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  1. 1. DEPATMENT <br />OF <br />INTERNAL MEDICINE<br />WEL-COME<br />PowerPoint Presentation By Dr.P.L.John Israel<br />
  2. 2. Acute Disseminated Encephalomyelitis<br />Acute Disseminated Encephalomyelitis (ADEM and its variants (modified from Francis et al)<br />
  3. 3. ADEM<br /> is an acute inflammatory demyelinating disease of the CNS<br />Is usually a monophasic disease . <br />Onset is acute<br />Neurological dysfunction is either multifocal or focal <br />Most commonly effects young adults and children<br />Prevalence 0.4 – 0.8 / 100,000 / year <br />Sex Distribution – possible male preponderance <br />
  4. 4. AHLE<br />Is a more virulent form of ADEM.<br />Has distinctive pathological features of tissue necrosis and hemorrhage.<br />Both ADEM & AHLE are due to an aberrant immune attack on the brain and / or spinal cord triggered by temporally related infections or vaccinations<br />
  5. 5. ADEM<br />Uniphasic, para/postinfections or postvaccination inflammatory demyelinating disorder of CNS<br />AHLE<br />Hyperacute from of ADEM, usually occuring after non-specific upper respiratory infections, more tissue destructive.<br />
  6. 6. Site restricted uniphasic ADEM (postinfectious.Postvaccination)<br />Transverse myelitis<br />Optic neuritis<br />Cerebellitis<br />Brain stem encephalitis<br />
  7. 7. Chronic or recurrent forms of parainfectious or postvaccination encephalomyelitis<br />Combined central and peripheral nervous system inflammatory demyelinating disorder<br />Post vaccination : Rabies, influenza<br />Post infectios : Measles<br />
  8. 8. Common Causes of ADEM<br />Postinfectious<br />Viral<br />Measles<br />Varicella<br />Rubella<br />Herpes Zoster<br />Infectious mononucleosis<br />Bacterial<br />Myoplasma<br />Gram- ve organisms<br />Salmonella typhi<br />Protozoal<br />Cerebral malaria<br />
  9. 9. Post Vaccination<br />Viral<br />Anti rabies vaccine<br />Influenza vaccine<br />Small pox( vaccina) vaccine<br />Japanese encephalitis vaccine<br />
  10. 10. PATHOLOGIC FEATURES<br />A the Pathology of ADEM following infections and vaccines is indistinguishable in each other<br />Grossly the brain and spinal cord are congested and swollen<br />They even be normal<br />Sectioned brain on examination may show prominent vassals in the white matter<br />The Pathological hallmark on histology is white spread fossae of perivenous demyelination through out the brain and spinal cord<br />
  11. 11. Clinical Features<br />Headache<br />Vomiting<br />Fever<br />Confusion<br />Meningism<br />Focal or multifocal brain and spinal cord signs may be present <br />Seizures or coma may occur<br />A minority of patients poor recover have further episodes<br />
  12. 12. Investigations<br />MRI<br />Shows multiple high signal areas in a pattern similar to that of MS, although often with larger areas of abnormality.<br />Lesions are confluent an ill defined <br />Usually bilateral gray matter lesions ( in thalumus basal ganglia)<br />Perifocal edema and mass effect may be seen <br />There should be absence of previous demyelinating activity <br />Follow-up MRI may reveal a status quo lesion or resolution of lesion <br />Any new lesion on follow up MRI is not compatible with ADEM<br />
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  15. 15. MRI - Transverse myelitis: This 10-year-old girl presented with neck pain and difficulty walking. Examination revealed a C4 sensory level, hyperreflexia and paraparesis. Sagittal T2-weighted MR imaging through the cervical spinal cord shows increased caliber of the cervical cord extending from C2 to C5 and high signal intensity within the cord parenchyma<br />
  16. 16. Acute disseminated encephalomyelitis: A low power view of thoracic spinal cord stained for myelin reveals multiple foci of perivascular demyelination, some confluent. <br />
  17. 17. CSF<br />May be normal or show an increase in protein and lymphocytes<br />Oligoclonal bands may be found in the acute episode for do not persist upon recovery unlike in MS<br />The differential diagnosis from a first severe attack of MS may be difficult <br />
  18. 18. Management<br />The disease may be fatal in the acute stages but is otherwise self limiting <br />In general treatment should be initiated as early as possible and as aggressive as neccesary<br />Supportive care is of paraamount importance <br />AHLE is uniformly fatal <br />Treatment with high dose intravenous methyl prednisolone with a cumulative dose of 3 – 5 gms over a period of 5days followed by a prolonged oral prednisolone tapered overed 3-6weeks<br />
  19. 19. If patient does not respond adequately to steroids, intravenous immunoglobulin 0.4gms/kg body weight over 5days is given <br />Alternatively plasma pheresis can be considered <br />In very severe cases immuno suppression with cyclophospamide or mitoxantrone should be attempted <br />
  20. 20. Prophylaxis<br />With measles vaccine and frequent use human diploid vaccine has drastically reduced the incidence of ADEM in India<br />
  21. 21. Prognosis<br />Recovery may occur over 1- 6months <br />60-80% cases fully recover <br />Rest of them may show residual neurological signs intellectual impairment and behavioral abnormalities<br />
  22. 22. 22<br />