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    Adem Adem Presentation Transcript

    • DEPATMENT
      OF
      INTERNAL MEDICINE
      WEL-COME
      PowerPoint Presentation By Dr.P.L.John Israel
    • Acute Disseminated Encephalomyelitis
      Acute Disseminated Encephalomyelitis (ADEM and its variants (modified from Francis et al)
    • 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
    • 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
    • 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.
    • Site restricted uniphasic ADEM (postinfectious.Postvaccination)
      Transverse myelitis
      Optic neuritis
      Cerebellitis
      Brain stem encephalitis
    • 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
    • Common Causes of ADEM
      Postinfectious
      Viral
      Measles
      Varicella
      Rubella
      Herpes Zoster
      Infectious mononucleosis
      Bacterial
      Myoplasma
      Gram- ve organisms
      Salmonella typhi
      Protozoal
      Cerebral malaria
    • Post Vaccination
      Viral
      Anti rabies vaccine
      Influenza vaccine
      Small pox( vaccina) vaccine
      Japanese encephalitis vaccine
    • 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
    • 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
    • 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
    • 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
    • Acute disseminated encephalomyelitis: A low power view of thoracic spinal cord stained for myelin reveals multiple foci of perivascular demyelination, some confluent.
    • 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
    • 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
    • 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
    • Prophylaxis
      With measles vaccine and frequent use human diploid vaccine has drastically reduced the incidence of ADEM in India
    • 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