DEPARTMENT OF PEDIATRICS GOVT ROYAPETTAH HOSPITAL KILPAUK ... · PDF fileGOVT ROYAPETTAH...
Transcript of DEPARTMENT OF PEDIATRICS GOVT ROYAPETTAH HOSPITAL KILPAUK ... · PDF fileGOVT ROYAPETTAH...
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A CASE OF RECURRENT DEMYELINATION
DEPARTMENT OF PEDIATRICS
GOVT ROYAPETTAH HOSPITAL
KILPAUK MEDICAL COLLEGE
Presentor-Dr.S.VIGNESH KUMAR
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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
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• 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
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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
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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
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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
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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
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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
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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
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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
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CSF analysis:
Glucose-51
Protein-16
Cell count-no cells
Gram stain-no organism
AFB-negative
C/S-negative
Virology studies-negative for HSV
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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.
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MAY 2011
New Whitematter lesion in the supratentorial region, bilateral medial
thalamus,splenium of the corpus callosum
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Differential diagnosis
MULTIPLE SCLEROSIS
RELAPSING ADEM
Vasculitis of CNS
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FURTHER WORK UP
CSF electrophoresis:
Oligoclonal band-absent
myelin basic protein-not available
Ophthalmology opinion:No evidence of
Optic
neuritis
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Blood-immunology:
ANA-weak positive(1:100 dilution)
Pure tone audiogram: Normal
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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
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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
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AUG 2010 MAY 2011
SEP 2011
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CASE DISCUSSION
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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
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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
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Clinical manifestations:
• Sensory loss
• Optic neuritis
• Weakness
• Paresthesias
• Diplopia
• Ataxia
• Vertigo
• Epilepsy
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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
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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
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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
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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
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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..