DEPARTMENT OF PEDIATRICS GOVT ROYAPETTAH HOSPITAL KILPAUK MEDICAL
Transcript of DEPARTMENT OF PEDIATRICS GOVT ROYAPETTAH HOSPITAL KILPAUK MEDICAL
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®ular 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..