Acute Flaccid Paraplegia
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Transcript of Acute Flaccid Paraplegia
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Approach to a child with
weakness
Dr. Pushpa Raj Sharma
Professor of Child Health
Institute of Medicine
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Weaknessparesisparal!sis
Upper Motor Neuron.Upper Motor Neuron.
Lower Motor NeuronLower Motor Neuron
MyopathicMyopathic
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Pattern of weakness
Si"n #M$ %M$ M!opathic Atroph! & ''' '
(asciculations & ''' &
)one ''' & '&
Distri*ution Re"ional Se"mental Pro+imal
)endon refle+es ''' &&& '&
,a*inski-s si"n ' & &
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Important fact
When there is a discrepanc! *etween the
histor! and ph!sical findin"s/ it is usuall!
*ecause the patient complains of
weakness/ whereas s!mptoms are
actuall! due to other causes.
Child Abuse may be the cause.
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Motor $euron Disease
As!mmetric #pper and lower motor neurones
Am!otrophic %ateral Sclerosis 0A%S1
Sporadic A%S
Hereditar! A%S
Supero+ideDismutase2 Chromosome 342 Dominant
5ther A%S Recessi6e7 Dominant
Multi&s!stem disorders
A%S with5phthalmople"ia78+trap!ramidal
Disorders Pol!"lucosan*od! disease
Motorneuronopath!
with cataracts and skeletal a*normalities
Multiple s!stem atroph!
http://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/autonomic.htmlhttp://../Journal%20articles/autonomic.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/spinal/als.htm -
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S!mmetrical and pro+imalSM$ Chromosome 9:2 Recessi6e
Andro"en Receptor 0,ul*o&spinal Muscular Atroph!1
;&linked2 Recessi6eHe+osaminidaseA 0)a!&Sachs1 Chromosome 492
Recessi6e
Hand weakness
,ul*ar in6ol6ement
Motor $euron Disease
http://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/synmot.html -
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%ower motor neurones onl!< Distal %ower Motor $euron 0%M$1 S!ndrome
I"M6s=M4"an"lioside
I"M6s=al$Ac&=D4a"an"lioside
Also see Multifocalmotor neuropath!
< Pro+imal %ower Motor $euron S!ndromes
,rachial am!otrophic diple"ia
> Associated with I"M 6s asialo&=M4
Rare #pper ? %ower lim*s with anti&Huanti*odies
Motor $euron Disease
http://../Journal%20articles/antibody/pnimax.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/antibody/pnimdem.htmlhttp://../Journal%20articles/antibody/pnimdem.htmlhttp://../Journal%20articles/nanatomy/proxmot.htmlhttp://../Journal%20articles/antibody/sneuron.htmlhttp://../Journal%20articles/antibody/sneuron.htmlhttp://../Journal%20articles/antibody/sneuron.htmlhttp://../Journal%20articles/antibody/sneuron.htmlhttp://../Journal%20articles/antibody/sneuron.htmlhttp://../Journal%20articles/nanatomy/proxmot.htmlhttp://../Journal%20articles/antibody/pnimdem.htmlhttp://../Journal%20articles/antibody/pnimdem.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/over/labdis.htmlhttp://../Journal%20articles/antibody/pnimax.html -
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%ower Motor $euron S!ndrome without anti*odies
0PMA1
A%S 6ariants
< Hereditar!
< Sporadic
(ocal motor neuron disease
< MonomelicAm!otroph!
< Paraspinousmuscleam!otroph!
< Cer6ical am!otroph!
Motor $euron Disease
http://../Journal%20articles/synmot.htmlhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/musdist/paraspin.htmhttp://../Journal%20articles/musdist/paraspin.htmhttp://../Journal%20articles/musdist/paraspin.htmhttp://../Journal%20articles/musdist/paraspin.htmhttp://../Journal%20articles/musdist/paraspin.htmhttp://../Journal%20articles/musdist/paraspin.htmhttp://../Journal%20articles/spinal/als.htmhttp://../Journal%20articles/synmot.html -
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Paraneoplastic motor neuro0no1path!
< Mild weakness With l!mphoma
< Se6ere weakness With *reast cancer
Hopkins@ s!ndrome Acute post&asthmatic
am!otroph!
Polio 7 Post&polio s!ndrome
SM$3 0SM$C1 deletions
$eurofi*romatosis/ )!pe 39
Motor $euron Disease
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Symmetric & Proximal Hereditar! Spinal
Muscular Atroph! SM$ Chromosome 9:2 Recessi6e
Andro"en Receptor 0,ul*o&spinal Muscular
Atroph!1 ;&linked2 Recessi6e
He+osaminidase A 0)a!&Sachs1 Chromosome
492 Recessi6e
Motor $euron Disease
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Rapid onsetAcute A+onal Motor $europath!
0with Camp!lo*acter jejuni or serum I"= 6s=M41 Poliom!elitis Porph!ria
t!pes cause neurolo"ic attacks< Acute intermittent
< Barie"ate Porph!ria
< Coproporph!ria
< &amino&le6ulinic acid deh!dratase deficienc!
#rine All t!pes produce increased &amino&le6ulinic aciddurin" attacks
Motor $euron Disease
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Painful
Ac:uired
5thers
Motor $euron Disease
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Acuired !oxic %ead2 Dapsone2 ,otulism2 )ick Paral!sis
"n#ectionsPolio
West $ile
Central 8uropean encephalitis
Creutfeld&Eaco*< Am!otroph!
< Pol!neuropath! 0F Dem!elinatin"1
Motor $euron Disease
http://../Journal%20articles/nother/toxic.htmhttp://../Journal%20articles/nother/toxic.htmhttp://../Journal%20articles/nother/bot.htmhttp://../Journal%20articles/nother/toxic.htmhttp://../Journal%20articles/spinal/infect.htmhttp://../Journal%20articles/nother/infect.htmhttp://../Journal%20articles/nother/infect.htmhttp://../Journal%20articles/nother/myelin.htmlhttp://../Journal%20articles/nother/myelin.htmlhttp://../Journal%20articles/nother/infect.htmhttp://../Journal%20articles/nother/infect.htmhttp://../Journal%20articles/spinal/infect.htmhttp://../Journal%20articles/nother/toxic.htmhttp://../Journal%20articles/nother/bot.htmhttp://../Journal%20articles/nother/toxic.htmhttp://../Journal%20articles/nother/toxic.htm -
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$eakness% paresis% paralysis indicates the lesions in the upper motor$eakness% paresis% paralysis indicates the lesions in the upper motornurone or lower motor neurone or myoneural unction or muscles.nurone or lower motor neurone or myoneural unction or muscles.
Preser'ation o# sensation% "ncreased or absent or diminished erks%Preser'ation o# sensation% "ncreased or absent or diminished erks%
atrophy or hypertrophy indicates the site o# lesions.atrophy or hypertrophy indicates the site o# lesions.
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)he major clinical dia"nosis associated
with A(P 0nG 941
=uillain&,arre s!ndrome 0JK.3L1/
Central ner6ous s!stem infection 04.3L1/
)rans6erse m!elitis 04K.L1
$on&polio entero6irus infection 0.3L1/
H!pokalaemic paral!sis 09.3L1.
( )ussain ")* Ali S* Sinniah M* +urup ,* +hoo !-* !homas !*
Apandi M* !aha AME Paediatr Child Health. 3KK Mar2K0J143&
JK
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Lyu* /.0+. et al. 1 Neurol Neurosur2 Psychiatry 34456789:4:0;
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Lyu* /.0+. et al. 1 Neurol Neurosur2 Psychiatry 34456789:4:0;
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/ecurrent uillain -arre> Syndrome
5f the 33K patients of acute idiopathic
dem!elinatin" pol!neuritis 0AIDP=,S1
seen o6er a se6en !ear period/ 49 patients
0M(441 had a relapsin" course 0.NL1.)he! had J episodes at a 6aria*le
inter6al of J months to 39 !rs.
!aly A-* upta S+* Anisya ?* Shankar S+* /ao S* ,as +-* Na2araa ,*Swamy )S.E Assoc Ph!sicians India. 4OO9 Apr2J013O&93.
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(ollow&up of A=,S
At a follow&up of 4 !ear or more/ 3K
patients reco6ered and J had residua. Hun" P%/ Chan" W$/ Huan" %)/ Huan" SC/ Chan" C/ Chan" CE/ Chan" CS/ Wan" QW/ Chen" ,C/ Chan" HW/ %u
CH. Pediatr $eurol. 3KK (e*2JK031N&O4.
permanent neurolo"ical defects in childrenunder 49 !ears of a"e was 4.4K million
annuall! Rantala H/ #hari M/ $iemela M. Arch Dis Child. 4OO4 Eun201K&N2 discussion KN&O.
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Subacute "n#lammatory ,emyelinatin2
Polyneuropathy.
pro"ressi6e motor andor sensor!d!sfunction consistent with neuropath! inmore than one lim* with time to nadir
*etween and N weeks/electroph!siolo"ic e6idence of
dem!elination in at least two ner6es/no other etiolo"! of neuropath!/ andno relapse on ade:uate follow&up.
Complete recovery was achieved in 69%of cases and partial recovery in others. @h S1* +urokawa +* de Almeida ,* /yan ) 1r* Claussen C.
$eurolo"!. 3KKJ Dec O24044149K&43.
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Chronic "n#lammatory ,emyelinatin2
Polyradiculoneuropathy
A low fre:uenc! of antecedent e6ents.
Weakness accompanied *! sensor! loss
and diminished tendon refle+es
Pain and cranial neuropathies are
uncommon
Pro"ressi6e weakness for two months. Sladk! E)2 Ashwal S Peripheral neuropathies in childre. Pediatric $eurolo"! Principles andPractice. 8d. Swaiman QS2 Ashwal S. Jrded.4OOO. Pu*lisher Mos*!
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=, S!ndrome 9NL
Chronic inflammator! dem!elinatin"
pol!neuropath! J4L
Associated with colla"en
6ascular disease L
5ther immuneinfectousdisorders L
Causes "n#lammatory
Polyradiculoneuropathy
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)@P+"NS> SBN,/@M9 Acute post0
asthmatic amyotrophy5nset
After acute asthmatic attack %atenc! 4 to 4N
da!s
Mild pain %im*/ neck or menin"ismus
Rapid onset weakness
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Weakness
Sin"le lim*2 As!mmetric2 Ma! *e Pro+imal ?Distal
Se6erit! Mild to se6ere
Arm or le"
Sensor! $ormal
)@P+"NS> SBN,/@M9 Acute post0
asthmatic amyotrophy
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CS(
Pleoc!tosis Protein F Increased
MRI Ma! show si"nal 0)31 in spinal cord
Pro"nosis Permanent paral!sis
)@P+"NS> SBN,/@M9 Acute post0
asthmatic amyotrophy
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laccid or hyper tonicitylaccid or hyper tonicity