Acute Flaccid Paraplegia

7/24/2019 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

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

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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!

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



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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



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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



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Painful

Ac:uired

5thers



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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

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


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


asthmatic amyotrophy


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laccid or hyper tonicitylaccid or hyper tonicity

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Acute Flaccid Paraplegia