What does this specimen Show

1
What does this specimen Show?

• Optic Nerve Stained for Myelin.

2
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

3
(No Transcript)
4
ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM)
- A Clinical and Neuroradiological Profile of
Pediatric Patients.

• Presented By
• Dr Jaimin M Patel
• M.D.(Paed)

• Under guidance of
• Dr.Varsha Tripathi
• M.D.(Paed),MRCP(London)
• Paediatric Neurologist

• With the help of
• Dr Manisha Shah.
• D. C. H.

5
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.

6
(No Transcript)
7
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

8
ADEM- Acute Disseminated Encephalomyelitis

• Acute-Disseminated-Encephalo-Myelo-Itis (ADEM)
• Usually monophasic
• Demyelinating disorder
• Characterized by diffuse neurologic signs and
  symptoms (polysymptomatic)
• Nuroimaging- multifocal lesions of demyelination

9
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

10
Clinical Features Complains
11
Neurological Examination.
12
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)
• (NOT INDICATED)
• May be normal or may reveal areas of patchy low
  attenuation in the white matter.

13
MRI Scan
R
14
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

15
MRI
16
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 (lt1 yr, No improvement in 48-72 hrs , AHLE,
  Recurrent)
• 1 gm/kg/day iv for 2 days
• Plasmapheresis
• Symptomatic Rx

17
Treatment.
18
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 (215 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

19
PROGNOSIS

• Complete recovery in 10 (77) of the survivors
• Relapsing disease in 2 (15)
• Mortality in 1 (7.6) case. (Post Measles)

20
Thank You