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39. Xiao F, Tseng MY, Teng LJ, Tseng HM, Tsai JC: Brainabscess: clinical experience and analysis of prog-nostic factors. Surg Neurol 63:442-449, 2005.

40. Yang SY, Zhao CS: Review of 140 patients with brainabscess. Surg Neurol 39:290-296, 1993.

41. Yang SY: Brain abscess: a review of 400 cases. J Neu-rosurg 55:794-799, 1981.

U

Hector Roldan1, Mario Garcia-Conde1, M

patient was a young adult and hydrocepha-

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onflict of interest statement: The authors declare that therticle content was composed in the absence of anyommercial or financial relationships that could beonstrued as a potential conflict of interest.

his article is an expanded modification of a posterresented at the 77th Annual American Association ofeurological Surgeons meeting, San Diego, California,

SA, May 2– 6, 2009. A

ariano Ginoves-Sierra1, Rosa Rodriguez2

can was taken, which showed no abnor-

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WORLD NEUROSURGE

eceived June 2, 2010; accepted November 30, 2010

itation: World Neurosurg. (2011) 75, 5/6:716-726.OI: 10.1016/j.wneu.2010.11.043

ournal homepage: www.WORLDNEUROSURGERY.org

vailable online: www.sciencedirect.com

878-8750/$ - see front matter © 2011 Elsevier Inc.

ll rights reserved.

Acute Hemicerebellitis with Obstructive Hydrocephalus in a Young Adult

INTRODUCTION

Acute cerebellitis is an inflammatory reac-tion of the cerebellar parenchyma caused bydirect damage inflicted by a microorganismor by an autoimmune response after an in-fection or a vaccine (8, 9, 13, 17). Its etiologyis mainly viral (especially varicella-zosterand Epstein-Barr), although some bacteriaand even parasites have been involved (7, 9,14, 20, 21). Both cerebellar hemispheres aresymmetrically affected and the syndromehas usually few complications (2, 12). Thepresentation of acute cerebellitis as a selec-tive inflammation of one cerebellar hemi-sphere is exceptional, and only 13 caseshave been described so far, all in the pediat-ric population (11, 12, 22). We report a newcase of this rare entity documented on pa-thology, with the unique features that the

Key words� Hemicerebellitis� Hydrocephalus� Infection� Magnetic resonance imaging

Abbreviations and AcronymsCSF: Cerebrospinal fluidCT: Computed tomographyMRI: Magnetic resonance imaging

From the 1Department of Neurosurgery and2Department of Pathology, Hospital

Universitario de Canarias, Tenerife, Spain

To whom correspondence should be addressed:Hector Roldan, Ph.D. [E-mail: hecroldan@gmail.com]

Citation: World Neurosurg. (2011) 75, 5/6:726-730.DOI: 10.1016/j.wneu.2010.10.040

Journal homepage: www.WORLDNEUROSURGERY.org

Available online: www.sciencedirect.com

1878-8750/$ - see front matter © 2011 Elsevier Inc.All rights reserved.

us ensued. We also discuss the diagnosticnd therapeutic problems resulting fromhe scarce experience.

ASE REPORT

18-year-old man was admitted to the Neu-ology Department at another hospital with3-day history of headache, dizziness, and

ntermittent diplopia. No fever, previousistory of infection, vaccination, or drug in-

ake was registered throughout the previ-us month. The neurologic and systemicxamination recorded at that hospital wasormal. A computed tomographic (CT)

� BACKGROUND: Acute cerebellitispopulation, usually of viral or autoimmugood prognosis. Only 13 cases of unilateTo the best of our knowledge, this is thereported in a young adult that causetreatment.

� CASE DESCRIPTION: We report an uprevious infectious symptoms, who desecondary to hemicerebellitis. A ventrcerebellar hemisphere was later explorean inflammatory reaction of the leptomeand antivirals, the neurologic symptomsin 2 week’s time.

� CONCLUSIONS: Hemicerebellitis musis of cerebellar mass lesions, even innificant hydrocephalus may develop. Desuspicion is still required to accurately dseems to be localized meningitis. The echyma supports the usefulness of cortico

alities, and a lumbar puncture revealedymphocytic pleocytosis (450 white bloodells/mm3, 0 red blood cells/mm3, 80%ymphocytes, 189 mg/dL of proteins, and 50

g/dL of glucose). The cerebrospinal fluidCSF) culture was negative. Treatment withcyclovir 900 mg IV q8h and cefotaxime 2 gV q6h was started. No corticosteroids weredministered by then.

The patient initially evolved poorly, com-laining of progressive headache. Three daysfter admission, another CT scan showedriventricular hydrocephalus, and the patientas transferred to our hospital. On arrival, theatient was conscious, but slow and mildly

rare entity, described in the pediatricrigin, bilateral and symmetric, and witherebellitis have been reported thus far.ase of hemicerebellitis in the literatureute hydrocephalus requiring surgical

ual case of an 18-year-old man, withoutped acute obstructive hydrocephalusr drainage was placed. The affectedd biopsied, and edematous tissue, withes, was obtained. With corticosteroidsppeared and the MRI evolved to normal

e considered in the differential diagno-g adults. Although rare, clinically sig-the availability of MRI, a high index ofose this entity. The histologic substrateatous reaction of the cerebellar paren-n the treatment strategy of this disease.

is ane oral cfirst cd ac

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isoriented. He had neither focal deficits (cer-

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HECTOR ROLDAN ET AL. ACUTE HEMICEREBELLITIS WITH OBSTRUCTIVE HYDROCEPHALUS

ebellar examination was normal) nor menin-geal signs. A new non-enhanced CT scan re-vealed triventricular hydrocephalus and anasymmetric compression on the roof of the

Figure 1. Nonenhanced computed tomographic striventricular hydrocephalus with asymmetric co

Figure 2. A and B, Axial and coronal T1-weightedgadolinium depicting a left cerebellar hemispherthe coronal image, the foliar pattern following thbest appreciated. C, Axial T2-weighted MRI discwhich is the real origin of the mass effect. D, Aof admission showing absence of foliar enhance

biopsy area.

WORLD NEUROSURGERY 75 [5/6]: 726-7

ourth ventricle (Figure 1). A ventricularrainage was inserted and high-dose dexa-ethasone was added to the antiviral and an-

ibiotic, which were not discontinued.

on admission at our institution, showingsion of the 4th ventricle.

etic resonance imaging (MRI) scan withss with irregular peripheral enhancement. Inmmatory foldings of the pia-arachnoid can beincrease in the cerebellar water content,-weighted MRI with gadolinium after 4 weeks

. There appears the porencephalic cavity at the

s

30, MAY/JUNE 2011 ww

Two days later, the patient was com-letely asymptomatic. An MRI disclosedn edematous right cerebellar hemisphereith irregular gadolinium contrast en-ancement (Figure 2A–C). CSF samplesere collected from the ventricle and pro-

essed for serology (syphilis, toxoplasma,pstein-Barr, varicella-zoster, herpes, andorrelia) and culture of bacteria, mycobac-

eria, and fungi. The same serologic testsere carried out in blood and HIV was de-

ermined. All the CSF and blood tests wereegative, as well as the HIV. The Mantoux

est was also negative.Surgical exploration through a retromas-

oid approach was undertaken. The cerebel-ar surface appeared congestive and norain tumor was encountered. The petrousura was intact, and no communicationsith the middle ear or the mastoid cells

ould be found. The cerebellum was biop-ied, and the biopsy specimen showed cer-bellar tissue with normal architecture andarked edema, meningeal thickening, with

ascular congestion. The most striking fea-ure was a prominent inflammatory in-ltrate in the meninges consisting mainlyf lymphocytes and some leukocytes (Fig-re 3). Immunohistochemical stains re-ealed a mixture of T- (CD 3�) and B-CD 20�) lymphocytes. In addition, the T-ymphocytes were either CD4� or CD8�,

ith no restriction (Figure 4). There was novidence of malignancy.

The patient evolved very satisfactorily.he ventricular drainage could be removed

n a week. The acyclovir and the antibioticere withdrawn after 3 weeks and the cor-

icosteroids were slowly tapered off. Thedema and the enhancement clearly di-inished on MRI in 2 weeks’ time, and the

euroimaging after 4 weeks was normal ex-ept for the surgical porencephalic areaFigure 2D). No cerebellar atrophy could beemonstrated. After 3 years’ follow-up, theatient leads a normal life, including heavyports.

ISCUSSION

nilateral cerebellitis or hemicerebellitis isxceedingly rare, and only 13 cases haveeen published until now (Table 1). Ourase is the oldest one reported so far. Onlyhe cases reported by Omeis et al. (18) and

elek et al. (16), aged 16 and 15 years, re-

cans

magnic mae inflalosingxial T1ment

pectively, were the closest to our case.

w.WORLDNEUROSURGERY.org 727

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HECTOR ROLDAN ET AL. ACUTE HEMICEREBELLITIS WITH OBSTRUCTIVE HYDROCEPHALUS

The etiology of hemicerebellitis is relatedto an infectious, postinfectious, or postvac-cination disorder (17). However, two of thepublished cases had absence of fever as well

Figure 3. Histopathologic examination of the biopCerebellar tissue with severe mononuclear inflamvascular congestion.

Table 1. Features of Hemicerebellitis: Re

Cases References

1 Iester et al., 1995 (11)

2 Sawaishi et al., 1999 (20)

3 Usano et al., 2000 (23)

4 Sekhara et al., 2001 (22)

5 Omeis et al., 2002 (18)

6 Jabbour et al., 2003 (12)

7 De Bruecker et al., 2004 (4)

8 García-Cazorla et al., 2004 (6)

9 De Medonca et al., 2005 (5)

10 De Medonca et al., 2005 (5)

11 Melek et al., 2006 (16)

12 Martinez-Gonzalez et al., 2006 (15)

13 Oguz et al., 2008 (17)

14 Present case

728 www.SCIENCEDIRECT.com

s a previous history of even mild viral ill-esses or vaccine administration, like in ouratient (5).

The neurologic clinical presentation can

ecimen (Hematoxylin-eosin stain 60�).ory infiltrate, meningeal thickening, and

of the Literature

Age (years) Hydrocephalus Surgery

5 No

9 No

6 No

9 No

16 No

13 No

4 No

5 No

5 No

11 No

15 No

Not reported No

13 Mild

18 Yes

WORLD NEUROSURGE

ollow roughly two patterns: 1) headachend vomiting due to posterior fossa hyper-ension or 2) cerebellar hemisyndrome withr without hemiparesis that is presumablyaused by compression of the pyramidalract below the cerebellar decussation or byrossed cerebellocerebral diaschisis (6).nly three cases experienced posterior de-

erioration, and two ended up operatedpon to relieve pressure in the posteriorossa (12, 18, 20). The primary symptomsf our patient pointed to posterior fossaypertension, but deterioration was causedy hydrocephalus, which was relieved onlyith a ventricular drainage. Posterior fossa

urgery was undertaken to explore and ob-ain a biopsy of the cerebellum in order tochieve accurate diagnosis. No clinicalmergency forced open surgery, as the pa-ient was already asymptomatic.

CSF samples can be normal or show lym-hocytic pleocytosis (4, 20). Acute cerebel-

itis has been related to measles, mumps,ertussis, scarlet fever, diphtheria, and

yphoid fever as well as infections due tooxsackievirus, poliovirus, echovirus, vari-ella-zoster virus, Epstein-Barr virus, her-es simplex virus, Borrelia burgdorferi,oxiella burnetii, mycoplasma and malaria

14, 20, 21), but, in the cases reported ofemicerebellitis, blood and CSF cultures,erologies and PCR are usually negative, ex-

iopsy Available Treatment

o No

o Antibiotics

o No

o No

es Steroids

es Steroids

o Steroids

o No

o Acyclovir

o Not reported

o Steroids � mannitol

o Not reported

o Steroids

es Antibiotic� antiviral�steroids� external

sy spmat

view

� B

N

N

N

N

Y

Y

N

N

N

N

N

N

N

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HECTOR ROLDAN ET AL. ACUTE HEMICEREBELLITIS WITH OBSTRUCTIVE HYDROCEPHALUS

cept for one patient in which Coxiella burnetiiwas isolated (20). No cultures specific forviruses were available in our cerebellar tis-sue samples, and blood and CSF serologieswere negative. Our data exclude tumor (themixed population of T and B mature cells onthe biopsy specimen specifically excludeslymphoma), but we cannot add up to theetiology of the condition.

Because of the rarity of this entity, andthe absence of fever or meningismus, it iscomprehensible that our first diagnostichypothesis was brain tumor. This consider-ation led us to indicate a surgical biopsyafter initiating corticosteroids. Other diag-nostic options include cerebellar infarcts,acute disseminated encephalomyelitis,vasculitis, drug-related inflammatory pro-cesses (e.g., drug intoxication or chemother-apy), and Lhermitte-Duclos disease (2, 5). Allthese disorders could be easily excluded in our

Figure 4. Immunohistochemical phenotype of theBoth T (CD3�) and B (CD20�) cells are abunda

case through clinical history or biopsy. The o

WORLD NEUROSURGERY 75 [5/6]: 726-7

ornerstones of diagnosis are a high index ofuspicion and contrast-enhanced MRI, as ini-ial CT scans may be normal (5). Literaturehows a few cases with only cortical T2WIbnormalities (6, 11, 22, 23), whereas the ma-

ority of patients disclose both cortical andubcortical signal changes (4, 5, 12, 16-18,0), like in our case. The foliar enhancing pat-ern (pial contrast enhancement along theerebellar folia) has been advocated as spe-ific of cerebellitis (3, 19). Should we have hadhe suspicion of hemicerebellitis in our

inds, close examination of the MRI wouldave unveiled focal foliar enhancement with aisproportionate increase in the cerebellarater content on T2WI and absence of a well-elimited mass. This fact, together with theapid onset of symptoms, could have drawnur attention to hemicerebellitis as the firstiagnostic option, leaving the brain tumor tosecondary place and possibly delaying bi-

matory infiltrate.per panels), and a

balance of CD4� and

psy until a second control MRI would have

30, MAY/JUNE 2011 ww

een taken. Undoubtedly, MRI diffusion andpectroscopy, as recently published, may bealuable adjuvant to diagnosis (16, 17).

Only two biopsy samples are available outf the thirteen cases reported in literature (12,8). In both cases, the presence of edema andhe lymphocytic leptomeningeal infiltrate areoincident with our case. Interestinglynough, the term hemicerebellitis used in casesike ours refers to a clinical and radiologiconcept (mostly related to the cerebellarwelling that causes the mass effect and theydrocephalus) rather than a pathologic one,s the histologic substrate seems to be local-zed meningitis. The MRI manifestation ofhis localized meningitis is pial enhancement.here is no evidence of inflammatory cellular-

ty in the cerebellum, but only an edematouseaction. This fact is coincident with the highignal on T2WI and the absence of parenchy-al enhancement.

T cells was found.

inflam CD8�

Cerebellitis are usually self-limited and

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HECTOR ROLDAN ET AL. ACUTE HEMICEREBELLITIS WITH OBSTRUCTIVE HYDROCEPHALUS

benign (2, 8, 12), and only four cases ofsecondary hydrocephalus have been pub-lished (1, 2, 10). To our knowledge, no othercase of hemicerebellitis leading to a clini-cally significant hydrocephalus has beendescribed thus far. Only Oguz et al. (17)reported on mild radiologic hydrocephalusin his case, which resolved only with ste-roids. This was also the only case of hemi-cerebellitis recurrence, which curiouslyenough took place in the other cerebellarhemisphere. The role of CT scan remains torule out complications such as hydroceph-alus.

The fate of most cerebellar hemispheresaffected is to evolve to parenchymal atrophy(6, 11, 15, 17, 18, 20, 22, 23). Cases of resti-tutio ad integrum or a residual slight T2WIhyperintensity have also been reported (4,5, 12, 16). In our patient, no clear atrophyis present. Nevertheless, surgical poren-cephaly may obscure this consideration.

In most cases of cerebellitis, the onlytreatment used is high-dose corticosteroidsin short courses without antivirals (2, 7, 12).Gohlich-Ratmann et al found that the finaloutcome was better with corticosteroids(12). As our pathologic report reinforces,the cerebellar edema is the basis of the masseffect. Whether the origin of these changesis direct damage or an autoimmune re-sponse to the systemic infection, we cannotconfirm. In any case, corticosteroids seemto inhibit the autoimmune cascade and tostabilize the blood– brain barrier, thusmaking brain edema wear off, and they areconsidered the cornerstone in the treatmentof cerebellitis.

CONCLUSIONS

Hemicerebellitis with atypical clinical pre-sentation (no fever and no previous historyof infection or vaccination) and pseudotu-moral appearance on MRI is an elusive en-tity that must be included in the differentialdiagnosis of cerebellar masses of youngadults. Even though MRI is the image ofchoice, CT scan must be taken on an emer-gency basis to rule out complications suchas hydrocephalus. MRI T1- and T2WI andhistology show that in hemicerebellitis,there is a localized inflammatory reaction of

the leptomeninges together with edema of

730 www.SCIENCEDIRECT.com

he underlying cerebellar tissue. This factupports the usefulness of corticosteroidherapy in the management of this entity.

CKNOWLEDGMENTS

e thank Victor Garcia-Marin, Ph.D.,hose advice regarding the final correc-

ions to the manuscript was of the utmostmportance.

REFERENCES

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5. Martínez-González MJ, Martínez-González S, García-Ribes A, Mintegi-Raso S, Benito-Fernández J, Prats-Viñas JM: Acute onset ataxia in infancy: its aetiology,treatment and follow-up. Rev Neurol 42:321-324,2006.

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onflict of interest statement: The authors declare that therticle content was composed in the absence of anyommercial or financial relationships that could beonstrued as a potential conflict of interest.

eceived 30 March 2010; accepted 19 October 2010

itation: World Neurosurg. (2011) 75, 5/6:726-730.OI: 10.1016/j.wneu.2010.10.040

ournal homepage: www.WORLDNEUROSURGERY.org

vailable online: www.sciencedirect.com

878-8750/$ - see front matter © 2011 Elsevier Inc.

ll rights reserved.

RY, DOI:10.1016/j.wneu.2010.10.040