A CASE OF RECURRENT DEMYELINATION

DEPARTMENT OF PEDIATRICS

GOVT ROYAPETTAH HOSPITAL

KILPAUK MEDICAL COLLEGE

Presentor-Dr.S.VIGNESH KUMAR

UNDER GUIDANCE OF

Prof.Dr.A.VIJAYARAGHAVAN MD,DCH.,

Prof of Pediatrics

Prof.Dr.B.SASI REKHA MD,DCH.,

Prof of Pediatrics

Prof.Dr.LEEMA PAULINE MD.,DM.,

Prof of Neurology

Prof.Dr.GOPINATH MDRD.,

Prof of Radiology

• 12 yr old female child Porselvi , born of

NCP, hailing from Tiruppur, brought by her

mother on 12th may 2011 with C/O

Vomiting

Headache

Seizure

HOPI:

Apparently normal child brought to our

hospital on 12th may with sudden onset of

Vomiting-one episode/projectile

Headache- bi-frontal

not associated with

photophobia

Lt focal seizure-10 min

Past h/o:

10 months back child was admitted in cmc

with complaints of

Vomiting/giddiness/headache/GTCS

Fever-day 2

3 episodes of GTCS during stay in hospital

CT brain-hypodense lesion Lt frontal

lobe

MRI-focal altered signals in Lt frontal

lobe

EEG-Abnormal record with spikes

Discharged with AED&regular follow-up

Antenatal h/o: Uneventful

Birth and postnatal h/o:FTND

2.5 kg

Developmental h/o:

Attained age appropriate milestones

Average school performance

Studying 7th std

Family h/o:

No h/o seizures/similar illness in family

G/E:

Child drowsy/oriented/Afebrile

Vitals:Stable

Anthropometry:

Ht-146 cms/149 cms

Wt-36 kgs/39.5 kgs

Head to toe examination:

No dysmorphic facies

No neuro-cutaneous marker

CNS:

Higher functions -Drowsy/oriented to

time&space

Cranial nerves -normal

Motor system -normal

Sensory system -normal

No Cerebellar signs

No signs of meningeal irritation

Spine and cranium-normal

Other system examination-normal

Lab parameters:

Tc-8600

Dc-P-57/L-38/E-5

Hb-8.8g/dl

Plt-1.6lakhs

Ps-normocytic hypochromic anemia

Esr-10/22

Mx-neg

urine R/E:normal

RFT-normal

LFT-normal

RBS-normal

Sr.electrolytes-normal

Sr.cholesterol-normal

CSF analysis:

Glucose-51

Protein-16

Cell count-no cells

Gram stain-no organism

AFB-negative

C/S-negative

Virology studies-negative for HSV

AUG 2010

Focal altered signal noted in LT FRONTAL LOBE involving Periventricular

Region,head of caudate nucleus,genu of Corpus Callosum and

sub-cortical white matter.

MAY 2011

New Whitematter lesion in the supratentorial region, bilateral medial

thalamus,splenium of the corpus callosum

Differential diagnosis

MULTIPLE SCLEROSIS

RELAPSING ADEM

Vasculitis of CNS

FURTHER WORK UP

CSF electrophoresis:

Oligoclonal band-absent

myelin basic protein-not available

Ophthalmology opinion:No evidence of

Optic

neuritis

Blood-immunology:

ANA-weak positive(1:100 dilution)

Pure tone audiogram: Normal

MANAGEMENT

IV dexamethasone 0.3mg/kg/day-5days

Oral prednisolone 1mg/kg/day-10days

Tab sodium valproate 1 tds

T.fst ½ bd

Discharged & advised regular follow-up

SEP 2011

Well defined long hyper intense lesions in right superior frontal gyrus,

right corona radiata,right superior temporal gyrus and right side of pons

AUG 2010 MAY 2011

SEP 2011

CASE DISCUSSION

ADEM MS PATIENT

Age <10yrs >10yrs 12yrs

Stupor/coma + - -

Fever/vomiting + - +

Family history No 20% No

Sensory

Complaints

+ - -

Optic Neuritis Bilateral Unilateral No

Manifestations Polysymptomatic Monosymptomatic polysymptomatic

MRI Imaging Widespread lesions:basal

ganglia,thalamus,cortical

grey-white junction

Isolated

lesions:periventricul

ar white

matter,corpus

callosum

New Whitematter

lesion in the

supratentorial

region, bilateral

mediai thalamus &

splenium of the

corpus callosum.

CSF Pleocytosis(lymphocytosi

s)

Oligoclonal bands Normal

Response to

steroids

+ + +

Follow-up No new lesions New lesions New lesions

Multiple sclerosis

Chronic,remitting-relapsing disorder

Multiple white matter lesions in CNS

Separated by time and location in brain

Etiology:

Unknown

Genetic

Immunologic

Infections

Pathology:

Demyelination with formation of plaques

Clinical manifestations:

• Sensory loss

• Optic neuritis

• Weakness

• Paresthesias

• Diplopia

• Ataxia

• Vertigo

• Epilepsy

Revised McDonald Diagnostic

Criteria for MS

CLINICAL

(ATTACKS)

LESIONS ADDITIONAL CRITERIA TO MAKE DX

2 or more Objective clinical evidence

of 2 or more lesions or

objective clinical evidence

of 1 lesion with reasonable

historical evidence of a prior

attack

None. Clinical evidence alone will suffice;

additional evidence desirable but must be

consistent with MS

2 or more Objective clinical evidence

of 1 lesion

Dissemination in space, demonstrated by

≥1T2 lesion in at least two MS typical

CNS regions

(periventricular, juxtacortical, infratentorial,

spinal cord); OR

Await further clinical attack implicating a

different CNS site

CLINICAL

(ATTACKS)

LESIONS ADDITIONAL CRITERIA TO MAKE DX

1 Objective

clinical

evidence

of 2 or more

lesions

Dissemination in time, demonstrated by

Simultaneous asymptomatic contrast-enhancing and

non-enhancing lesions at any time ; OR

A new T2 and/or contrast-enhancing lesions(s) on

follow-up MRI, irrespective of its timing; OR

Await a second clinical attack

1 Objective

clinical

evidence

of 1 lesion

Dissemination in space, demonstrated by

≥ 1T2 lesion in at least two MS typical CNS regions

(periventricular, juxtacortical, infratentorial, spinal cord);

OR

Await further clinical attack implicating a different

CNS site AND

Dissemination in time, demonstrated by

Simultaneous asymptomatic contrast-enhancing and

non-enhancing lesions at any time; OR

A new T2 and/or contrast-enhancing lesions(s) on

follow-up MIR, irrespective of its timing; OR

Await a second clinical attack

CLINICAL

(ATTACKS)

LESIONS ADDITIONAL CRITERIA TO MAKE DX

0 (progression

from onset) One year of disease progression

(retrospective or

prospective) AND at least 2 out of 3 criteria:

Dissemination in space in the brain

based on ≥1 T2 lesion in periventricular,

juxtacortical or infratentorial regions;

Dissemination in space in the spinal cord

based on ≥2 T2 lesions; OR

Positive CSF

Treatment:

*High dose intravenous methylprednisolone

*Rehabilitative care

*Disease modifying agent

#Interferon-b

#Glatiramer acetate

#Fingolimod

#Natalizumab

Prognosis:

*recovery complete

*progression slow

*long periods of remission

CARRY HOME MESSAGE

CSF-Oligoclonal bands/Myelin basic

protein

Revised McDonald Diagnostic Criteria

for MS

Regular follow up of cases suspected

to have multiple sclerosis with MRI is

supportive for diagnosis..