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Adem

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  • 1. DEPATMENT
    OF
    INTERNAL MEDICINE
    WEL-COME
    PowerPoint Presentation By Dr.P.L.John Israel
  • 2. Acute Disseminated Encephalomyelitis
    Acute Disseminated Encephalomyelitis (ADEM and its variants (modified from Francis et al)
  • 3. ADEM
    is an acute inflammatory demyelinating disease of the CNS
    Is usually a monophasic disease .
    Onset is acute
    Neurological dysfunction is either multifocal or focal
    Most commonly effects young adults and children
    Prevalence 0.4 – 0.8 / 100,000 / year
    Sex Distribution – possible male preponderance
  • 4. AHLE
    Is a more virulent form of ADEM.
    Has distinctive pathological features of tissue necrosis and hemorrhage.
    Both ADEM & AHLE are due to an aberrant immune attack on the brain and / or spinal cord triggered by temporally related infections or vaccinations
  • 5. ADEM
    Uniphasic, para/postinfections or postvaccination inflammatory demyelinating disorder of CNS
    AHLE
    Hyperacute from of ADEM, usually occuring after non-specific upper respiratory infections, more tissue destructive.
  • 6. Site restricted uniphasic ADEM (postinfectious.Postvaccination)
    Transverse myelitis
    Optic neuritis
    Cerebellitis
    Brain stem encephalitis
  • 7. Chronic or recurrent forms of parainfectious or postvaccination encephalomyelitis
    Combined central and peripheral nervous system inflammatory demyelinating disorder
    Post vaccination : Rabies, influenza
    Post infectios : Measles
  • 8. Common Causes of ADEM
    Postinfectious
    Viral
    Measles
    Varicella
    Rubella
    Herpes Zoster
    Infectious mononucleosis
    Bacterial
    Myoplasma
    Gram- ve organisms
    Salmonella typhi
    Protozoal
    Cerebral malaria
  • 9. Post Vaccination
    Viral
    Anti rabies vaccine
    Influenza vaccine
    Small pox( vaccina) vaccine
    Japanese encephalitis vaccine
  • 10. PATHOLOGIC FEATURES
    A the Pathology of ADEM following infections and vaccines is indistinguishable in each other
    Grossly the brain and spinal cord are congested and swollen
    They even be normal
    Sectioned brain on examination may show prominent vassals in the white matter
    The Pathological hallmark on histology is white spread fossae of perivenous demyelination through out the brain and spinal cord
  • 11. Clinical Features
    Headache
    Vomiting
    Fever
    Confusion
    Meningism
    Focal or multifocal brain and spinal cord signs may be present
    Seizures or coma may occur
    A minority of patients poor recover have further episodes
  • 12. Investigations
    MRI
    Shows multiple high signal areas in a pattern similar to that of MS, although often with larger areas of abnormality.
    Lesions are confluent an ill defined
    Usually bilateral gray matter lesions ( in thalumus basal ganglia)
    Perifocal edema and mass effect may be seen
    There should be absence of previous demyelinating activity
    Follow-up MRI may reveal a status quo lesion or resolution of lesion
    Any new lesion on follow up MRI is not compatible with ADEM
  • 13.
  • 14.
  • 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
  • 16. Acute disseminated encephalomyelitis: A low power view of thoracic spinal cord stained for myelin reveals multiple foci of perivascular demyelination, some confluent.
  • 17. CSF
    May be normal or show an increase in protein and lymphocytes
    Oligoclonal bands may be found in the acute episode for do not persist upon recovery unlike in MS
    The differential diagnosis from a first severe attack of MS may be difficult
  • 18. Management
    The disease may be fatal in the acute stages but is otherwise self limiting
    In general treatment should be initiated as early as possible and as aggressive as neccesary
    Supportive care is of paraamount importance
    AHLE is uniformly fatal
    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
  • 19. If patient does not respond adequately to steroids, intravenous immunoglobulin 0.4gms/kg body weight over 5days is given
    Alternatively plasma pheresis can be considered
    In very severe cases immuno suppression with cyclophospamide or mitoxantrone should be attempted
  • 20. Prophylaxis
    With measles vaccine and frequent use human diploid vaccine has drastically reduced the incidence of ADEM in India
  • 21. Prognosis
    Recovery may occur over 1- 6months
    60-80% cases fully recover
    Rest of them may show residual neurological signs intellectual impairment and behavioral abnormalities
  • 22. 22