placidity, and hyperorality. In experimenta1 animals, it res~~ l LF rrom bilateral removal of the amygdala; in humans, an incomplete picture is generally seen secondary to extensive temporal lobe damage, as may occur during herpes simplex cncephaIitis or in degenerative or post-mumatic brain damage.

ID/CC A 6-year-ald maIr i s brought tu tile emrrgetlcy room wit11 acute- onset projectile vomiting, severe headache, and blurring of vision.

MPI The parietlz reports unsteadiness of gait that has progressively wor~rned ovcr the past 2 months. He has no hiftnry of seizures, fcvc.~, or neck stiffness.

PE Papilledema: no meningeal signs; nyta5rnuc in all dirrctions of ga;.~: trlmcal ataxia; cranial nerves normal.

Labs CBC: mild arlett~ia.

Imaging CT/MR. bfi~irk: hornogeneo~ts, enhancing maw in cerebellar vermis compressing and filling fourth ven trick; dilated third and lateral ven~ic les (due 10 oh<truc~ivu h?clrocephal~~~).

Gross Pathology Soft. wuIl-circ~im~cr~he~l, liah t-gravisli mass on cerebellar vcrmis.

Micro Pathology Once intracranial Ilressurc (ICP) is controllrd. CSF on lumbar puncture shows malipan1 celIs: highly malignant tclrnnr charac- trri;lc.d bv deupfv staining n ticlei wi th want ~\~opla5111 arranged in pseudomsettes.

Treatment C:oriicosteroids for increased ICP; entire nc-urnis irradiation;

surgical extil-pa tion; c hernothcrapy.

Discussion ,4 common tumor. of childhood and thcr most prev;~lent brain tunlor in clrildrcn less than 7 years of age, medullohlastoma is clacsified a% a primilive r~e~tmectc~rl~rmal tilttlor (PNET).

Atlas Link I-1 PPG-P3-018

PE

Labs

Imaging

Micro Pathology

Treatment

h 40-vex-old man cnmplains that "the whole room seems to be spinning" (VERTIGO) whilc also experiencing ringing in the ears

(TINNITUS) and nausea.

The p x t i ~ n t also curnplaitls of a sense of fullness in his cars ancl acids that his hearing has progressively diminished over the p;wt few years. TJis symptoms were initially unilateral but have now become bilateral. His ilInes5 has run a course of remissions and relapses. I-Ic clenies any weak~lpss nC the liml>f and Elas no I I ~ P I O T ~ of char di~cIiargc or trauma.

VS: normal. PE: anxious; neurologic exam normal: caloric teds

Iilaterally nor~nal.

Pure-tone a~id io~net ry reveals sensorineural bearing lass that is more markod for lower frequencies; Iolldness recl-~~itrnen t ~~rr.st.nt; short-increment senqi rivitr; index (S1ST) shows high scow; \QRL ncgativc.

MRJLT, hcad: normal (perrormed to rule out intel-nal auditnl-v canal pathulugv) .

Gross distention of the endolymphatic system ( E N D C > L ~ V ~ ~ T ~ ' ~ C

HYDROPS) .

No specific treatment; symptnmatic relief wit 11 vestibular suppressan$ diuretics, and low-soclium diet. Surgical intcrven- tiorr i~ ~ u n t ~ o v ~ r s i a l .

Mi.niPse1< is n disease of t11c inner car characterizerl by acute onset and recurrent attacks of vertigo; i t is often associated with nalivea and vr~~niting together with diminished hearing and tinuitus. iUeho11g.11 the exact etiology i s is~niknown. rnclolym- phar ic liydrops (clue to uxccss of tnrlol~mph in the scala media) has been linked tn ohsisuction of resorption, rtefective membrane exchange, and incrcasccl cnclol!mph inflow (secondary to alltrgy, vasomotor Fact nrs, or retair~ed sodium ant1 water).

M ~ N I ~ R E ' S D I S E A S E

Labs

Imaging

Gmss Pathology

Micro Pathology

Treatment

Discussion

'1 6O-yearold woman is seen with complaints nf having dirficulty walking and lwo episodes of involuntary hand jerking ( PARTUL SEIZL~RF,).

Her attendant I-rveals that over the past few months her memory has deteriorated. She has slow mencation and urinary incontinence. She is not diabetic 01- I~yperrensive.

Funduscopy reveals papilledema; tone of lower Iimhs incr~ased and strength rerIiiced (sP.~.%T~c P A K ~ P ~ R E S T S ) ; deep tendon reflexes exaggerated; hilateral Bahinski.

Rolltine laboratory te-stq normal.

XR, sk~t l l : hyperostosk of left parietal hone and sagittal suture. CT (with contrast): left parietal pamqagittal tumor, MR (gadolinium): intense tumor enhancement; d d extension and invasioii into superior sagittal inu us.

Irregular. firm, gitty mass arising superficidly. indenting and compressing braEn but not invading it.

Wherling pattern of meningotheliaI cells with regular, oval nuclei, indistinct cytoplasm, and psatnmoma bodies.

Surgical resection; radiation fur unresectahle cases.

Meningioma iq a primary intmct.ania1 neoplasm arising from cells of the arachnoid granulations; it is chilracterixed I l v slow p w t h , benign behavior, and expansile rather than inF1tra.tive growth. Common sites involved include the cerebra1 convexity, parasagirtal area (aq in this case), sphenoid wing, olfactory Forwe, cerchellopontine angle. fomtnrn magnum. and spinal cord. The tumor is usually solitary, is more cc~mrnon in women, and is found in middle and later ages. Multiple rneningiomas mav he found in patients with neurofihromatosi~ tvpe 2.

Atlas Links

ID/CC A 59-year-old whitc female presrnts with a severe, dull retro- orbital headache, vomiting, and diplopia.

HPI She has smoked two packs of ciprettes a day for 22 years and has hcen diagnosed with lung crancer.

PE VS: bradycardia; mild hypertension. PE: papilledema (due to

increased iritracranial pressure); right pupillary reflex abnor- mality in efferent pathway (due to right oculomotor nerve palsv) .

Imaging CT/MR: rouncl, discrete, ring-enhmchg lesion in right frontal lobe; surrounding vasogenic cdcma; shifting of midine structures to left (> I-cm shift considered severe).

Micro PathoIogy Biopw shnws small cell carcinoma.

Treatment Cansider ~urgical reseciion; radiation therapy; dexarnethasone (to control in tracranial pressure).

Discussion BIood-borne brain metastases comrnonlv occur in patients with systemic malignancy. Common primary cancers that rcsult in intrarranial metastasis are lung, breast, GI. and GU cancer and melanoma.

Atlas Link TTET3 PM-P3-021

7 METASTATIC B R A I N TUMOR

ID/CC h 20-year-old woman complains of recurrent. throbbing headaches associated wit11 prr~found nausea nnd light sensitivity.

HPI Shr tl;~.: l ~ d qirn ilar hearlac ties several limes each vcar sincc the orl~et or hel- met~~t~-lral p~riorls. The 11cadaches O C C I I ~ 011 one side of her hcad. She also report5 seelng ''flashing lights" like I igh~ni~lg r~loving across Ilel- lielcl of visiot~. Stress, sleepIessness, and anviety ~ ~ s l ~ a l l y precipitate thest. Iieadaches. Her mother suffcrh horn migrairlc l~carlachcs (positive fiirnily hisror y ) .

PE YS: r~orrnal. PE: fr~riduscopic exam and vis~ial field resting tlol-lnal: 11e11tc1logg.i~ rvarm ~~t,rmal.

Treatment Prup11:-lactic thurapv wilt1 avoidance of precipitatin~ factors and rlt-\~g< SU'CII as beta-l>lockers, ~riryclfc nrlticiepl-ttwi~nrs, or calcil~m cl ~ a i ~ n e l l1lockps5: ;ll>c~ri i v ~ t hvr.;~p\' r3~1ring acute attacks with NSAIDs, sumatriptan, crgotaminc, 01- transnasal butorphanol .

Discussion Migraine heacl;~che jq the second most common callce of primary headache ( t h e mmt cornrllclrl in !lie ITnirerl State5 is tension heaclachc). 111 thc United Statcs, an cstin~ated 17% 'of

women and 6% of mcn arc affcctetl t ~ y 111is dEsnl-iI~r, The hfilditdie is ch;lra~teristic;llly preceded hy rl prodmme and is

episodic, grarl~iiil iri onset, usuaIly unilateral. and most conl- 111orllv i t l rhe temporal area. PI-ecipirati 111: Carton may ir~cludc rtjerlwks, t'ns~ii~g. rmotional strcss, 3 r d foods cor~tai ning h-l-atnine, ~n.onr)~r~rli~trn gll~ianiate, nl- t~iu-ites. TIlr muse is unknown bllt appears tn invnlve variations in crrcl~ral hloc,rl flow and .icrotoi~crgic pathways.

IDJCC PI 36-year-old white L'etnaIc p;1j75 a n einergencv visit to 11cr opE1~11alrnologist hecarwe of loss of central vision and pain on movement of her left eye (duu to optic neuritis): she also presents with scanning spucch and intention tremor in the hands.

HPI Five years ago, she crnigat~d to rhr Unitecl Shtes l r sm Sweden. She has hecn s~fFrring from recurrent paresthesias in the hands, 5 arms. and Iegs; weakness in the l e ~ and arms: vertigo; and Madder urgency (mu1 tiplc unrulatocl nerirologic yrripcntris). c;1

I ler Carnily doctor rolrl her he had "3iv~reria" and recorn- -i

PE Dimirlishecl visual acuity; ccntral scotoma found on visual ficld charting; hyperemia and edema of lcft optic disk: defective affer- ctlt p ~ ~ p i l l a r ~ ~ t-eac~ion to light in lefi y e ( . ~ . Z R ( ; V S GUNK PcPIL);

paresis of medial rect~ls mtrscle on lateral conjugate gaze but not on comer-gence (BIWTE~LV. 1K'l'EKNtlr:LL~R OI~H'TI~ALMOPLEGL~) ;

mystagnus in a l~duct i i~g e y ; r lec~~icil . sen~ation r it n ning down back and into legs producecl t>y neck flexioi~ ( L F I E R M ~ X ' S SIGN):

leg spssticitv and irlcseasecl ckcp tendon reflexes.

Labs 1 .P: marked increase in CSF IgG concentration; prescrice of oligo.oclond bands in IgG region on CSF agzarose electrophoresis; CSF mthe~~wire normal. Ahnorma! visual, auditory, >and somatoseilsory evoked respoilses.

Imaging MR, brain: multiple discrele high T2 signal ah tlnrmarities in perivcntricular and ot t1t.r whi t r ulatter al-ras (uspcciaTIy corpus CalIosum).

Gross Pathology Pathologic haIlnlark 01 disorder coilsists of distiilctive small gray plaques of dernyelination prrsrnt in I N S wllite nlartel-; oplic ne111,itis.

Micro Pathology Demvelination ancl glicrqiq; lipirl-laden mac~aphagt-s.

Treatment Mainly suppol-tive; cor,tico.itc-roids: ACTI-I: azattlioprinc: cvclophorphamidr.

Discussion MuItiplu scPcrosis is a n idiopatllic deinyelinating rlisorder whosc courw is market1 hv intermittent remissions and exacerbations.

Atlas Link a 'CIT1-l PG-P3-023

?! MULTIPLE SCLEROSIS

ID/CC A 2,i-year-old Female has had marked weakness ancl drooping of the eyelids ( P ~ S I S ) in the evening Tor the past 4 werks; she does not experience anv weakness in the morning follo~ving a good night" sleep.

HPI She has also been suffering from double vision (nr~~.cn*m) at h e

end of rnch day.

PE Ptosis developq on sustainrd elevation of eyelids: hysphonia develop5 as patient is asked to narratc complainrs ;I[ lengih; wealmess of Forward flexion of head develops after repetitive resistance to force; patient could not maintain lzer lipper limb

in ahdlicted posiriorr for more than a minute.

Labs CIearat improvement in strength with edrophonium adminis- tration. EMC;: progressive decrement in voltage during repetitive, low-frequency stimulation of motor nerve. Positive serum titer of antibodies to acetylcholine receptors.

Treatment Xc~tylcholinestrra9e i nhihitors (pyridostigmine) ; prednisone; thymectamp; piasmapheresis.

Discusion Myixsthcnia paris is an autoimmune disease that is due to the rlevelnpmenr of specific antibodies to one or more acetylcholine receptor subunits, reducing the availabilip of acetvlcholine

ID/CC An obese 35-year-old male goes to a clinic because of dktal muscle weakness i n both upper and lower Iimbs and gradual diminution of vision.

HPI His faher suffered from a similar muscular weakness. The ~atient alsn suffers from mental retardation. 1 z-

m

PE Fmntal balding; typical facial wasting; bilateral cataracts; distal C 70

muscle weakness in both upper and lower limbs; difficulty 0 r

releasing grip after h audshake; percussion over tongue and a 0 < thenar eminence reveals myotonia: mildly reduced deep tendon

reflexes; normal sensory exam; moderately atrophic testicles: cqrdnovarus deformity of both feet.

Labs Decreased plasma IgG. EMG: myopathic potentials; myotonia.

Imaging ECG: nonspecific ST-T changes.

Micro bthotogy Muscle biopy reveals iuternal nuclei (nuclei in center of the fiber rather than in periphery), type I fiber atrophy, and ring fibers.

Treatment Phen~loin: carbarnwepine; quinidine; procainamide; acetazw lamide; surgery required to correct Foot deformities.

Discussion The most cornman form of musc~~lar dystrophy among whites,

myotonic dystrophy i q transmitted as an autosomal-dominant trait. It is associated with a genetic defect that encodes myotonh protein kiuase; the myotonic dystrophy gene locus has been

MYOTONIC DYSTROPHY

ID/CC A 5-year-old male is rrfkrred 10 ;I ~perialist hy his physician for cwli~;ltir)n nf arl abdominal mass and a rcccn tly no~iced IF ft-sided o r b i d proptosis.

HPI His parcnts complain of cveigl~ t low, poor feeding. and a con tin- uous lou,-grxlc fcvcr for the past few months.

PE

Labs

Imaging

Gross Pathology

Micro Pathology

Treatment

Discussion

Atlas Links

PV.: mar-ked cachexia: Icft-sided orbital proplnsis and ecchv- mores; large, smooth intra-abdomiaal mass palpable.

XVarkcd elevation of urinary catecholamines and rnel;tholil~s var~illyltnai~d~lir acid (\%$A) n i ~ d honiovanillic acid (m'h).

CT, a hdon~eii: intra-abdominal mass arising from and obliterating left adrenal $and. Nut (hune wan) : rnrtarratic lvtic lesion in left ~~-11ira.11 rexitrn o f sk111l.

Sr~lirl, ro~xnrl saft-tumor mass obli tcrat i~~g left adrenal gland; gray on cut surface ~I lo~ving extenxivc hemorrhage a tic1 nprr-osis with c v s t formntion.

Anaplastic, small, rcmnd-to-uval li yperclir-c~waric cells uvith scant

cytoplasm in sheets aild at placcs fornriug Homer-Wright pseudorosettes; fmv ganglion ceIls wen: c l~c t rn l l microscopy reveal< pt-esrnce or neurosecretory granules.

Srirfiical rc-st-ction: clnei~iotherapy with ~clophosphamicle and ;idriarnvcin.

h'currjhlastonia is a prirnarv malignant nenplasm that ariscs from irn~narut-e cellv nf thr adrenal meclulla and sccretet; catechnla~nine~; i t usually nrcul-5 in children under thc age of .5 and presents with a n ahdomin;~l mas\. Ke~rrol>lasrornas Innst cornrr- only ni.lginar~ in the adrenal glands hut ma!! also arise in the retmprrironpal sympathetic ganglia, pervis, neclc, r )l- posterior rr-ieciiasti~~um. Hutcl~inson'..; rle11r.c )l>laqtoma present5 with uxlctlsi~r. skrill arirl orki~al melaqtaws that prnducc cxophtl~almos: rnctaqtases to lynlpll nodes. Iiver, lung, aiid hone are mmmon.

I D j C C A 60-vear-olcl male is seen ly a ~~eurn leg i s l ror an evaluation of deteriorating cognitive skills.

HPI Over. the past Few wreks, the patient has sta~~ecl in fled and has 11itcl urinary and b o ~ ~ e l inron~inence.

PE (:ogriitinn impairccl; irnpaircd ant hi il;xiion tvithnut eviclencc of z rn

primary motor; sensory, or cetehetlar rlyf~mction ( G ~ ~ C m

. L P R ~ Y ~ . ~ ) ; dcep ~ P I I C I O ~ > reflexes in tart; pupils equal, rourid, and 0 r

react ivr lo light anrl arrommoclation; plant;trq l~ililterally Ilexor; 0 t, <

h~nd.rls cloes not reveal ;my papilletfern;~.

Labs Lr: nurmal opening pressure. I,ah parameters wi tl~iri rior~nal lirnits.

Imaging CT: ventricular enlargement with relatively little cortical atrophy. N~zc, c i s ~ e r n o ~ ~ a p h v : persistent a c t i v i ~ of radionuclide in lateral vun~riclcs after 48 hours (characfcristic OF normal pressure hyclmcephal~~s).

Treatment Tlisertir>n of a vcnri-ictiloperitoneal slrunt.

Discussion P s ~ ~ ~ d n b ~ ~ t h x t - is due to bilatcral dvsfunctiori of the corti- cohulhar tczc ts. In acldirio~i lo clysphagia, ~lpsarthria. and 11yer- art i ~ c . gag r e f l ~ x e ~ . patients may ecxpcsicnce episodes of

spnntxnroils crying o r Inlr~hter. In most patic~lts, the c a w e o f

nn1.1nal pl-rssure hvclrocephalus is ncl t k r ~ o ~ m , all l in~~gl t it may Fc~llo~u rl .;l~harachnoitl l~ernori.h;+ge ot- tneningitis (5ometimcs years later). The value s f its carlv diagnosis lirs in the k ~ c ~ that i i

is a treatable dementia.

F*s" NORMAL PRESSURE HYDROCEPHALUS

-

ID/CC ;4 ilGyear-old white male complains to his inwrniqt of increas- ingI~. severe headaches upon awakening of a few monthq' duration: the headaches persist r hr.o~~gt~out the afternoon and are mild in t h e evenings.

HPE U%iIc in the doctor's office, the patient suffers a seizure and i s

brought to the crncrgcncy room.

PE Funduscopy reveals papilledema.

Labs Normal.

Imaging CT/MR: large frontal lobe mass with focal nodular calcifications,

Cross Pathology Calcified qstic tumor with gelatinous consistency and areas oF necrosis and hemorrhage.

Micro Pathology Tumor has few anapIadc features; regular cells aligned smoothly; spherical ri~lctei with finely ~ r a n u l a r chromatin, calcifica~ions, and increased vascularip with areas of intraru- mi~ral Irleeding.

Treatment SurgicaE resection/chemothcrapy with radiation.

Discussion LlsuaIIy low grade hirt occasionally anaplastic. olignden- drogIiomas resemble astl-ocytomas in most respects but grow mnre slowly and are mare sensitive to chemotherapy: calcification i s nored in 90% o f cases.

OLIGODENDROGLIOMA .*

IO/CC A 4Cyear-old maIe with a history of insulin-dependent diabetes meilitus (IDDM) presents with tingling, numbness, b&g, and aching in the lower leg and feet.

HPI The discomfort is paatict~larly promillent at night and is often relieved by walkmg, A hoop over the feet to prevent contact with

:- bedclothes is often helpful. The patient takes in.& irregmIarly.

L A

PE Mild weakness; mild distal sensory loss md loss of position and vibration sense in both legs; hilateraIlv reducecl ankle and knee g GI jerks. -<

Labs Nerve conduction velociries slowed. EMG: features of denerva- tion. EIuvated glycosylated hemoglobin levels indicate poor 1~Iood sugar control.

Treatment NSMDs and carbarnaxepine erfective in reducing cliscomfort; good glyccmic control to sIow progression or neuropathy.

Atlas l ink EEFTF MC-259

PERIPHERAL NEUROPATHY-DIABETIC

ID/CC A 35-vear-old m,~lc who has 1>rc11 diagnowd wii h rn~lltiple sclerosis vivi ts his phvvician will1 aotnptainr 5 01' difficulty swallowing (DYSFI-IAC.IA) a tld naqal rcpgiiation or Innd.

HPI Six mon (11s ago h p sz~l'fer~rl an attack or retruhu1lx11- neuritiq: 2 inonthq go h e dcvclopcd spastic weakness of both lower limbs.

PE Sperch is n1onc)torloti s, slliri-erl, and high-pitched ( " D C I N . ~ ~ Ut.c7s" n ~ ~ z ~ ~ r r n r . 4 ) ; rlrihhles from m o ~ ~ t h ; cannot prc~tr-udt. Tiis

tonpe, which lies on the floor of the mouth and is small and spastic: pala1 a1 mr)veir~rnin al ,~eut: jaw jcrk exaggcratvrl; pal it-111

is crr~olionallv labi le.

Imaging MR. I~rain: multiplc Coca1 wI~i~c-matter plaq I ~ r s .

Treatment Gen~1-al rnanagem~n t of ni~~lt iple ~ c l c r o ~ i s ; no spccific rreattnenl availnhl~.

Discu~sTon Tkc common cau~cs (11. pseit(lol~~~lt~ar palsy inch~rle hilnternl cel.'l>rovaucuI;~~. accicleil t s ilivnlving thr internal capsule, niolor ilt=rlrc>n disease. and n l r ~ l t i p l ~ srlel-o~is.

PSEUDOBULBAR PALSY

ID/CC A 40-!car-old male dies ~ h n r t l v afltfr being brnught to the emrrgency room with the Lcrnost severe headache of his life."

HPI His father died of chronic renal failure at the aKe of 45.

Imaging CT, Ilcad: hyperdense blood in cistcrns and sulci. Z' - m

Gross Pathology Brain rc-veals staining or inferior surfices of hrainstetn, cerebel- C n 0

l11111, ancl cercbral hernispt~~res w i l l 1 fresh blood duc to congeni- , ta1 herry anellrr:.;tn r.crpture: both kidneys reveat polycysric 0

G1 < cllangu~: multiple cycrs ~ p r t i ill Iivcr.

Discussion In t hi? cace, hemnrrh age was c a i ~ ~ e d 11y a r~rpti~sed ill tracranial mneurysm in n paticn t with autosornal-dominant poIycy~tic kidney disease. Other causrs of .itibarachnoid hemorrhage inclurle AV maIformatinn and trauma.

Atlas Link q , J T T PG-P3-031

SUBARACHNOID HEMORRHAGE

I D A ~ O - ~ P ~ T - - O I C I Mack male complains of constant bifmntal headache and bIurred vision uf 3 wceks' dnration.

HPI He has had mild intermittent frontal headaches for the past 8 months a11d has become irritable; for the past month h e tias

hecn extremefy drowsy and ofteti sleeps for :3O hours at a time.

Ten months ago, he fell from a moving vehicle and lacerated his scalp.

PE Kiateral papiflederna; dilated left pupil: right spmlic hernipare- sis: deep lendon reflexes nn right ~ i d e are hrisk; right-sided Babinski; tlo tneningral rigrl~ present.

Labs LP con trair~rlicatecF cIiw to raised irr I rarranial prexsure.

Imaging IT, head: hyperdense crescentic exka-axial fluid colle&ans (early); hypoclense fluid collection with thick membranes (late).

Gross Pathology Old blood encaqed in thick adherent h r n w membranes.

Micro Pathology OI ILY~ rnemhrane composed of panulation and fibrotis tissrie

with hcmosiderin; inner membrane shows fibrouq tissue only.

Treatment Surgical drai nagc hematoma.

Disc~ssion S ~ i h d ~ ~ r a ! hcmatoma is a traumatic Iesion charartel-izect by acc~trnulalion ofblor)d beween the ilura and arachnoid. It is ca~lsed by laceration of the bridging veins and results in displacement of the brain and possible cerebd herniations.

Atlas Link [Tr;-TlZ PG-P3-032

SUBDURAL HEMATOMA

ID/CC A .f&year-oId man is referred to a ne~irologist because af progressive anesthesia and weakness of both arms, occipital headaches, and a stiff gait.

HPI He has n o history of significant trauma it1 thr past.

PE No motor cleficits: lack of pain and temperature sensation in Z rn

both hands and arms {due spit~othalamic tract involvernenl) c m 0 but preserved position and tactile smsation {dorsal coltirnns un- 0

it~valved and pmprioceptive sensation sparttcl); t~nitnpairerl pain ,r, < and temperature sensation below arms: thenar muscles of both

hands atrophied; areflexia in both upper limbs; brisk deep ten-

don reflexes i11 both lower linths.

Labs Normal.

Imaging MR/CT, spine: cystic dilatation within c e n d cervical c o d .

Gross %thology Spinal cord shaws central cavitation in longi~udinal. ancl cleftlikc Fashion.

Micro Pathology Hydromvelia i s lined hv epet~clvmal tissue; q7ingomyeIia i s not.

Treatment Surgical shunting.

Discuss$on Syringomvelia mav he primary (associated with AmoW-Chjari malformation) or acquired (post-traumatic, pclqr it~flarnmamry, tumor-associated) .

SYRINGOMYELIA

ID/CC A 7'9-year-old white woman complaiiis or a throbbing, unilateral headache ~ha l is innst severe a r o ~ m d her forclleatl aucl temples.

HPI She has hacl rccurrenl bouts of fever over the past year atid also complait~r of malaise and muscIe aches. She reports sonle weight loss and occa'iiar~al vision problems in her right eve. She also reporis pain in her mandible when she is eating (J,IW

C.L\C7D IC:4TTOK ) .

PE T7S: f e ~ ' ~ r , PE: nedlllar enlargement of temporal artery with tenderness.

Labs CRC: nnrnlal 14%C count; mild anemia. Markedly elevated FSR, l~suallv > 100 mm/hot~l:

Gross 'Pathology SwoIlen. cordlilie, ~egi~ientallv rloclular temporal artery.

Micro Pathology Granulomatous in tlarn ina tory infilt~ilr uf rnedia and zidven~i ria on temporal artery biopsy: rl-agrnentatior~ of in te t-nal elastic

I lamina nith rnufritzucleatcd ~ ' 1 1 ~ and fibrotic p;itches.

Treatment Steroids should Ise starr~cl empirically hcfore hiopsv coilfirma- tion to prevent blindness.

Discussion Temporal arteriris i s the inoqt common vasculitis in the United Statcs; it frerll~rntlv coexists with polymyalgia rheumatics and carries a risk of ipsilareral blindness clue 50 thrombosis of the ophthalmic artery. Diagnosis and treatment nrr based nn clini- raI grounds, since biopsy

Atlas Link i:.l_l PPH-P3-034

TEMPORAL A R T E R I T I S (GIANT

is positive in only 60% nf cases.

CELL A R T E R I T I S )

lD/CC hri 18-yea1--0lrl man present5 with headache, ataxia, and pmges- sive Fosu of visiatl.

HPf His father died of mrtaslaric bilateral renal cell crarcinoma at a rclativcly yo~mg age.

PE Cerebellar ataxia; n p t a p u s : past-point in^ and inability to z m

perform rapid alternating movements ( u ~ \ u ~ m c ~ c ~ o ~ m . s ~ , ~ } ; C w 0 liu~rh~scnpir rxam rmeals prescncc of retinal hemangiornaq and ,

morlerale papillede~na (due to increased intmcranii~l pressure). Q GI <

Labs UA: normal (hemnluria may qignal rend cell carcinoma).

Imaging C,T/MR, head: cerebellar solid/cystic lesion with enhancing mural noduIe. CT, ~~hdomen: renal, hepatic, and pancreatic qs".

Gross Pathology Nemangioblastornas of ccrchcIIum and retina; iutnor occasionally located in medulla ur ccrvical spinal coi-d.

Treatment Sltrgical removal oi' t unior; photocoap~lation for treaunen t of

retinal lesions.

Discussien I'on TIippcl-Lindi~ri disease i s ;I rare autosomd-dorninant 1 3 e ~ t t = o ~ ~ t ~ i t ~ t o u s dvsplasia. The gene has lxrn linked to the I-af-1 oncogene on chromvsomc 3 arid has a variable penetrance and dclaycd uxpressir~r~. T h e conditiotl is asqociated with renal cell carcinoma tfia~ is ofi e t ~ mt~ltifocal or bilateral.

A , VON HIPPEL-LINDAU DISEASE

ID/CC A 45-year+old Hispanic feniale is brought tn the grmecologst for a n evaluation of a gross difference in the size of her bras& of recell t origin.

HPI Her medical history is unremarkable. Despite the recent i t l -

crcase in thc size of her right breast, she does not feel any pain and feels only a sensation of fullness.

PE Very large maw with firm, "wooden-log" consistency involring alrnost all of right breast, tnaking it ~ M T ~ C P the size of opposite I~rena: mobile mass: appears well circumscribed; collateral bluish veins seen on skin along with striae; no peau rlbrange appearance; no nipple retraction, axillary lyrnphnrlenopatl. o r hcpalori~ugaly; opposite Ilreast normal.

Imaging US: largc, smooth m~iltilohulated mass.

Gross Pathology Largt tumor with ntirnerotis cystic p a c e s nn cut section of strorna, producing recesses and lanpjtudinal openings and causing a leaflike (phyllodes) appearance.

Micro Pathology Ahlmclance of normal-looking ducts, acini , and F t r n n m 14th no s i w s of cellular aryia and low mitotic index.

Treatment Sirnplc excision.

Discussion :\ less common I~enign tllrnol- nf'hreast that is also known as fiant fihroadcnema, qstusarcoma phyllodes is a bulky tumor lhal, ill though u s ~ ~ a l i v benign histolngicallv, may recur following excisinrl anrl sr~~netinres l~nrlergoes malipan t degeneration (5% to 10%). It rarely metastasizes to lymph node.; or rlistant sites.

Atlas Link W a T l PM-P3-036

BREAST-CYSTOSARCOMA PHYLLODES

ID/CC ,427-year-oldwomanwho isactivebtrainingforamarathon notes a painful lump in the upper outer quadrant of her right breast of' 2 davs' dura-rion.

HPI She has no history of fever and no known family history of breast cancer.

'PE Rehction af ovalying skin in upper outer quadrant oC right breast; indurated lesion the size of a lemon in same area; axil- Iarv lymph nodes not palpable.

Imaging Manlrno: irregular mass with focal areas of eggshell calcification.

Gross Pathology Yellowish, fat? fluid an aspiration.

Micro PathoIogy Excisional biopsy shows localized area of granulation tissue within which are numerous lipid-laden macrophages subjacent to necrotic fat cells.

Treatment Nn ocher active management required.

Discussion Fat necrosis of the breast is a unilateral localized process associ-

ated with tmuma, breast biopy, reduction mammoplastv, and radiation. It is easily confused with cancer due to induration, fibrosis. clystrnphic calcification, and retraction of overlying skin: the key distinction is the presence of pain.

BREAST-FAT NECROSIS

ID/CC 82-vuai.-old woman presenls with painful bilateral breast masses.

HPI The pain i s cyclic in nature and increases in her premenstrual phaw, at which time the masses enlararge rapidly and then shrink. Slw Fcc l~ that both hrcasts arc ~lodulai- and is corioerr~ed h a t

\lit= may ll;ivt' rancer,

PE Mildlv tcndcr mils< palpahlr ill tipper anti outer- quadrant of rigFi r ;uirI left I > ~ P ~ s I ; 11ol h breasts nodular with multiple thickened areas; no change5 in overlying skin or nipple nuted

(VS. breast canccr); n o axillary I~inphadu~~opath): f o t ~ ~ ~ d .

Labs Acpiration frnm hreast mass rereal? nonl>loodv fluid; mass &sap pears completely after aspiration.

Imaging Mamma: nodularitv and henip1 calcificatioiis, n o malipiat~t tpar~lres.

Gross Pathology C:ysis nf various 47es rarlging from trlit-I-o~copic to sevrra1 mil- limetcrs s~~rraunclcrl by drnse fil~rotic tisst~c; contains clear or l ~ r < ~ ~ v r i fl~ii~l.

Micro Pathology PI-olif cration of x i n i in lobules ( s c ~ ~ ~ o m r . ~nr:riosrs).

Treatment Kt=nss~lr.ance ant1 symptomatic management.

Discussion Fihrncvsrir r l i seas~ of r h e hi.ea$r i q cnnlmon in wr~rnen Iwtween the age? of 3-5 a n d 55 and carries at1 it1cre:lsed risk nf ~IIMS~VC

brca~r cancer in patients with epithelial hypcrphsia and ahpia. Fi'ihrocvrt ir ci~ar~gc.; mav result from ho~-njonu imhi1l;lnc~~ with either an excess of e~tsngen or a rleficienr~ of prt)gesteronc.

7 BREAST-FIBROCYSTIC D ISEASE

I'D/CC h .59-vear-ald white female comes to her ramily docLor beca~ise of a presumed "jnfuc tion" in her right breast: she cornplait~s of

pain and swelling.

HPI Her history is unremarkable.

PE VS: no fever or other systemic sign of infection. PE: right breast warm. rock-hard, and swellen with no areas of fluctuation; one- third of breast erythernatops with shinv overI~i~g skin havi tlg peau d'orange appearance; painful to touch and pscsstire: several axillary lymph nodes enlarged and firm; qome coalescent.

Labs Rolltine lab work normal.

Micro %thology Large ~pheroidal cells and fine stroma infritrared 'by lympho- cytewn hreast skin biopsy; lymphalic blood ves~els occluded hy nlnlor cells.

Treatment Chemotherapy and radiotherapy, hormone therapy; poor prognosis.

Discussion Inflarnmatorv carcinoma OF thc breast is defined as hreast cancer with angiolymphatic spread: it is characterized by a nlalig- nant course wich earlv and widespread rnelaslases. Perform skin biopsy in patients dia<gnosed wit13 breast infection who do nnt respond promptly to an tihiotic treatment.

Atlas Link PTT'lT Pi-P3-039

BREAST-INFLAMMATORY CARCINOMA

ID/CC X 35-year+Id female r~isheq to the emergency room and waits to

sre a doctor hecati~e she i~ c~ncerned about a bloody nipple discharge that she discovered this morning.

HPI She exercises, is very health conscious. and alwavs has ~ a f e sex.

PE Palparion arnund Iefi nipple reveals blood coming from one of the duct openings and a small, soft lump heneatlr areola; no breast masses or axillary lymphadenopathy.

Imaging Marnrno: negative. Ductographv: dilated ducr with intraluminal filling defect.

Gross Pat h o l o a Epi theliaI papillary growth with fibrotic cornponrlits, characier- istically located within a lactiferous duct.

Micro Pathology N o cellular awia nr anaplastic changes on spccirnen of kfoody discharge; only blood intermixed with foamy macrophages and hcnign ductal epi tllclium with fibrova~c~zlar core.

Treatment Surgical resection of lactiferous duct.

Discussion Papilloma of the breast is a benign proliferatiori of ductal epithelial tissue and is, the most common cause of serous/ sang1"neous disc11arg.e.

BREAST-INTRADUCTAL PAPILLOMA

Imaging

Gross Pathology

Micro Pathology

Treatment

Discussion

Atlas Link

A 4E-year-old w o r n presen 1s a palpable mass in the left breast.

The patient has been adrnitred to the hospital to obtain an excisional biopsy and for planning Eurther management. The patient's older sister recently died of metastatic breast cancer.

Ideft hreast mass on palpation; nipples normally located without evidence of retraction; no evidence of axillary lymphadenopathy or hepatomegaly.

Mammo: frequently normal or an asymmetric density without deliilabte margins.

Firm, white, irl-eplarlv shaped 3-cm mass was removed from each breast.

Histologic sections reveal terminal lobulus distend~d by inter- mediate-sized cells with scant mitotic activity: neoplastic cells infiltrate the stroma with individual neoplastic culls in a single file (NIAN FII.E PAITEKV) that surrol~nds the terminal lobule in a target-appearing fashion.

Modified radical mastectomy with axillarv lymph node sam- pling; radiotherapy; adjuvant chemotherapy if required. Frequent mammopnphic surveillance is needed owing to the high incidence of a second primary in the same or opposite 'breast.

Infiltrating lobular carcinoma is the most common malignancy of the terminal lobule. I t accounts for 10%) tto 13% of all hreast cancers.

BREAST-LOBULAR CARCINOMA

ID/CC h G8-yc:lr-old whi t t woman vivits her drrrnalologist because of a long-srandiug itching, ppainIess, scaling, and oozing erythemataus rash over her I-igh~ nipple.

HPI Mrr- first menstrual period started at age 9. and she has never b e ~ n married or had children; her menopause started at age 56.

PE Nipple c>n right breast retracted and appears eczematous with redness, some edema. and desquamation; oozing of yellnwish ~xltc1aie: paitlless leF~ axillat-y lymphadenopathy: no hc- patnrnegaly o r lumps in oppositc l~rcast.

Gross Pathology Ductal carcirloma wit11 exlension to overlying skin.

Micro Pathology Charactrristic cell5 are scattered in he epidermis and are rnucin pc~~ i t ivr and have l a r ~ e nuclei and abundant, pale-staining cytoplasm (P.M:ET's C:ELLS).

Treatment MarIified raclical mastectomy with a x i l l a ~ lymph node dissec- {inn and tarnosifen tlaerapv.

Discussion Pagel's carcinoma is a scaly skin leqinn in the areola and nipple arking from ductal adenocarcinoma ~ i t h i n suharucllar excretory ducts and progressing uutrzrard.

Atlas Link I"'T.r PG-P3-042

7 B R E A S T P A G E T ' S DISEASE

ID/CC ,I 52-yeat-ald urlrnar.rled white nulliparous female smokcr with early menarche prrrenrg with a painless lump in her right breaft.

HPI Thc patient has a history of atypical hyperplaqia of the right I~reast. I-Icr mother died of breast cancer at 4(j years oTagc.

PE rZ 3-rm. Fixed, hard, and nontender mass in upper outer quadrant or right breast: retraction of overlying skin a ~ d nipple; no

nipple discharge: palpable axillary lymph nodes on right side.

Labs R o u t i n e lab work rlorinal: nnrmal aIkzline phosphamc (no l~orie rne~aqiaws).

t, < z Imaging Miammo: spicdated mass with architectr~ral distortion and multi- ;

ple clustered pleomorphic rnicrocalcifications; skin th ickening 0 r

and 1 . ~ 1 rilrtini~. CXR: no evidence of mutastasis. 0 0 <

Gross Pathology IIard. in-egltlar whitish mass wilh granules of calcification and focal yellow al-ras of 11ecrosis. Profounrl fibrosis with induration in sZrtlma (DESMOPLXSTI(: RT;A(TIO~') .

Micro Pathology FNA large pleomnrphic cells arrxtlged in glaildq, cords, nests, and shcors in d e n ~ e fihrous stroma: mmor cells estrogen and progesterone receptor negative by Ilow q.tomelry. Core biopsy: anaplastic cells wi (11 Iligh mitotic index ronsistcnt with infiltrat- in3 dtictal arlenocarcinoma, riot otherwisr sprcifirrf .

Treatment Surgerv; tarno~ifen (For e~trogcn-rcccp~flr-positive tumors in prernenopa~~sal women); aclj~ivan r che~nnthei-apy with posrihle Ron? marrow tfi~~lxplan ration: radiotlirrapy.

Discussion tnfiltratin~ ductal hreast carcitiow~a is rhe most common type of breast cancer. Approximately o n e in ninc women in the Unitecl States will develop breast cancer. Risk factors inclirde family h i s tory, early menarche, late menopawe, obesity, exogenous estrogens, atypical hyperplasia of breast, and breast cancer in the opposite breast.

Atlas Link PM-P3-043

3 BREAST CARCINOMA

ID/CC A 25year-old black female visits her family doclor for a painless right breast lump that she discovered on selfexamination; she is othel-wise a.symptomatic.

HFI Her medical history is unremarkahlc.

PE Small, encapsulated, well4efmed, rubbery, freely movable 3cm maTs in right Iower quadrant of right breast; no overlying skin changes; no nipple retraction: no Iyn~phadenopathy: other

breast nor-mal.

Labs All rorltin~ lab work normal.

Imaging Marnrno: oval low-densiq lesion with smoath margins; "popcorn calcifications" seen with dcgcneration.

Gross Pathology Solid mass; no areas of necrosis or hemorrhage (centrat myxoid degenerarion in older patienb).

Micro Pathology GlancluIar structures with ductal and stromal proliferation with no cellular atypia.

Treatment Surgical excision.

Discussion Fihroadenoma ir; the most common benign breast hunor in young women; i t sometimes enlarges during pregnancy or normal menstrual cvcles.

Atlas Link PEETE PM-P3-044

7 BREAST FIBROADENOMA

ID/CC A '2'-velar-old female presents with an abnormal cervical Pap smear.

HPI S11r 1 1 ~ s no history of irregular menstrt ~ a l bleeding. postcoital bleeding, intcrmcnstrual bleeding, or vaginal discharge. She tlclivcred her first baby at the age or 18 ancl l-lns had multiple sexual partners.

rmaging Colposcopy reveals a sl~spicinus area Crow which a hiopfy is taken.

Micro Pathology Biopsv xhcltvq loss nT nnrmal oricn tation uf srlua~nour rplls; 4 3

atvpical cells qeen with winkled nuclei ar~rl perinuclear haIos -< ;Z

involving fulI tl~ickncss of squarrlous epitheli~lm; bascrncnt rn 0

membrane intact. o r a c 3

Treatment Coile biopsy of area with regt~litr follow-up examinations. <

Discussion Ccrvical dvsplmia is a precursor of crrvical squamous ccll carci- Izrlrna; i r i s as~nciatecl with infectjon with human papiuomavkus (HPV) types 16, 18, and 31.


* CERVICAL CARCINOMA (IN SITV)

- - - -- -

ID/CC A :'9-vear-olcl Vietnamese female \;isi~s her family doctor beon! we

clf pr.r >tracictl nawsea, vaginal bleeding, dyspnea, and hemop-is.

HPI Her 11 istorp t-ewalc one prcvious nnrnmal gestiitiorl and one spnntaneou~ abortir)tl as twll ar a dilataticli~ x~zd clttrttage 4 months ago for a hydatidiforrn mole.

PE Vaginal cr;amin:~lir)ri wi ( 1 I s p r c l ~ l IIIII i.c.veals bluish-red vasa11ar tumor and enlarged uterus; adnexa nnrl ovaries normal.

Tabs Markedly elevated Fcruln arll-l ~~rinal-y hCG levels.

Imaging CXR: mllltiple metaqtatic nodules ( "c:\rut)~ r 3 . k ~ ' ' set-UKD~WS

1 r5!oh-s).

Micro Pathology Euagger;uerl 11-np h a h l a ~ ! ic (cvtntroplir~l~l~stic and ~yncytiotr.cl- phr>I~lastic) tissric proli r ~ r ' i l l i c ~ l l will1 et~~lotiit't~~ial penetration: celltil;~r ;lt!.pin anrl h e m a t o g e ~ p h a t i c 5prcacl.

Treatment Chrmo~h~iapv: fi~llow-11p with serial senurn hCG Ievel~.

Discussion C:I~o~~ioca~-rir~oina i s ;I m;~ligtianr ~ e ~ i . t t i ~ ) r ~ a l tl lnlor hat inay clewlop d ~ ~ r i n g nnr~nal prcgnaurl: i ~ f t ~ l - cxacuation o f hvtlatidi- form molc. nr aftcr pre~icws sporat:tnrous ahor-tiorls.

Atlas Link U .EI J PM-P3-046

C H O R I O C A R C I N O M A

I D JCC A 5%year+ld Hispanic multipvida in her 20th week of pregnancy comes to the gynecologist's office complaining of a mas9 in her abdomen.

HPI She i s pregnant far the fifth time. She has had no prior abortions or Gsections.

PE ITS: RF normal. PE: no edema; uterus currecl height for gestational age (at level of umbilicus) ; ill-defined, painless, nonmov- able mrmss 5 cm Frrrm midline on rne.iogastriutn; skin riot red or warm: no exudate; no fluctuation; mass seems to disappear on contraction of rectus muscle.

Labs Koutinc lab work on hlond, urine, and stool normal.

Imaging CT JMR, nbdotnen: circumscribed mass.

Gross Pathology Coarselv trahecula ted tilmor resembling scar t isslle; appears to

arise From musmloaponeurotic waU.

Micro Pathalogy Elongated, ~pinclie-shaped cells: tibrnl>lastic process; no evi- ~Ietlce or atypical mitoses otl hiopslj.

Treatment Surgical excision: r d i otherapy.

Discussion t'i type nffibromatosis of the anterior abdominal wall in women. desinoid tumor is associated with previous trauma, multiple pregnancies. and Gnrdner's syndrome. 1 t frequently recurs after excision.

DESMOTD TUMOR ""A

ID/CC ,4 t Gyear-old girl is seen with complaint5 of colicky lower abdominal pain together wi I 11 nausea m d vomiting aqsociated with the onset of menses.

HPI She achievecf menarche at 14. and her initial cvcles were irregular lltit painless (dr~c to anovulatinn). She does nor complain of any irregularity or excewive Ideeding arid has ncr ~~rinarv complaints or diarrhea.

PE Abdominal exam normal; p ~ e c o l o f i c cxatn reveals h l ~ ~ d - stained pad; pelvic exam not performed d11u to inlac t hymcn; rerial exan1 normal.

Labs Ko~rtine lab parameters normal.

Treatment Symptomatic relief ui th prostaglandin qmthetase inhibitors w ~ c h as ~nefenamic acid or naproxen sudii~m; inlractahle syr1plorn.s may require suppression of ovldation using combined cstro- gen/progcsterorte or progestogens.

Discussion Ptimary dysmenorrhea is defined as painf1d periods I r which n o organic o r psychological cause can be found; the pail1 is calicky and uqually hegins shortly after or at the oilset of menses. I t is thought to he due to an increasc in the procluction nf pmstxglandins. leading co uterine vasoconstriction m d painl'i~l contxactiorls. Occurring only during ovulatory cycles, primary dysnienol-rhea is most com~nonly hund in women under the age of 20.

DYSMENORRHEA 2

IDJCC A 60-?car-old obese, nulliparous white female preqencr with interrnittcnt postmenopausal vagina! bleeding of 3 months' dr ira tion.

HPI Shc- has a I~istol-y of diabetes, hypertension, and infertility with polycystic ovaries; menopause began at 56 years of age.

PE Utems not enlarged on himan~tal paIpation.

Labs CBC: rnilcl anemia. Stool and urine I P F I ~ rvithin normal limits.

Imaging US. pelvis: thickening of endometrial qtripe.

Gross Pathology Hysceroscopic biopsv performed wi I h rlilatation and curettage; fungating mass iisualized.

Micro Pathology Adenacarclnama.

Treatment Radiation ttiet-apv; hvstrr-eeclotny.

Discwssian Enctometr-ial carcinoma is an estrogen-dependent cancer and is an important differential d ia~gus is of pn51tn"erlopausaf bleeding.

ENDOMETRIAL CARCINOMA

--

HPI

PE

Imaging

Gross Pathology

Micro Pathology

Treatment

Discussion

2'i-yeal-+ld white TcrnaEe is aclrnitted to the infertility clinic for evaluatinn or her inability to conceive: she also complai~ls of pain during intercourse ( D W ~ ~ ~ I K E I ~ N 1,h) a11 cl pain during menses ( II\TMEUC)RRI TEA).

She i s nulligravida. She arlmiu to having rectal pain during menstruation: she also complains or having a11 abundant rnenstn~al period (M ENORKHAGIA OK HYPERMENORRHEA).

Bluish spots in posterior fornix on vujnl-il \ p e c z ~ h ~ m rxam; on

I>imauu;~l exam, fixed, tender bilateral ovarian masses palpable d t irirlg rneu.;truation; induration in pouch of Doudas with multiple small nodules palpable through posterior f'ilrnix.

Lapdi-uwcql-'): pelvis: ova t-ies adhere tv htnad liqnment and show reuac tion ancl scarring in addition to endornefriomas, with tlerlse pei-itl~hal and p~riovarian adhesions and thickening of uterosacraf ligaments. US, prlvis: rloi~specific c ~ u t i c enlat-gentent of'ovaries.

I\l-own iqh nod~~les on titerosacral livments, ovarics, and pouch of Douglas.

Litparuscopic biopw of affected arms shows ncld~tlrr to consist of r>therwisr normal-looking. ii~~ictionir~g enrlomet rial glands.

Oral contraceptives, progestogens. danazul, G n W . sur-gical rernoval/coag,rl~lazion of lesions.

Enclomerl-iosis r r fP r~ to endorn~rl-ial tis~ue that is present out-

qidr the uter11~ and produccs synpforns that varv with Ioci~lion. ' i ido~ne trial implari rs (endninetr~r ]inas or Lhchocolate cysts'')

most frrquen tly involve both ovaries.

ID/CC A 25-year-old Filipina in h e r 20th week of p r e p a n y prcscnts wirh vaginal bleeding but no pain.

HPI Slle h a u I ~ c c n frcling inordinatdv nauseated and Isar suffered fi orrr 1.i n ~ i n g in her- ears.

PE 1'5: rnor l~~ate liyprl-tensinn (BP 150/95). Uterus large for gestational agc (three finger hreadrh~ above r~mhilicus) ; lowcr e r t r ~ m i e 2+ nonpitting edema.

Labs Markedly incseaqed PhCG. LA: proteinuria but 110 casts seen on

micl-osctlpir. exain. F:Ievar ed I>lnncl tu-ic acid level. Ibl-yotvpe: rliploirl KX (completr mole): triploid LKY or XXX (partial molc).

Imaging IIS, pelvic;: complex "snowstorm" appearance and no fetal parts in i~rerir~e c.:l\<ty.

Gross Pathology Characrrr-istic appear-ance of clusters of p p e s .

Micro Pathology Choi.ionic villi marketllv rnlxl-gecl aiicl hyrlmpic rvitlr surrounding cvtn- and svnc7.tintropI~ot~lxstic tisrr~c pmlifcrarion and lack of ;~tlcquatc. vasc~tl;lr supplv.

Treatment T)jlatarion and suction curettngc, periorlic rletermination of :(; levels to irlenti l l clevelnpment nI' iiisasive mole or

cAIiorinc;~~-cirio~~i:i.

' D ~ S C U S S ~ O W A gcstat ional nct~plasm that nlav p r e s r ~ ~ r as pai~lless vapnal I>lcrding, pmeclampsia in the first triinrslrr, or hyperemesis glaridarnm, hydxtirl i fhrrn rnnlp m;ly develop inro malignant

* HYDATIDIFORM MOLE

ID/CC A 53-year-old female complains of increasing fatigue, insomnia, and depression.

HPI Fur the past (5 months she had episodes in whicll her face

and neck have become hot and red ( I I ~ T FLLSHES). She has been amenomheic for the past 7 months: prior to hi.;, her meilstrual l~i=,torp was nol-mal.

PE Thinning of the skin; him~tism; atrophic vaginal mucosa wit11 drcrensed sccrcticms.

Labs Increased 24ho11r tlrinary gonadotropins (1,H anrl FSH).

Imaging XR, plain: asteoparosis of thoracoltunhar spine.

Treatment Estrogen replacement therapy brncficial.

Discussion The estrrjgen deficiency srate prodl~cerl I>? mrnopause has shol-t-rati~e ( h o t flashes). rncdium-range (vaginal atrophy), and tong--range (ostropotoris) consequences that can he relieved or

prrvrntrd b y eslrogen replacernun 1. C(1i-ninnn ~ i d e effects in patierlls taking hormone t-eplacement tl-terapv ii-tclitdc irregular I~lerdine;, eight gain, fluid relen~ioti, and eudumrtrial hyper- pla~ia. Ncvcrtlic-lrss. pogtmenopausal hlccclir~g s l ~ n ~ ~ l d he workr-d up with i t11 etirlom~~rial 11iopsv to rule out enrlomptrial cancrr.

* MENOPAUSE

IQ/CC A 56-year-old white nullipamus woman is referred for pvalnation of a pelvic mass founcl on a routi~le plryl;ic;~l.

HPI Sl~e reports increased Frequency of micturition and irregular periods until they ceas~rl 3 years ago. She has a history of breast cancer in the distant past.

PE brge cystic mass the qize oTa gr-ap~Frt~it in right p e l r i ~ that can

hc fc.1 t above the plibis qyrnphysis.

Labs Cjh-125 levels elevated; LFTs normal.

Imaging GT/US, pelvis: ?tic pelvic mass arising out OF right ovary.

Gross Pathology Partly sulid and partly cystic mass.

Micro Pathology Papillary S ~ I - E I C ~ I I ~ C S of t~enplasric- ciliated ra l~ imnar cells; psammoma bodies.

Treatment 5111-gical sta<+ng a11d rrsection: cl~einnrherapv.

Discussion Ovxian catlcer i s the third tnnst common type nf p u c o l o g i c cancer; 111e serous type is most common and is often bilateral. Tt

i< often arlvancerl at rlw time of rliagnosis (ompntal ma.ises, liver rnasscs, asrites) .

?In OVARIAN CANCER

ID/CC X 20-vear-olcl Asian female visitq her family cloctor hecause of chronic, intermittent left lower quadrant pain.

MPI The pain is not accornpiniud 11v d~spa r - e~~n ia , mrnsir~zal irl-egu- laritv, vaginal discharge, atlrtorninal rlistrnrinn, nausea, vomiting. r,l- diarrhea. I t i s not corrrlated ~ith hcr menstrual periods.

PE Left adnexal mass on himanual exam; ucerosacral ligaments 12orrnal; pouch uf DougIas 11ormal: McBurrie\.".; poin r nontender: no cviclrncc of ascitcs.

Labs Rouiii~e %ah work on 12lond. urine, and stool normal; CA-125 levels not elevated.

Imaging US. pel~is: large (5-crn) simple cyst in left ovary.

Micro Pathology Vaginal smears lor cytohor~nor~al evaluation rcveal excessive estrogenic ctimulation and lack of pro~eslarinnal erect.

Treatment Follow-up bv ultrasound (sizahrc pcrcentagr disappear spon ta- neouslv) ; laparoscopic rcmo\al if prrsistcn t.

Discussion Follicular ovarian cvqt i s the most common cause of'owriai~

O V A R I A N CYST-FOLLICULAR

Labs

Imaging

Gross Pathology

Micro Pathology

Treatment

Discussion

Atlas Link

X 25-ycar-old woman complains of loss of weight and intense r i g h ~ lower ahrloinir~al pain and nausea that began wllen she ~ ~ e r l t <jogging verterdav afternoon.

Intermit tent episodes of similar pain have occ~irred over the pml several davs. She 1ias 1-egi11;lr i n ~ n ~ t r u a I ycles with average flow and no rlysmenorrhea and I~ad hcr last period 3 weeks ago.

1's: mild hvpot~nsion; normal I-IR (IIR 90). PE: right lower quadrant tenderness; pelvic exan1 rrveals tcnrler, lnohile &cm risht adnexal maqs antericrr lo ulerus.

CBC: normal; pregnancy test ncgltivc.

327, I<t'R: i~-~-egular calcified mass it1 region nC right on-ar.;. US, pelvis: cystic tumor about 8 cm in diameter replaciilg the right oval-v.

Cystic mass replacing the right ovary: thitl, fibrous wall with

solid nodule at one aspect containing sebateo~ls material and mattcd hair: tooth strllctm-es also seen.

Malul-e tisqtle e l r r ne i~ ts representing all three germ cell layers are prerenl, including gkin with aclnrxal strrlchlres, hone, cartilage, teelh. thyroid, F>mnchi. inteqtine, ailcl neural tissue.

Surgical resection curative.

Pri~nar>, l>t.nigr~ teralonias nr dermnid cysts nriqit~ate fro111 germ cells: tllmors are cystic and contain elements of all three germ ceIl lavers. Complications of leraromas include ror-siot~, inf'ection. rupture lleaclir~g to chemical perttonicis. iz~Cel-tilitv, secre- lion ni' thwoid hnrmone leading to hyperthvmidism ( S I ' I ~ L ~ ~ I A

cn ; \~11) , and carcinoid s?ndromc duc to scroronin secretion; r;~relv, sclltarnolir cell carrinnma milv d~velnp in a dermoid ryst.

OVARIAN TERATOMA

PE

Labs

Imaging

Gross Pathology

Micro Pathology

Treatment

Discussion

Atlas Link

h 23-yem+ld married woman is seen wirh complaints of inability to conceive after a year 01' unprotected i n1~1-cowse ( I A - F E R T I L ~ } .

Her last menstrual pcriod was ?I months ago. and since tnenar- che she has only 4 to 5 periods each year (OLIGO~~T;YORIU<E~) : a pregnancy tcst at Ilorr~t. waq negative. She also complains of excessive facial hair. Her father was diabetic.

Paticnt obese: excessive facial hair and malepattern hair distribution on rest of body (I-ITRSLTSM} h~tt 110 virilizatiun ; pelvic exam nc)rmal; secondary sexual cl~aractrrist ics well developed.

Elevated LW; decreased FSH atlrl loss of normal p~riodicity (1,I-T > FSI-I. 3: I ratio); serum testosterone and androstenedione clcvated: serum estradiol (total a n d Free) hithin normal limits in early and midfnllicular phases; pattern of secretion abnormal with no preovr~latory or midluteal increase; TSH ar~rl prolactin levels normal.

P'S, ~~-anm,;vagir~al (high resolution) : morpj~ologir features of polycystic ovaries (multiple peripheral follicles < 8 rnm in c1iarnett.r; prominell t echor!enw stroma) .

Ovaries urilarged with pearly-white capsule and multiple ~ S L S

avpr-aging I cm in diamrter wirhin qrl-oma.

Cysts lined by gmnnulosa and theca cells. t h e lattcr luteiniyed; st 1-nma shows l~yperthecosis anrl lihro~is.

Reduce eight: nvulation inductior~ wir h rlorniphcnc; laparc- scrrpic ovarian diathermv or laser drilling irl cIrt~g-resistant cases;

1~11-dose corr~ billed contraceptive pill if curtrracrptiosi is desircd.

Polvcvstic ovarian srnrlrome (Stein-Leventhal syndrome) is a clinical synclrome ot obesity, hiutism, aad secondary amenorrhea or oligomcnorrhea with infertility due to anovulation,

arcompanied hv multiple-follicle cyqts within hot11 o\aries. PCOS patient5 arc at incrAei\serl risk for breast and cndonwtrial rarcino- mas (d11e 'to tln~ppnsecl LH stirnu1atiorl).

POLYCYSTIC OVARIAN SYNDROME

ID/CC h 17-!car-olrl wl l i t~ Female visits her family phvaician b e r a ~ ~ s e she has never had a menstrual period (PRIMARY ARIFVORR~FEA)

and lacks breast development.

HPI She ha5 a histnry nf low birth weight and l?mphcdema

PE Short stature: In~v-set em-s; webbed neck: cubitl~s v a l p ~ s ; low hair- line: shield-like chest with widely spaced nipples; harsh systolic murmur heard on hack (rlucx 10 cui~rctat ion of' aorta): hymplas- tir t~ailq: s11o1-t Fo~u-th metacarpals: high-arct~ed pakite; absence of pubic and d a r y hair: srn;~ll clitoris ilnd iltes~~q; ovaries no! pat galdc.

Labs High serum and urine FSH and 1.H; no Bars bodies on h~tcc;ll smear. L3rvot);pe: 45,XO.

Imaging US. pelvis: infantile sucak ovaries. Echo: bictispirl aortic valve.

Gross Pathology Fikr-<I t ic and a~rnphic ovaries.

Micro %thology Xhwncc of fclllicIes in ot arics; normal twarian stronla replaced hv fibrous streaks.

Treatment Growth hormonc and androgens for increase in stature: suhse- quuri t rstrogen thcrdpy to protect agai~lst osteoporosis.

D~SCUSS~OR The most common karvotype is 45.SO: less commonlv. m a snicisixi. Tlrrnrr's s~rldrorrie is associatrrl with fr-rqurnt skeletal, renal ( I i n r s ~ ~ h o e kidney), and catdiovasc~rlar anomalies.

P R I M A R Y AMENORRHEA-TURNER'S SYNDROME

ID/CC A 39-yeawlcl black female prrsents will1 a several-tnnr~th-long historv of profuse menstruation I F ~ P F R M E N C ) R R I I ~ - ~ } and

frequent rnenstruaT periods (POI SMFVORRI I F 4).

HPI Furr her- qu~qtion ing alco reir;ds painful periods (DWMEUORRI 113)

a n d irirl-easing urinary frecluency. She has a history of infertility and recurrent spontaneous abortions.

PE Enlarged, irregtlar uterus .on himanual palpation with swesal rria\';e.; nri po\t erior wall.

Labs CBC/PBS: h\-poch~-omic, microcvtic anemia.

Imaging US. prlvi%: muItiple heterogeneous masses cliscorting uterus.

Gross Pathology Occur in myonlctrium (95% ill-e intramural) and are round, firm, and well cil-c~nnucribecl.

Micro Pathology In ttrli~cing bunclles of unilbt-tn, clif'fer~ntiated. elongated mlrmth muscle cells with few mitows and no ar~;ipli~ria; maligrtant transfor-marioit rare.

Treatment Myomerrornv: hvsi ~rectnrny.

Discusdon TI~P most common tumor of the uterus ancl thc most common tumor in women, utcrinc fibroids are estrogendependent and commonly occrlr after 30 yc.;l~-s of age: ~ h c y lend to regress aftcr menopause.

* > , UTERINE F I B R O I D S

ID/CC A 60-!car-old woman \;isits l ~ e r pmecnlogist becatire of a foul- smelling, blood-tinged, purulent vaginal discharge.

HPI Shc has nevcr hrcn married and has never been prepant. She is l~vperrcnsivc and take5 oral I~vpoglvcemic agents for diahetes inellitti%.

PE VS: RP normal at present. PE: ovcrwight: fleshy, bulky? fz lr ipt- ing tumor protrtiding fi-nm cervical ns rln vasinal speculum exam.

Ernaging CT/MR: Iarge, cnmplrx mass arising From I I I P ~ I ~ S .

Gross Pathology large. flerhv rumor- or rnynmetrium with areas or ~iecrc~sis and hcl-nclrrhage.

Micro Pathology Rackground ol ~pindle-shaprd cells wi th mom than I0 mitoses

per high-power field on hinpqv; many mitorrs h a w ahnarmd nlr totic spindle.

Treatment tZrIriamscin, progcstins, cornbinaliol~ chemotherapy.

Discussion :I higlllv aggressive malignant rumor or rn~wtletril~m.

Ieiomvosarcoma of the utcrrls may arise in a leioin).t)rna or de t lnvt l . I t spreads I y contiguity, liematng-enously, attcl through Ivmphatics.

Atlas Link E T f T T PI-P3-059

? UTERINE LEIOMYOSARCOMA

ID/CC .A GFi=year+ld woman is rekr-red hi- intraciahfe n~Imr growth and pruritus.

HPI Shc has ~ F O fc11 an ohsttuction i11 ~ h c flow of her urinc. She was :I prostitute and wa'i ~re;~ttrl o f t e ~ ~ ti)r STBs. She is a chronic smoker.

PE Cvnecologir exam reveal5 rxcoriation marks over \ ~ ~ l v a : exnphydc grnwth ariqing from leri labia niajora; left inguinal Iy phadenopnthv.

tabs C:vr;tc>scnpv reven15 lower l~re~hral ~ ~ e n o c i s (clue to involvement Iw vulvar growth).

Gmss Pathology Gross ~x~imination reveal5 firm, rxophyric growt 11.

Micro Pathology Micrnscnpir exam or piinch hiopsv sperimeai rcveals invasivc, welldifferen tiaterl s q ~ ~ a m o u s cell carcinoma with keratinuation.

Treatment C:onFir~n diagnosis: preoperative rarlintherxpv to shrink tumor rnilqs; radical i~~ lvcctomy with lymph node rlis~ection.

Discussion \'11h.;~r cancer is a cliscasc of oIder women with ;I mean ngc of t i 0 veaw. I t iq associared with smoking. and it5 reccrlt i ncrea~e in incidencc among younger women is associated wi tll papaw mavirus. Carcinoma in sit11 ( r ~ ~ l w r intraepithelial neoplasin. or XTN} arid qquarnolrs dysplasia ;11- t considcrrd prcclirsur Icqions.

VULVAR CARCINOMA

ID/CC rZ 75-yuar-olcl white woman visi1.r her p c c o l o ~ s t Fnr a routirxe checkup and i s round to have white spots r m her genitalia.

HPI She co tnp la in~ oFslight outer vaginal itching bur rlenios any pc>$~n~eraopar~saI hlcr-ding. vaginal rlischar~e, or drug intake.

PE Hypochromic rnacules 011 1al)ia majorn extcndir~g to perincunl and inner ~liighs in patchy distrih~ltinl~ scale formation IDESQLIAMA~C)N): skin thickened and rough (FI ITI :RRE.~~T~TTC) : no

regional I~mphadenopathy; atrnpllic vaginitis on vaginal speculum exam.

n - Micro Pathology Biopsy reveals hyperkeri-ltosis and Gl~rosis wirh thinning of

4 z sqrialnous cpitlielium; lynphocvtic in fl;lmnlatory infiltra~ion, m r)

most prevalent n~rrounding t>tood vesseIs; no chronir inflamnra tory respclnse; 110 r i p s of malignant transfr~rnli~timn. 0

CI <

Treatment Biopsy; subsequent treatment clcpenrlent on diagnosis.

Discussion Vulvar leukoplakia is a clinical diagnosis that can be attributed to a varipty nf disorders t h a ~ all pradricc while patchcs. Caures may he h e n i p dirorc1~1-s such as ritiligo, as well as inflatntnatory conditions, prrmaIignant cor~r l i i ions ( e . g . , d!rstrnpllies), or s q ~ ~ a t n o ~ ~ s cell carcinon~;t. Always perform n biopsy.

* L a - VULVAR LEUKOPLAKIA

ID/CC A 73-year-old woman i s brought to a gmecoIogist by her daughter, who became aware of a genital ulcer while helping her mother shower.

HPI Hcr history reveals weight less and dyspnea together with h~pertcnsion and arthritis.

PE Hat-d, nodular, hnm pigmented and ulcerated lesion on upper left labia rninora; no i ~ ~ g u i nal lynp hadenopathy: scattererl cxupitant rides on chest auscl~ltation.

Labs CDC/PBS: slight anemia.

Imaging CXR: multiple metastatic nodules.

Micro Pathology Biopsy reveals malignant melanoma cells with lymphocytic reaction infiltrating into undrrlyi ng dermis; cells stain positive for SlIOO antigen and are negative for mucin.

Treatment Surgery with regional lymph node dissection and adjutxnt chemotherapy.

Discrrsdon VlrIvar malignant melanoma is the second most common wEvar malignancy (the first is squamous cell carcinoma); r n e t a m ~ i s and prognosis depeild on the extent orvertical growth.

VULVAR MALIGNANT MELANOMA

ID/CC A 25-year-old woman presents with amenorrhea of ti weeks' duration and pelvic pain over the past day.

HPI She h= a history of va<@al spotting off and an for the past 2 weekq and has heen using an IUD for the past 3 years. She ha5 no history of vaginal discharge and no urinary symptoms, and her previous mensrrtial I~istory is normal. Shr has had multiple bc~~tts of pelvic inflammatory disease.

PE VS: BP normal. PE: p~lzllor; abdnrninal distention and decreased bowel sounds: cervical motion tenderness; uterus soft and slightly enlarged on prlvic exam; soft, tender, boggy mass in right adnexa and pouch of Douglas.

Labs CDC: anemia. hCG levels lower than expected for this period of firstation: culdocenteds reveals presence of blood in cul-de-sac.

Imaging US, pelvis: no products of conception in uterine cavity; cloughnut-shaped mass in right adnexa; echogenic free fluid in culilc-sac.

Gross Pathology Extm~~terine pregnancy, most commonly tubal.

Micro Pathology Uterine curettage reveals presence of Arias-Stella reaction in the absence of villi.

Treatment Laparoscopic linear salpingostomv and seamen tal rrsection; methotrexate,

Discussion Other risk factors for ectopic pregnancy itlclude previous tubal surgery, tuba1 ligation, endomeiriosis, previous ectopic pregnancy, and ovulation induction.

ECTOPIC PREGNANCY

ID/CC A 58-ycar-old grand mdtipara develops a marked drop in her blood pressure follo~ving uncontrolled bleeding immediately aftcr delivery.

HPI She delivcl-cd twins at 35 weeks' gestation with polyhydramnios.

PE VS: hypotension; tachycardia, PE: anxious; p a l l r ~ r . ; low central 2.enous pressure; uterus sort and flabby with inclistinct outlitle.

Labs CBC: anemia; mildly decrpawrl hematocrit. Coag;lllation profilr normal.

Gross Pathology I!rerus grossly overdistended and flabby.

Treatment Fluid resuwitation; blood transfuvion; uterine massage; maintai ti contraction with an oxytocin infusiorl: ergotarnine fur ~~asoconstriction: if folrn~l, remove retained placenta; cllcck for cervical. vaginal, or- uterine 1acer.alions and uterine ruptrlre; h ~ p o g a s ~ r i c artery ligation and/or- hysrel-ectorny if other mmsures Tail.

Discussion Primnry posrpartltm hemorrhagu (PPH) is defined as loss of

500 mL ar more of I,lood witllin 21 hours or a vaginal dcliverp { 1.000 rriT, af'let- a C-section) or anv atnount cj f h leet l i~~g that is surficient to produce a hernoclylamic compromise; primary causcs i11cl11cle urel-ine aton): rvhiiled placenta. and soft risque in jury. Faclors asrnriatccl with an iiicl-easecl risk of uterine atnny ;~ncl r - r la in~r l p l a c c n ~ i n d udr high multiparity, a maternal age greater than 35 years, delivery after an antepsrrtum hemorrhage, multiple pregnancies, polyhydramnios, a past history of PPR, and coagulation disorders. Sheehan's synclt-eme-a cIinicaI svndromc uf hvpopi t l~itarisin seconcl;~ry 10 isrhelnic pitltiitaty necrosis-is ;I peculiar complicaticlt~ of massive po.;tpar!mn

* POSTPARTUM HEMORRHAGE

ID/CC A 28-ycnr-01d woman prcsents with wefling of hcr cntire left leg nf 1 cli+v's rlt~rat inn.

HPI She d~lfvered a nnl-ma1 full-term male baby 2 dr~ys ago.

PE L t 4 leg erythemataus, warm, swollen, and tender.

Labs Rrniliiie tcqn tinrni;~l; normal clotting profile.

Imaging US. Doppler: clot in left ftan~ural win. V e r ~ o ~ ~ . ; ~ p h y : cnnfirma- tui-y "5oTd staii(l;~rrl" hut tiv~aIlv not required.

Treatment [V hepnrin ancl monitoring of clotting time ;lucl Pm ele\ation of limb: analgesicr; ancl soaks.

Discussion Phlepasia alba dolens (painful white leg) is due to iliofcmoral vcin i l~rr~ii~bosis nucurring in Iatp pl-egnanrv find postpartum: it is relared ra compression by a gravid utens and hypercoagda-

POSTPARTUM THROMBOPHLEBITIS

Ill /CC A 30-year-old woman presents with fhtigue. significant weight loss. and amenorrhea of 2 years' duration.

HPI She had a baby 2 years ago and suffered significant postpartum bleeding. Shc bottle-fed her Rally because she was unable to lac- tate after delivers.

'PE VS: hypotensinn (BP 85/60) . PE: skin tenling: fine wrinkling atnllnrl eyes ancl mouth; loss of ndllary ancl pubic hair.

Labs Decreased levels of trophic hormones @H, LH, ACTH, TSH, GH, prolactin): decreased levels uf target glai~d hormones IT,<, TI. cortisol, estrogerls) .

Imaging MR. pi tuital-v (usuallv before and after iiljection or gadohniuin DTPA) : abnormal signal in pit~utar.; grand.

Gmss Pathology Soft, pale, and liernort-hagic pituitary gland in carIy stages; shrunketi. fihnlus, and firm in latrr- stagus.

Treatment Mornlone rrplacemcn t: cortisol; levothyroxine (T,) ; estrogen-11rogcstero~lr replacement.

Discussion Sheehatn's syndrome is mmt commonIy caused hv postpartum infarction of the pituitary. During pregnancy, the anturior pituitary grow4 to nearly twice i t5 normal sizc. During delivery, 105s of hloocl or hypovolcmia decreases flow to the pituitary, inducing vasospasrn h a t lead.: to ischemic necrosis of the anterior pituitary. The posterior pi t z~ i ta ry is supplied by arterieq and i u Illerefore mucll less susceptible to iscllcmia. Lnss of trophic hormoncs leads to atmphy of target organs. Ischerr~ic necrosis tnay also occur in males and in nonpregnanl f ~ ~ n a l e s (rraurna, sickle cell anclnia, dissemi~latecl in travascular coagulation, \ 5 ~ s c ~ ~ l a r accider~ ts) .

7 SHEEHAN'S SYNDROME

ID/CC ASfi~ear-r~ldwhiteprimi~~daat36weeksofgestationvisit~ her ohqtetrician for thr first time in her pregnancy complaining o l swollen legs and headache.

HPI Her medical h isrory is unremarkable, and her pregnancy had apparrtl~ly developed w i t h no complications until the onset of her m p t e m s .

PE VS: hypertension (BP 170/110). PE: excessive weight gain (19 kg) ; fundusropir exam does not s11ow changes of llvpcrten- sive retinnpathy 3+ pitting pedal edema: 1 t pcriorhital edema; fundal heighl appropriatr: retal parts palpable; fetal heart solinds nortna!.

Labs CRC:/PBS: complete blood c o u t ~ ~ s and coagulation profile normal. Serum uric acid concentrations raised: mildly elevated AST and ALT; 9l- proteinuria.

Imaging US, OR: single live Ferns; Lie longitridinal: presentation cephalic; normai biophysical profile; placental infarctions seen.

Micro Pathology EndothtliaE cell s~velling with ohliteration of glorneruIar capillarv lumen on renal biopsy.

Treatment Antihypertensive agen n; delivery of Fcnls and placenta, usually by Gsection.

Discussion Preeclampsia occtirs in 5% of all pregnancies; i t is most comnlon during the last trimester of a first prepanq. It is cllancterized by the triad of hypertension, proteinuria, and edema, Pro~ession to eclampqia may occur with visual disttirhances. seizlrres, and coma.

IWCC A 14-year+ld male is admitted to thc hospital complaining of pain and .welling in the Ieft leg.

HPI T11c pain has heen prescnt Tor 2 months hut has become progressively worse over chat period. Thcrc is no llistory of trxllmx or infection.

PE VTS: mild fever. PE: tenderness ancl Fr~riform swelling over left fptnu~.

Labs Elevat~d ESR. K a r p t y x : translocation of the long arms of chromosomes 11 and 22.

Imaging XR, lcfl femur: lylic lesion in rned~~llary zoilc of rnidshaft with cortical deqtructinn and "onion-skin" appearance. CXR: no evidence of metastatic spread.

Gross Pathology Large areas of' bone lvsis 21.5 tumnrs erode cancclfo~ts t~-aheculae nf long hones outward to cortex.

Micro Pathology Biop~y of I ~ o n c rcvuals sheets of unifor-tn, small cells rescmbIing Iwr~phocytes; in many place5 t urnnr cells surrollnd a central

clrai, area. forming a "pseudorosette.'Tell origin of tumor is unhown.

Treatment "Melt" tumor with radiotherapy: surgical resection: chew other- ally: regular fnllow-up for recurrcncu.

Discussion Diaphysis of the long hones is the most common site of occur- rence of Ewing's sarcoma. Five-year n~rvival is 70% for locaIlv rcscctahlc disrrasc bu t only 50% for those with advancer1 metastasis.

1 EWING'S SARCOMA

ID/CC A 45-yea~lr l woman visits an orthopedist hecausc of an inability to extend her Ealu-th and fifth fingers.

HPI She has a long-standing history of alcohol. abuse and has been to

the emergency room sereral rimes for alcoholic gastritis.

PE Mild icterus; [lalmar erythema; muscle wa~ting: malnol~rjsh- tnent; ahdome11 rcvcals 2 + asci tic fluid (due to alcoholic liver clamage); fourth and frfrh fingers of right hand reveal flexion contractme wid1 nodular thickening and hick bands of tissue palpable ~rporl drawing examining finger- acrnsq palm.

Gross Pathology Infiltration of pnlmal- Eawia with fibrous tissue and suhseq~~cnt contraction riefbrmiry.

Micro Pathelogy Infil~t-ation nf pretendjnous fascia with rnvofihrohlasts with fih~osis nf pretend inn^^^ bandq.

Treatment Surgcry (release of contractures and aclhesiot~q) ; rrequen tly l-cctC11 rs.

Discussion Alsv callecl palinx fibromatosis, Dllpuyrren'~ contracture is of 11n known etiology hut is associated with alcoholism and manual labor. It is associated with diabetes and anticcln~llsant medications.

CONTRACTU RE

ID/([ A 60-year-old woman is hrough t to the ostl~opedic clinic with compfaints of pain in the left hip and inability to bear weight on he lcft leg.

HPI Thee years ago s l ~ r sustained a fracture or the neck OF the femur that was treated with intel-nal fixation. Slie is an alcoholic and has hecn taking oral steroids for many yeiirs fur a ctironic %kin ailment.

PE 1\11 movement? at left hip are restricted by pain: mrahle to hear weight on the limb.

Imaging XR, left hip: increase in bone density of femoral head and col- l a p ~ c n l articular s~~rface; dynamic hip screw in placr. MR. hip: Inore sen5itivr.

Treatment Total hip replacement arthropIa-ty significan tlv r~dnces

morhiclirv.

Discussion Fracture of the neck oaf the femur is the most common cause of avilwular necrc3vis of the femoral head: 01 PI. risk lactors iilclude rxccs~ivtr alcohol consumprion, strroid therapy. radiation [her- apv, sickle cell anemia, and deep spa diving (Caisson's disease). Normally blood is supplied to the head by three routes: rhrntlgh vewels in the ligamen tlim teres, tliro~ngh capsular vessels rc-

flccted onto tllc femoral ncck, and thnmgh hranchcs of nr~tri - en1 v~sce l s within the s~xl>sra~~ce of the hone. W ~ e n the fracture occurs. nutrient vessels arc necessarily severcd, capsular vessels are injured to varying degrees, and bIood supply is maintained only through the vessds in the ligamenturn tern. This ic a variable q~~antity and is of'ten in~ufficien t, r e d r i n g in avasculru- necrosis of t I ~ c fcmoraI head.

HIP-AVASCULAR NECROSIS OF FEMORAL HEAD

ID/CC A 12-year-ald obese mde is brought to the horpital with canlplaints of sudden-onset pain of the left hip along with a limp.

HPI The pain is felt in the left groin and often radiates to rhe left thigh and k n e ~ .

PE Left leg externally rotated and about 2 cm shorter; limited range of abduction and internal rotation; upon flexing left hip, knee is drawn toward left axilla.

Imaghg XR, left hip (AP view) : growth plate widened and irregular.

Treatment I-Iead was fixed with pins or screws to prevent further slipping.

Discussion Slipped femoral epiphyses affects youth 10 to 18 years old, with boys mare commonly af'fected than girls; affected children may hc overweight and in some cases have delayed sexual deveIop ment. represent^ a Salter-I-Iarris type I epiphyseal injury. Twenty-five percent are bilateral, of which 15% ta 20% occur sjmul~neously. Avascular necrosis of the femoral hrad and osteoarthritis mav arise a~ complications.

HIP-SLIPPED CAPITAL FEMORAL EPIPHYSIS I C "r'

- - - -- -- -

ID/CC A 2.5-vear+ld Ce~nale athlete is hmright to rlie ER after she hurt her right knee.

HPI She I-iad failen on a hyperextended right knee i h a ~ has been un- stahlc since the fall. Slw recall5 having heard a "popping" ronnd ;& t the time o f the i n j~~ry

PE Kighr kneeexhihits~ffisionandpositiveanterior"rirawsi~" (!ilia can he p ~ ~ l l r d fol-ward on fc~mur with knee flexed) ; insta- hiliry o f right kncc. joint (drmons~raled hp moving uppri- end of [ilia fcrrwat-d on I'cmur with knec flexed only I0 ro 2C) d e ~ c c s 1 L A ~ I - I ~ U ~ K TES-v] ) .

Imaging MR. knee: indistinct, I~etcrogenous ~ignal in expectcd region of rhc anterior cruciate ligamenr.

Treatment Surgical I-ecc~nsirucrion; pla5ter cylinder for 3 weeks followed by hainstring and quatlricepq e x e r c i s ~ ~ .

Discussion The ar~tel-io~- cl-l~ciate ligament is torn by a. force dsivit~g h e upper cnd of thc tibia forward relative to the f e n ~ r ~ r or hy 1lype1.- extension of the knee; [he posterior mlciate ligament is torn hy a Force driving the upper end of the tibia backward.

KNEE-ANTERIOR CRUCIATE LIGAMENT INJURY

Labs

Imaging

Cross Pathology

Micro Pathology

Treatment

Discussion

Atlas Links

A 60-year-ld obese female is seen wi th corn plainh of gradually progressing stiffness and pain after use of the right knee.

Thc pain and stiff'ness are accornparlied by welling and deformity of the joint. She also reports difficulty walking aflcl limita- tien of rnovenletl t.

Tenderness, pain, and cr-epitus of right k n r e on mnrion: firm m~cllii~g (ca~isrrl hy underlving bony proliferations) and joint cffi~sion; fix~rE rl~fol-mities: bony enla~,geme?lt ancl a. varus angulation, causing limiter1 motion at+joint; harlcls show bony swellings en distal interphalanged joints (HEBERD~N'S V O U E S ~ .

Synovial fluid shows n o evidence of iliflammation; nosnlai viscosir)r and rnucin clot tests: pror~in, glucose, and compleinent Icvr*ls also normal; serum rheumatoid fi~ctor no1 raised.

SR. right knee {.Q and lateral views): narrowing of,join t space (medial > lateral) ; suhchondral I~one sclerosis: suhchondral r y t s and osrrophytus.

1,atr wages nf the discasr show eburnation of-joint uorface, rt?modeling of joint surface, osteophytes around lateral margins of joint, su bchondral hone cysts, and hur~r ~clel-osis.

Loss uf articiilar cartilage, bone resorption, and irregular and variable new hone and cartilage furn~:~tioil.

I'ain relid, impruvcrnent of mohili!y, anrl correction of dc formi~; join 1 replacement.

O~teoarthritis. a degenerative joint rliqeaqe. is characterized hv t h c degcntra~ion of articular cartilage and by progressive desu-uctinn ancl rcmrjdeIing OK the-joint struckrrrs. The condition affect< largr weight-hearing joints such as the knees, hips, and lutnhar a11rl cervical vertebrae: otf~el- jnints commonly af tec ted are the PIP, DIP, and first carpometacarpal joints. It is more common in women, and irs incidence increases with age, partic~iZarls aft~r- .55.

P?@ OSTEOARTHRITIS

ID/CC A 12-year-old male presents with a swelling above thc right knee and associated pain.

MPI There is no history of trauma at the site of pain. There has been no discharse From thr swollen rq ion and no fever.

PE Bony-hard, tender, roughly circular swelling ahove right knee (distal femur); averlying skin Temperature normal; mechanical re.;triction of nlovement nf right knee.

Labs Normal ESR elevated serum alkaline phosphatase (may be used xs marker of treatment response).

Imaging XR, plain: osteohlztstic bone lesion at distal end of femur with characteristic "sunburst" or "onion-peelu pel-io~teal reaction; pcriosteal elevation hl; rnetaphyseat tumor (Cot~~~Ih"s TRMNGLE) .

Gross Pathology Firm. whitislt mass with osteoblastic hot2 P sclerosis originating from rnetaphysis adjacent to epiphyseal growth plate and invading through cortex, lifting up prt-instcum.

Micro Pathology Bone biopsy shows multinuclcitted @ant cells, anaplastic cells with pleomorphism, and osteoid production with I'oci of sarcomatous degeneration.

Treatment Stlrgical amputation; consider Iinlh salvage: radiotherapv, chemothe~-apv.

Discussion Osteoget~ic sarcoma is the mo7t common prirnarv malignant tomnr of hone (excluding mvrlnma and I ~ p h o m a ) ; it may he oqteohlasuc or osteo1;vzic. Pathologic fiactr~req may occur; pulmonary metastases are fseqlreu t. These i~ an increased risk ~+ i th Paget's disease, prior t-adiation, and l-~eredjtarv re tinohlastoma.

Atlas Link EEF'JT PM-F3-074

OSTEOGENIC SARCOMA

ID/CC A 70-year-old male inlmigrant from Etlgland presents with pain in the right leg, producing an awkward gait, along with hilateral hearing loss.

HPI Hc has also noted a progressive increase in his hat size.

PE Slight bowing of right tibia; normal rectal exam; mixed conduc- i i w and sensorineural hearing loss confirmed by audiometry; pliy~ical exam otherwise normal.

Labs Markedly elevated alkaline phosphatase; rnijdly elevated serum calciiim and phosphorus; normal serum transaminases; increased urinary excretion of hydroxyprohe.

Imaging XR, skull: scattered islands o f hone rysis {OSTEUI~OKQSIS

CTRC:IIMSCRJP'~A); mixed thickening (hlastic) and lucency (lytic lesions) of bonc {COTTON-1~0~. A P F E M C E ) . XR, leg (right side): bone soft with disorganized trahecufar pattern; bowed tibia.

Gross Pathology Expansion of bonc cortex, hlastic h m ~ e lesions, and bowing of long bones (thick ivory bones).

Micro 'Pathology Mul tiplc cement lines with unminer-ali7ed nsteoid; indicative of excessive osteobIastic and osteoclastic actni ty.

Treatment Ostcotomirs: calcitonin: diphosphunate; mithramycin. r;l

Discussion A condition of probable viral etiology, Paget's disease is charac- z o tetized by osteoclastic destruction of bone initially with excessive VI

o<!eohlastic repair, producing bone sclerosis. Mihen extensive, the resulting irlct-eased blood flow leads to high cardiac output congestive heart failure. Other compl isation? are pathologic fracture and osteosarcorna ( I % or patients)

PAGET'S DISEASE OF BONE f I

ID/CC A 5-year-old male i s hrought to a physician with sudden-nset progressive ~evet-e pain, swelling, and rednew of-the right knee jo in t .

HPI HP lias had a high-%grade fever tbr. the part 2 days. A few days ago IIP injured his right leg, and the injury subsequentIy became infected: Ilc is now r ~ n a b l ~ to mow his right Irg properly.

PE L'S: fever. PE: infected ~vound on r j g h ~ leg; right knee red, swollen, and tender: all movements reqt ricted by pain.

Labs CRC: neutropMic leukocytosis wilh shift to thc left. Elevated ESR: p o v i a l fluid (obtained following joint aspiration) opaque and yellowish; joint effusion has WBC cormt > 50,000/ pL; Gram stain reveals gram-positive cocci in clusters: culture vields cnagulasp-pnsitivc Staphy Eococc~ds aureas.

Imaging XR, right knee: early. soft tissue swelling and joint effusion: late, articular el-nsinns and reactive sclurusis. KLiC. g-;l!Iiurn scan: increased uptake by right kilcejoin I.

Treatment Broad-spectrum parented antibiotics initially. then speciFic ant i biotic5 following culn~re srnsi titit)! rPporrs: if necessary joint may be opened. washed, and closed with a suctiorl drain and immohili7erl ~ i i~ t i l signs of inflammation subside.

DTscussion Septic arthritis is caused by pyogenic organisms and is more cnnirnorl among children. especially males. S. rrivrruq is the most common cause; other urFrlisrns inclurle streptococci, gonococci, pi~cumococci, and Missuria mrne'n,gitirlr.~. Organisms reach the joint \-La hematogenoils ronte5 (most common; the primary rocus

may IIP a pyoderma, throat infectio~~, TV dn~j: tlqe, etc.), secondary to adjacent osteornyelitis, ar via penetrating wot~ntls, or the conditiot~ may be iatrogenic.

Atlas Links E37E IMz-041~. IMZ-041B

9 SEPTIC ARTW RITIS-STAPHY LOCOCCAL

I D /CC A 55-yrar-old woman presents with at1 achiig pain in the back of her neck, a feeling of stiffness, and a "grating" sensation upon rnovmnent.

HPI She ;11w has a historv OF a vague, ill-rlrfined, anrl ill-localized pain sprcadirlg OVPI- her shouldcr region. She cIocs nol contplain of ally noticeahlc motor weakness or sensory loss over any part of t h e hodv and has n o bladder or bowel campIaints.

PE N ~ r k slightly kyphotic; posterior cervical muscles tender; neck movements s l ig l~~ lv r e s ~ c t e d at extremes due to pain; audible crepitation on movement; diminished suphator and biceps reflex in the left upper limb: no motor or sensory loss demon~tnhle .

Imaging XR, cervical spine (latcral view) : narrowing of intervertebral disk space with formatian of osteophptes at vertebral matgins, especially anteriorly.

Treatment There i~ a strotlg te~ldcncy fur he symptoms of cervical spondylosis to subside spontancouslp. Trea~rnent includcs analgesics, physiotherapy, and support of the neck by a closely fitting collar of plaster or plastic. Surgical intervention is I-rquired tbl- patients who are unresponsive to conservative theixpv as well as for rhose with progressive inyelopathy or

z Discussion Degen~ratiw arthritis occurs predonlinantly in [he lowest three cervical jointr. T ~ P changes first affect the central interverte- v,

bra1 joint5 and later affect the posterior intervertebral (facet) joint?. Osteophytes commonly encroach on the intervertebral Foramina, reducing the space rnr transmission or the cervical nriAves. If the rmtricted space i s f i~rther reduced by the traumatic edcma of h e contained soft tissues. manifestations of nerve pressure are likely to occur. Rarely. I he spinal cord it.;elC may suffer darnage, prnducing a cer-viral myelopathy.

SPINE-CERVICAL SPONDYLOSIS

ID/CC A 32-v~artllcl homeless white male is brought to the emergency room hy all amh~~lance fnllowing convulsions that took place on thc street.

HPI The patiell t is dirhevel ed and unshaven in l i i ~ appearance. A history; cannor be obtained llecnllse he is arnne and unable to

respntld tr, qilesr icltls.

PE Dehydration; jaundice; alcohol on breath; 2cm laceration on occipital area with no hleeding: semicomatose state with response to pain only: p~lpils equal: fine tremor in extl-emiries; p ~ l m a r erythrnla; hepatomegaly.

Labs CBC/PBS: maerocytic, hypochromic anemia. Elevated direct anrl inclirect bilin~bin; elevated AST and ALT; ASTJALT ratio n1' 2 1 ; elevated alkaline phnsphataqe: elevated PT; low serum albumin: h ~ ~ ~ g l v c e m i a .

CKISS Pathology Fatty liver; tnicrorlodular cirrhosis; market1 gastritis: hl-onchiaI aspiration.

Micm Pathology Hrpatacytex clistendcd with fat; hepatocellular necrosis: Mallory bodies (t~yaline); cytoplasmic vacuolkntion of stem cells in hone marrow; mvofihl-illar necl-o~is: diffuse axnnal degeneration.

Treatment Vitami11.: (thiamine xlld folatc) : gIitcose: I-el~ydration; trear acute withdrawal and drliriunl tremens (DT?) with t>enzc>diazepines.

Discussion Ncoholic DTs usuallv occur 2 to 5 clays aftcr cessation of drinking ant1 are rliaractel-i~ed Ilv sei;.ut-~s. ~ P ~ ~ I S ~ O I ~ S , agitation, disorientation, vist~al and tactile I~allucinations, and autonomic instabilitv. DT PI-ophjaxis cerisists of benzodixepi r-les and resl~xints 10 prevent d a l n a ~ e to patient and lo 0th~1-S, DTF have a rntlrtality rate or 15% if l~nt rea t~r l .

ALCOHOLISM -

Labs

Imaging

Gross Pathology

Micro Pathology

Treatment

Discussion

A 24year-oId white male visits his family doctor complaining of low back pain and stiffness of the spine for almost 1 yeal; incl-easitlg in severity

The back stjffnrss is eased h!, a hor shower, and worsens afser prolonged inactivity.

Stooped posture: loss of lumbar Inrdnqis and fixed kyphosis; tenderness over sacroiliac jointq; reducerl chest expansion; pl-ominent ahdomen.

EEcvatrd ESR; negative rheumatoid tests; positive HLA-BZT.

XR, plain: sclerosis and blurring OF margins of sacroiliac joints; afikylosi~ and fusion of vertebrae ( U ~ i h ~ ~ ~ ~ SPINE") in long- standing cascs.

Calcification nf intervertebral disks and longitudinal ligaments.

Similar to rhetlmatoid arthritis, but in different location and no rhcumatoicl nodules.

Phvsical therapy; NS.4IDs; srilfasdazine.

Also called Marie-Striimpell disease and associaterl with HLA-B27, this inflan~matnrv arthritis with event~ial ankylosis of the spine is tvpicallv see11 in young mates; long-standing cases may present with iritis and aortic insufficiency. Ankylosing spondvlitis is also associat~rl with Reiter's svndrome ant1 inflammatory bowel disease.

ANKYLOSING SPONDYLITIS

ED/CC A5.%~-e;~r~ldmaleisbroughtiilto thcu~nergei~cydepartiiirnt diaphoretic and ashen in appearance.

H P I HP is ail experieizced pilnt who w7aq o n a crosscountry flight rrom New York to California. I-tis ascent was unrvuntful to ax1 al- tituclc of 30,000 feet above mean sea level in a n tlnpresst~l-ized airplane. Aftcr I hour at this c r ~ r i s i n ~ alti ~ u d e (and while on stipplemeillai oxygen). h e pilot noticed a gmd11al onset of weaknes and paresthesias of the right arm. These symptoms prc>gresqivelv worsened to involve both arms.

PE VS: normal. PE: disoriented and confused; normal cardiac and pulmc~n;u-y exam: ne~rvologic exam revealed severe flcxor wcak- nes5 at the right elbow and wrist, numbness of the Forearm. and impiirecl fine motor control.

Labs (;RC/Lytes: nnrrnal. Xkntilation-pel-fi~sion scan ~howc n o \ r Q nlisma~ch. XnGs (at room air): normal.

Imaging C:XR: mild increase in i 11 tent itial mal- king^. Considel- head CTT scan if mental scalus changes do nnt improve with hyperbaric repressut-iZalion. MRI may he user111 for neurologic dccomprcs siorl icknrss (DCS) 10 locali~e area of i r ~ j ~ ~ r y .

Treatment Initial managemurlt with hydration, 100% oxygen delivery by mask, and transfer to a hyperbaric chamher for compression therapy. Serious cases of DCS tnav require i nr~~hatioil and prtmor agents. Compression therapy is the definitive treatInunt, anrl Ireattnent shnuld be qtarted as soon as possible.

Discussion Decompression sickness, also reCerred to as Caisson's disease, c>cr.urs wl~en a person is subjected lo a rapirl I-eduction in ambi- ent pressure, resulting in the formation of dissolved I~ul>hles or nitr-ogen withi11 body tissues ("he bends"). DCS car1 resull t'rom high-altitude exposure (i.c., a n unpressurized aircraft at > 29,000 I'eet altitude) or firom work in pressill-ized tunnels or caissorls, and is most cornmanly associatect with compressed-air (S(:URA) diving.

DECOMPRESSION SICKNESS

IDJCC A 52-year-old white female complains to her fanlily rloctor of diff idty climbing steps for tllc past 6 mnnrhs and difficulty washir~a her hair Tor the past 2 weeks.

HPI She stales that she docs not feel tired nr short of breath h l ~ t that her l~gs and arms " j~ast will not cooperate." S l ~ c alro cotnplains aC intermittent fever.

PE Periorhital edema with pl~rplish discoloratio11 ( r r ~ ~ r o n o ~ ~ R % ~ H ) ; bntterfly rash on facc and neck; Raynaud's pher~o~nenon; scaling of skin with redness around knzzckles (C;OTTRC)Y'S

I.ESIONT); proximal muscle weakness with tenderness in all four cxmcmi ties.

Labs CRC: mild leukoqtosis. Elevated serum CK; elevatetl aldola~e; elwated ESR: antinuclear antibodies (ANAS) presrnt, particularly against tRNA. EMG: spontaneous fthrillatirln.

Gross Pathology Muwle edema progressing to tnuscle atrophy and fibrosis.

Micro Pathology Musclc biopsy shows lymphocytic infiltration, primarily in a perivasc~tlar Fashion hut also betwren muscle fibers on muscle l~inpsv: atrnphv and fibrosis seen.

Treatment Corticclsteroid~; mrthotrexate; azathioprine.

Discussion Dermatomyositis is a n idiopathic disorder primarily affecting c~lcler females: i~ is fi-cquen tly associated with malignancy (c.g., ovarian carcinoma).

Atlas Links IM"1051A, IM2-051B

DERMATOMYOSITIS

ED/CC A 10-year-nId male presents with a persistent low-*grade fever, skin rash, and painful swelling of both knees.

HPI: Kc also complains of excessive fatiwe and significant anorexia. He has no history of sorc throat. pedal edema/orthopnca, noc- tl~rnal dyqpnea, or involuntary movemen tq.

PE 1%: fever. PE: extensive ~rythematous rnaculopapular rash; $en- eraIizcd Iymphadenopathy; hepatosplcnomcgaly; arthritis or both knees; no suhcu taneous nodule^; no evidence of cardi tis:

no Roth's spots on Funrluscopy; no petcchiae over skin or mucnsa; normal cardiov~scular exan.

Labs CBC/FBS: leukocytosis; normoq-tic. iluri~lochrorr~ic anemia. Elevated ESR; blood cultures sterile; AS0 titers normal; throat qwnh sterile: rhelunatoid factor negative: Teukncytosis with elevated proteins and markedly low glucose and complement levels on syno~ial fluid analysis. ECG: normal.

Imaging XR, knees: effusion and soft tissue swelling. Echo: no vcgcta- tions or valvular disease.

Treatment NShIDs: corricosteroids.

Discussion Juvenile rheumatoid arthritis (JRA) rnorrt commonly affects the kncc joint. Patient5 w i ~ h JM s110uld undergo periodic ophthal- molo9ic exams to carefully monitor for the onset of iridocyclitis, which can Tend to blindness.

JUVENILE RHEUMATOID ARTHRITIS > $

ID/CC A 42-vcar-old wclman presrn 1s with dysphagia, butterfly rash, arthr;ilgias, myalgias. skin stiffness, mvplling of thc fingers, proximal muscle weakness, and chronic pain in the finger joints.

HPT She ha5 had these svnlptolns intrrmittentfy over the years. hut thrLv have worsened over the past year.

RE VS: BP normal. PE: er7jthematolls ra4h over face in biu~erflv distribution: sclmdactyly; telangiectslrias in pt*ril~ngual areas; nonerosive arthritis af wrist and ankle joints; proximal: muscle weakness a~icl tendernew: weaknew of twc-k n l~~sc les : n o sensory Iow; tlnrmal rendotl reflexes; positive Rapnaud's phenomenon.

Labs Elevated ESR: diffuse hypergammaglobulinemia; positive rheumatoid factor: higll titer nf'an tir~\ic?ea~- an tihodips ti peck led p a t t ~ r n ); sirondy positive test for antibody to RNP antigen (most v i c a l finding) ; anti-Smith antibody negative: anti-dsDNA an tihndy negative; normal complernen t levels; clcwted serum CPK luvrls; rnusclr biopsy and EMG suggestive of polymvc~~itis; normal RITs.

Treatment (Inrticoqteroids and NSAlnq

Discussion Mixer[ con nett ive tiswe disorder (MCTTT)) inrlurles cllaracteris- tics nf one er more traditional connective h ~ e diseases at the same time. thus making it hard to lahcl as one or the other. T h r s t disorder.; incltidr ~ ~ ~ s i e r n i c I~tpzis rsythemat nsus.

A , - - I NIXED CONNECTIVE TISSUE D I S O R D E R

IDJCC A Fi&year+ld woman v i q i t q her phvsician complaining of severe low Ix~ck pait1 after a Call from her hccl.

HPI Onsrt c ~ f rnenopa~lse was a1 48 w a n , Tkr patient i< not receiving hormone replacement therapy; she suffered a CrdIes' fracture lasr yrar rhal is in,il~~nitecl. nil-ected history reveal5 loss of height and a mild h~mchbaek deformity.

PE Patient thin: kvphosis noted; percussioi~ orer dnrsolumhar spine exqi risitelv t e i~~ le r : rig11 [ urisl show n~alliniterl Cc ~lles' fr-acf~~r-e.

Labs Serum caIcium, phosphates. alkaliuc phosphat:~sc. xnd PTH within tlormal liini ts: densitorneby uscrl tt, qua11 tifv ostropol-oris.

Imaging XR, dol-~olumhar spine: low orvertirnl height oT L4 vertebra (duc to collapse and comprcssion frarturr) and k!phosis. nEm (cl~ral-cncrgv x-rav absorptiomemy) : rtduccrl llrltle mass.

Gross Pathology Thincortex;thmtrabecu~ae,re~cedinnumber.ren~~tin~in i~icreasetl merl~illar-v spare: obvious rtxctl~re with Ilealing and deformity; collapse of vertebral bodies with kvphoscolioris.

Micro Pathology Bonc hinpn: thin but nnrmallv formed corr ex and t rabcc~~lae; normal calcification; trabcculac very slcndrr; microfracttires ant1 Ir.ac~ure he;~ling may he evidrt~ I .

Treatment Hi~h-protein diet: calcium and vitamin D supplementation; androgens (ii~~;lholic effect oil ho~ie estrogens (.ihowri to halt ptngreqsive hone low) : exercise (weight hearing acrs as stirnr~lus to boric Lormation): bracing of spinc to prtri7cnx furthcr

I f.mctu~-rs and rlefnr-mil? in a ae~w-ely rssieoporc~tic yline; bisphnc m

C: phorlate~ nrav he arlded, especially if liormone I-eplacemer~r 3

r herapy is contraindicatcd. 5 r 0

Di~cussion O~tcoporosir is charactcrizcd hy a reduction of total skeletal ~7 < maw due to increased bone resorption (bone Formation is normal) wit11 qealer- 1 ~ 1 ~ of lraheci~~i~r thaii compac! 'bone: i t

r e s ~ ~ l t s in a prerlispnsitinn to pathnlogir fracture. Cnmmnn fracuitc sites arc the thoracic and ll~rnhar spine, distal forcarm, and prnxitnal I'Pn11tr.

Atlas Link PGP3-085

* OSTEOPOROSIS

ID/CC A 4+ear+lrE male wi th a history of Ilvpert~t~sinn develops s11dden abdominal pain ( d ~ w to rnege~lreric thrombosis) far 11ir)l-P severe than prim rpisodeq.

HPI T h e pntien t had a pr~vinus episode of lrem;ltaria with periph- 13-711 edema [hat was diagnosed as gIo t t i~~~~~lnt i rp l~r i t i s . He has a history or inte t-n~i I ten 1 (i-ver. n-lalaisc. nwalgia, arthralgia, and olller vilgur svstrmic symptom^.

PE Liverlo reticularis; ~ ~ ~ l x ~ l t a n e n u . ; nodulec or [orearms and finger pads; pairif 111, ~lmpanic ;~bclomen; p~~rp\u-ic .ipr)ts in lower legs; radial and pemneal nerve involvement ( M o h o n . E r RITIS

bl Ll L,I I I ~LICX) .

Labs CRC: mai.ked n r t ~ trophilic le~~kocytosis with eosinophilia. Elrvarrcl ESR; presencc of HRsAg; positive P-ANCA.

Imaging :It~gic~, I-rrlal: m111tipIe small aneurpms rt11c1 infarcts.

Gross Pathology Fihrii~oid I IPC~OI i~.;?ina inflammatory infil~rnle of lnrdia and adverl t i tia of sinall and rnerlillm-si~e vusscls in segmental fa512 inn, will1 t l ~ r ~ n ~ l m s i ~ ancl possi hle wnuurysm formation.

Micro Pathology Segmental area5 of fibrhoid necrosis 1virI1 nel~tl-ophilic infi l~r~tion of' artena1 wall.

Treatment Stcruirls a n d other ixn1nunosuppres5ive agents,

Discussion Polvarte t i t is t~otlow is a type 111 hypersensitivity reaction charac- tel-izrtl IJV mr~Iti+cm invc l lvr i~~un t. Renal involvement i s most cnmmon. h u ~ otht-r psesen talions incliade pericar-cl i t is, myocar- dial inhrctinn. retii~al ncclirsiu~r, and asthma.

7 PPOLYARTERITIS NODOSA

XD/CC A 70-year-old female i q seen with complair~ts of inabiJity to comb her hair, put on her coat, and get up from her chair for the past 6 months.

HPI She complains of shoulder and pelvic area stiEfness and pain (er;pecially during morning hours), fcvrr, malaise, nnrl fa1 i ~ u e .

PE VS: low-grade Cever. PI<: pallor; st if% cleliberate movements: affec red join ta ~ l i o w rest1.i~ t ~ d mor.cmcIl t: muscle strength normal: rernainrlei- of p hysic;~l exam ncsrrnal.

Labs CBC: nornochromic anemia. ESR markedly elevated: other aclli r-pllasr reactan LF such a.5 fibrinogen and a,-glollulin levels increased.

Imaging XR: normal.

Treatment Low-dose oral ~t~rnids: watch for drvclopment of @nt cell arteriti5. which threatens vision in ~ t p In one-tl>ircl af patients.

Discussion Polyrrl~.;llgia rl~emnzltica is characteri~ed by aching and morning stiffness in the shoulder and hip girdlc~, the psnximal rxtrettii- tie>, the neck, atid the torso; t h r s p e c t r ~ ~ m of disease incl~tdes giant cell arteriris. Mean age at onsct i~ 70; wnrnen are affected twice as orten as inerl. A strong association with HIADR4 has I~een ohser~ed. Snme casrs recur and some parirn ts becnine srernid rlependenr.

POCYMYALGIA RHEUMATICA

1 D/CC A ST-vear+ld white Ccmale rninplains of increasing weakness for ~cvcrxl mnn t h ~ , especi allv when climbing slain and corn bing her hair.

HPI She also corriplains of difficulty holding her neck upright. For the past few weeks. die has also t~ad difficulty swallowing.

PE Atrophy of neck, shoulder, and thigh mmscles; motor weaknew in all proximal ml~scle groups; no sensory deficit; deep tendon reflexes reduced.

Labs Markedly elevated s e m CPK levels; antinuclear antibodies (ANAS) demonstrable: elevated serum tr.ansaminasr.s and aldolasc. EMG: mal-krtll?~ increased insertional activity; polypha- sic low-arnpli~t~cle motor rmit action pnteilti~ls with al~norinally low I - P C ~ L ~ ~ tInent.

Gross Pathology MurcTe eclema progressing to r n u s ~ ! ~ au-nphy and fibrosis.

Micro Pathology Biopsv Srom rhigh niusclcs reveals inflammatory infiltrate i t i

musclc, destr-uct inn of rnufcle fibers, and prrivascular infiltrate r)f'~nor~orluclu:~r cells: rcsidual mnscle lil>ers fn~all.

Treatment Highilosr gI~icocorticoirl~: niethotrexate; azathioprine.

Discussion b l y myoci t is is frequcn tly seen as a paranenpla5tic m;lnifest;ltion of ovarian, breast, uter-ii~e, or in testinn1 malignanq. An ~nssoci- atetl r~eoplasm should a l~~i lys he s n u ~ h t .

ID/CC A 40-year-old white feinalc cornplrlirir or paleness and blllish discoloration of the hands, mainly upon exposure to cold, wi th

redness upon rewarming ( ~ R ~ N A I -n's I)HENORIE~\'OK) ; increasing pain in thr knees, elbows, and I~ancls over seberal months; aiid recent difficulty swallowing solid food.

HPI Shc alsn has masklike facies ~vi,uirh a limited range of expression.

PE Smooth, shiny, tight skin over face and fingers; erl~tna n l hands ancl reet; palpable wbcutantlous caIcinosir;; pigmentation and relangieclasias of race.

Labs CBC: anernia. IJ\.pergammaglohuline~niil; an ti-Scl-70 antibody; posi the rhetunatoid faczor. PFTs: restrictive lung disease (fi hrosis) .

Imaging UGI: low of e~ophagcal r n o ~ i l i ~ ; dilated csophaglls.

Gross Pathology PuZrnonarv fibrosis wil h "I~oneycoml~" appearance: swellitlg of esophagra1 ~rrll; ~nalabso~t ion svi~drome; cnlargeil kidneys with areas of'inl'arczion: myocardiris and pcriuarditis.

Micro Pathology Dense fibrosis of collagen tissur of dennis with loss uf appendages and epidermal atrophy: intimal thickening of blond vesscls, pr-i- marily in kidney k ~ u t also in GI tract and heart.

Treatment Supportive: cdlcium channel blockers; clmeprazolc: cisapride: penicillamine.

Discussion Progressive systemic sclerosis (PSS) may he localized or- systemic XE I

(~isceral involve n-ien l) and rrl present with calcit~ o.;i 5, m C

Raylm~rl's phenumen otl. esophageal ilir'nlvement. sclerr~dacrylr: 3

and ielang-iectasia (CREST smm~om). 3 52

Atlas Links m--lT7 PG-P2-089, PM-P3-089 KDTE MC-226

PROGRESSIVE SYSTEMIC SCLEROSIS (SCLERODERMA) C

- - - - - -

ID/CC X 60-vear-old woman presents with swelling and pain in the left knee after undergoing a major surgical procedure.

HPE She has no history of fever or trauma.

PE Luft kncc warm and crepitant ltpon movument, which is restricted and painful; positive patellin- tap irldica~eq an effi~~ion.

Labs Syno\-ial flitjcl fi-orn left knee sllows incr.eased leukocyre cnunt, pt-erlor~iinantly net~trophils; normal uric acid levels; birefringent crystals, Irrcjth free and within lcukocvtes; calcium pyrophosphate crvsials how a weakly positive birefi-ingence and are rhomboid in shape.

Imaging XR. lcfr knce: punctatc and linear calcification in articular cartilage (CHOM)ROCALCISUSIS).

Treatment Anti-inflammatory hug, including salirylates, phenylhutxone, iridomethacin. and glucocorticoids, arc ~ffcccivc tu varying rlegl-ees: join t aspiration ma!) help; triamcinoIone intra-alicu- l ady frw reqiqtanr cases.

Discussion The term "pseudogo~~t" rcfers to acute attacks of arthritis associated wit11 r he presence in llie sylov4al fluid of hirefringerent crystals, hot11 Iree and within Ieukncyte~. Pseudogout crystals show a weakly positive birefringence, whereas monosodium mate crystals show a strongly negative birefringence. Grncrally, pseudo- gour crystals are also sr~~hl>ier. and ~nc~re dismhoid tha11 umre crystals. Ratliogmphic r~idrr~cr of calcinosis (presumably CPPD crv~tals) in cartilage and ocher suucntreq is often present. The rypical pattern involves calcification in arric~ilm. cartilage, Rbro- cartilage (meniscus nf the knee, pubic spmphysis, a n n ~ ~ l u s fihl-c- SIN) . svt~uvium, fibsou s capsules, tendons, and hursac. Common sites uf involvement include the knee, shnu tclel; wrist. elbow, F~;tr~d, and ai~lrle.


PSEUDOGOUT 3'

ID/CC A 30-year-old \vIii~u female co inp la i i~s 1I1itt the fingers of Imrh hanrls hecome pale nn exposure to cold.

HPI At tirnc5. tIic pain is also prccipitatetl hv emotional stress. Slre is nor taking any rlrugs and rl.nt=q not ~uffer from any otI~us t I i aposrd ai l~ncnt (e-g., collagen varcnlar disease: thyroid, itclrt-lial, ur pituitary disraucs). Svrr~pto~n.; are r-el icved when she smiks her hai lds in warm nqafer.

PE Peripheral ~ I I I F P S pal pahle; dipping patient's hands in coId water precipitated pain and resulted in development of digital blanch- ing: rr l r . ; l rmi i~g T ; ~ ~ I S P ~ C V ; ~ ~ I O S ~ F anrl r ~ t h n r of finger<.

Labs Lahorntol-v test.? exc-111de all rauseq or secondary Rapaud's cliscncc (co l la~cn vnscular d i ~ e a s ~ ~ and 't>lon~l rlvscrxsias) .

Micro Pathology Arrrrial wall ctlnnges it1 advanrecl starc nCdisease.

Treatment Protect hands anrl feer from expowre to cold: clnlg therapy with ii calcit~rn clinr~ncl blocker stlch aq rlikilipir~e or a synpatholvtic agrni s11ch as res~r-pi i ie or- gllanethirline.

Discussion Primary EZ;ll;naucI's phenomenon. or Riyna~rrl's disuase, is a \.dr;uxpastic clisr>rrle~; wllere;ts secu~i(l;~~-v Rayii;ii~d's pl~ennrnenon occilrs as a complitation of svste~nic rli5ease such as scIuroderma, swtcrnic lupus cr)rthtm;~tosus. and rclatc-ct i r n m ~ m o l o ~ c disorrlers. M:orr~ni are af f rc~er l approxirnatrly i i v r times moi-P than men, and the age at pl-esen tation i~ 11st1a1ly he t~reen Sfl and 10 vears. Thc iingers arc irlr-oTvcd murc frrqltenrlv th;111 r l ~ c lees.

RAYNAUD'S DISEASE

ID/CC .A 'LS-~~ar-old man pl-e7el-r t4 with hilatcraI conjrmctivids, painful swelling of the right knee, bila~eral heel pain. ar~d painless ulcers on 'his penis.

HPE Hr rvas diagnnwd and treaied for nongonocnccal uret hriris 1 week ago.

PE Bilateral coi~j~inctiviuis with atl~eriol- uveitis; circinate balaaitis; kerato-blmmorrhagieum on palms and soles; arthritis of' right knee and ankle.

Labs HU-R2'7 positive; s y n ovial fltiid rcvtals monocvtes with ptlagm mtosed neutropliils (W:ITER (:EI ,IS) : rheumatoid factor negative; rlrvatcd ESR.

Imaging XR, ri~llr knee and ankle: presence of joint effusion.

Treatment NSAIDF are mainslav of thcrapv: treat chlainyclial urethri~is with clu~\~cycline.

Discussion K~irer's syndromc is an HL11-B2'7-associated seronrgativc spondvloarthmpat hy that is sccn ;rlmosc exclu~ively in males arzd il; associated with conj~lrlctivi~is, urethritis. arthritis, and heeI pain. The conclition has traditionally heell classified as an STD. Imt it ha< alsn occurred follo-rving regional enteritis with

R E I T E RFS SYNDROME

Labs

Imaging

Gross Pathology

Micro Pathology

Treatment

Discussion

Atlas Link

A 47-year-~ld white female visitq her Camily doclor cnmplaining of' painfill p well in^ of lhe r i ~ h ~ knee.

She has a history of chrunic pail1 along with morning stiffness

in the hand joi nts laqting for a1 least 2 hours.

Symmetrical deforming arthmpathy (ulnar duda tir>ri ) ; 4- tissue s~ell inq a n d tcnderncss iri PI-oxi~nal it1 terphalangral and met;~car-pophalangei~l (MCP) joint: warring of smaIl muscles of hand; flexion oC MCP join t; hyperexi ension of proximal inler- phnlnn~ral (PIP) joint and flexion of distal i~~lerphalangeal (DIP) joint (SM'AN-NFI:K ~ E I ; ~ ~ I I T Y ) . ; eff ' i tsi~~~ nil right knee with overlying skin redness ilild increased t e ~ ~ ~ l > e ~ - a n ~ r e : subcutanecnlg

Incrra~ed ESK: inci-eased protci~l iind white count in thc synovial fluicl: positive rheumatoid factor (IgM or IgA against I@) : pnsitivc antiutrcleai ant iborlies (ANAS) ; polyclcrnal gmmopathy ; associil aed will1 HLA-DR4.

SK, plain: n a r r o ~ i n g of joint sparer: rirsion af,jnint (4u~;noas) ; demintlraljzation an tl l>nne rrosions; ,jnxta-t-artict 11ar nsteaporosis.

Rone ernsion with ankylosis; pericarrlitis, pleuritis; s ~ r b c ~ i ~ n e o u r nodufes with gcarlt~lo~na hrmation.

Plasma cell inLl tration of qmoiial mernhranes (suvorq~rs) wit11 rlertruction of articular cartilage, lendons, and ligarne~lts by thickened, infl arned synovial &sne ( I~ .L~NLIS) ; fibrosis.

Physical therap): thermal compresses, splints; NSAIDq; mcthutrcxate; gold: chlornquine: corticustcruids: other im~~~rinc>~nppr-essanrs: surgery.

r 0

Rhet~matnirl arthritis is thc most common autotmml~ne disease. Gl < Cktllar involvement is sfen in 5%) ooT rases; neurologic involvement of f l ~ t carpal t l~nnel cat1 he a romplication.

? RHEUMATOID ARTHRIT IS

Labs

Imaging

Micro Pathology

Treatment

Discussion

A 47-vear-olr! wnlnan visits h r r hcalth carc center complaining of dryness of the mouth (XF.ROSTC)MIA) ancl a gritty sensation in her eyes with dryness ( X ~ K O I ~ W I - b u h 1 1 . 4 ) .

811~ has been Iivpcrtrnsive for 20 years and liar su~t'fel-ed from long-standing rheumatoid arthritis, for which she has been treatcd wit11 NShIDs.

Filamentous keratitis with areas of denuded corneal epithelk~rn ( I < ~ ; . R A T O ~ : O ~ ~ ~ ; C T ~ ? T ~ S S~CLI) on slir-lamp examinat ion with rose Fwngnl dyt staining oC cornea; diminished tear famation as meas~irerl rjn strip of filter paper, with one end of paper placed inside 1owt.r evelid (Sc:tu~n{c~ ~17.s-r) ; parotid enlargement; excesnive!y clry mouth with ahundan t dell tal raries; charact~ris- tic w a ~ ~ - n e c k deformitieq of hands and 1111iar deviation (due to

Inng-standing cleforming rhcurnatnicl arttll-iris).

Low saliva flow rates will1 lemon juice stimulation (< 0.5 mk/ min j : h~crgan~rnaglo~~uFin~mia: positive antibodies to IgG dobdhw (RI r~,tYvl,4TOrn L\C;TO K) and antinuclear an tihodies ( ANA5).

Sialc>griiphy (x-rays fnllowing cani~lrlation ancl contrast injection of parotid ducts) : distortion of normal arlborization pattern. Nuc: impaired salivarp filr-icrion.

Salivary and lacrimal glands show inflam malorv infiltration with T cclls, I3 cells, and plaljrn;~ culls, with predominance olCD4+ T rells; d u d obstruction with glandular acirlar tisst~c atrophy with rat5 change.

Artificial tear preparations, increased and frequent oral intake of fluids. careful duntal hygiene, plaque ronrrxi programs. fli~oridc i~pplication.

Sjiigr-en'\ syndrome is dcfined as autoi tnnlune destl-tiction of salivary ancl lacri~nd glands; i r may he primary or associated with orher a ~ ~ l o i r n n ~ ~ r n e cliscases.

ID/CC An I Ryar-nId white female prrscnts 14th a rnalar rash that is uxaccrhatcd by sun r-xposuru ( ~ ~ w ' r o s ~ ~ s r m ~ r ; ) } ;IS w ~ 1 1 as with arthl-alg-iaq and jaint stif€ness invoIving her ankles, wrists, and knee joints; she also complains o f decreaqecl risual acuity. anorexia. weight loss, malaiqe. and weakness.

HPI She has a history of hcrnatl~ria and n u his~orv of rlr-uz intake prior to thc onsct of synplonls.

PE VS: hyperrensio~i (RP If{)/ 14)O) . PE: pallor: malar rash; pninfirl restricrion of movcnlent of ~ z ~ i s t , knrc. and ankle -joints; no c~hrious defurmiv; ruhitisl~ r-xridates in cytoid I)orli~s nn f~ir~duscopic exarn.

Labs CBC: Coom hq-posi tive anemia; neutropenia: thrornbocytopenia. Decreased C 1 q, C2, C4; positive antinuclear antibodies (ANAs), anti-native DNA, and antism antibodies: positive I-E cells; false- pr~sitive VDRL clue to an~iphnspholipid antihorlies. LJA: protein- ul-ia: RBCF anrl KBC casts.

Imaging XR, plain: n o et-wive cli;i~~ges. Kchn: n n veg~tatinns seen on

V ~ ~ I V ~ S (YS. endncardi t is) .

Gross Pathology Serosiris; prriuarrliris: ple~~ritis; splenntnegalv; I~yperkeratotic, ~ r y t h e ~ n a t o u s plaqil~cs.

Micro Pathology Thickening of hasemcnt n~rrnhrane c l r l renal biopqy; mesangial proliferatiori; thickened capillary walls, rreating "wireloop" appearance: clil'f~lse praliCcrative glomerr~lonephri tis: i t n m ~ ~ n e c o n ~ p l ~ x deposition in skin N ~ L I I lyniphocytic ii~filtration; vasculi- x tis with fibr-inoid ~~ecrosis of small artel-ips: almoqt any organ C

U

Treatment I-Iigh-rtos~ carticnsteroids for prolonged pcriotls; afternative cs r rlr~igs: chloroquine; qclophosphamidf as trealrncnt for lupus ~1 -< nephritis.

Discussion Sstcmic Er~pus cryth cmatosr~s (SLF.) i s a type In hypersensitivity reaction. Trnrn~tt~e colnples va sc~~ l itis ir the basic pathologic lesion; can he drug-induced {e.g., hydralazinc, pr-ocainamidu, isonia~idl..

Atlas Links Em PM-P3-095 EEKE IMZ-056

@ - I SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

ID/CC h $45-year-old white nvale cornplairls of cllronic nasal congeqtion and discharge over 111c pixst 5 months.

HPI Ten ~ ; L J T F ago he d~veloped an earache and cough along with bloodv sputum production, dvspnea, musclc pain, rcd eyes,

fever, and night swrat.;.

PE IIr.ied-~~p cl-lls~s of inuc~is in congestive na~a l inucosa wit11 shnllow ulcers and perroration of the nasal septum; sibilant rales disseminated in Iung- Gelds.

Labs CRC: mild anemia; noder rate leukoqeosis. UA: numerous RBCs; red cell casts and gt-anlllal- castq in 11rine. Positive cytoplasmic antineutrophilic antibodies (GAVCA) in serum.

Imaging CXR: I~ila~ernl scar~er-ed small nodular den~itje5 with no hilar adenopat hy (w. sarcoid) .

Gross Pathology Gmnl~lorna for-rr~a t ion ill Ilings; vasczilir is and inflammation involving upper reviraiory tract, lungs, peripheral arteries. and kidneys .

Micro Pathology Focal r~ersotizirag vasculi t i s i t~volving small vessels; grat11110rnas ant1 urusccntic glomcl-uloncphritis.

Treatment Immunosupprcssivc therapy with stcroids and cyclophos pliamicle.

Discussion Wegerit.~.\ gra~irlulomatosis is a systuniic autoimmlme vasmHtis that cnn\ists of necrotizing vasc~~li~is a11c1 necrot izing gra~lulw mils of the lungs and airwavs, as well as a uecroti~ing glomeruli- us. C;:.Zh'Ch is Fern in lhe n~ajorily of patients and serves as a marker oC disease activiry.


WEGENERrS GRANULOMATOSIS

lD/CC ,4 5X-vear-old mate complains of headache. a n x i ~ y . ~hor tness of breath. and incrcascd slerpin~ss (SO~W(II.EXI:E) u-l~il~ ' exp~Ti- c~lcing kin acute exacerbation OF COPD.

HPI The paticnt is a chronic smoker and aIso complains of recent

blurring of vision. He has a histoy of rpisorlic shortness or I~reilth, mucoicl cough, and occasional wl~eezing (consisteilt with prednrninantly bronchitic COPD) h u ~ tin hiqtory nf neurologic defiri t, previnns hypertension. or diabrtes.

PE VS: ~arlivcarctia: tachxpnea: milcl ystolic hvpertensirrn; no frvcr. PE: anxious and in modcrate respiratrrry distrc~~; llsing accessorv musclcs of rcspiratior~ wi I 11 p l-n longer1 expiration: mild central cyanosis and pallor; no clubbing: r xhern i r i~s warm: flapping tremor of hand (.IS ~'EKISIS) ; bounding pulses (dur to

high volumc) ; F~induscopy reveals early papilledema: chcst hm-I-el-shapcd ~ i t h bilateral sho~~ch i anrl occa%onal rales; nu focal neurologic dcfi cits.

Labs ,ZRG.F: hypoxia, hypercapnia, and partially compensated respiratory acidosis. CBc: pulvcythelnia.

Imaging CXR (PA view) : inct-eased hronchcxascular markings (dirty liing fields) .

Treatment Lowdose continuaus oxygen inhalation and, if required, mechanical ventilation to reverse acidosis; hrcxtd-spec~rurn an tibiotirs, broi~chodilators (iprat rcrpi~lttl I~roinirle anrl svmpathorn iineticq), and st~roids are ~ ~ s e r l in COPD patierits.

Discussion Dyspnea and Ileadachc arc t11c cardinal svmptoms of hvpercap nix. F-Iypcrcapni~ ~ I F c ) prc)d1ices il varie~ y of tlrllrologic al>tlor~nali ties: sv~nptoms include somnolcnce. blr~r,rcd vision, rcstFcssness, and anxiety that can progress to tremors, asturixirj, drlirium, and coma. Supplemental nxygen shn~hd hc uscd sparingly to avnirl inct-easing Pao,, which rcrnovcs the hypouic respiratory stimulris and leads ro respi rat al-y rlepression.

A CARBON D IOX IDE NARCOSIS

40965358 Underground Clinical Vignettes Pa Tho Physiology III

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Transcript of 40965358 Underground Clinical Vignettes Pa Tho Physiology III