What does this specimen Show? : What does this specimen Show? Optic Nerve Stained for Myelin. 7yr. male VP from Jamnagar : 7yr. male VP from Jamnagar Left sided seizures – 2 episodes
Referred for EEG from Jamnagar.
Pt started on CBZ, not ready for MRI
After 2 wks referred from opthalmologist for loss of vision, and optic neuritis
MRI done- Diagnosis ADEM ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM) - A Clinical and Neuroradiological Profile of Pediatric Patients. : ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM) - A Clinical and Neuroradiological Profile of Pediatric Patients. Presented By
Dr Jaimin M Patel
M.D.(Paed) Under guidance of
Paediatric Neurologist With the help of
Dr Manisha Shah.
D. C. H. Case History: ADEM : Case History: ADEM 4 year old male child
URTI for 3-4 days
Admitted with convulsions altered sensorium aphasia left hemiparesis
CSF prt: 72 mg/dL (40-60) cells: 8 cells/mm3
MRI scan:(T2 weighted)asymmetrical hyper intensities in midbrain (more on right side) and middle cerebral peduncle suggestive of ADEM. Case History: ADEM : Case History: ADEM Symptomatic treatment
IV methylprednisolone givenafter 48 hours no improvement- some deteriorationRt.third nerve palsybulbar weakness
IVIG given 1gm/kg/day for 2 daysRyles tube feeding started5 th day onwards improvement started child was hospitalized for 17 days
At present child completely normal, going to school ADEM- Acute Disseminated Encephalomyelitis : ADEM- Acute Disseminated Encephalomyelitis Acute-Disseminated-Encephalo-Myelo-Itis (ADEM)
Characterized by diffuse neurologic signs and symptoms (polysymptomatic)
Nuroimaging- multifocal lesions of demyelination Study… : Study… Retrospective Study
Period : 14 months from Aug 2003 to Nov 2004
Study Place: Medisurge and Sterling H.
Total No of Pts –13
Mean Age – 4. 02 yrs.
M : F = 9 : 4 Clinical Features & Complains : Clinical Features & Complains Neurological Examination. : Neurological Examination. ADEM : Investigations : ADEM : Investigations CSF (9 available) – 6 (66%) –normal, 3- (33%) Increase Pr. 1 (11%) -Pleocytosis
Mild mononuclear pleocytosis, raised proteins MBP content increased in 60% intrathecal oligoclonal immunoglobulins and IgG seen occasionally
CT Scan: (1 Pt-B/L hypodensities)
May be normal or may reveal areas of patchy low attenuation in the white matter. MRI Scan : MRI Scan R ADEM : Investigations : ADEM : Investigations MRI scan brain:
Multiple foci of demyelination in the cerebrum, midbrain, brain stem and cerebellum
Lesions are rather extensive and symmetric or asymmetric and more often located in the peripheral subcortical cerebral white matter.
Lesions in the thalami, hypothalamus and basal ganglia
Occasionally there can be cortical grey mater involvement also MRI : MRI ADEM: Treatment : ADEM: Treatment High Dose Steroids
High dose IV Methyl Prednisolone 30 mg/kg/day for 3-5 days
followed by oral steroids (Prednisoslone) for 6 weeks
IVIG (<1 yr, No improvement in 48-72 hrs , AHLE, Recurrent)
1 gm/kg/day iv for 2 days
Symptomatic Rx Treatment. : Treatment. Relapse : Relapse Usually a monophasic illness
Multiphasic disseminated encephalomyelitis MDEM (Two in my study)
Recurrences : defined as appearance of new s/s at least 1 mth. after the previous episode.
10-20% in different studies (2=15% in my study)
Recurrence appeared 1.5 - 32 months after initial presentation, but usually after steroids were stopped (2 months in my study)
6 week taper shows less recurrences PROGNOSIS : PROGNOSIS Complete recovery in 10 (77%) of the survivors
Relapsing disease in 2 (15%)
Mortality in 1 (7.6%) case. (Post Measles) Thank You : Thank You