1. Acute disseminated encephalomyelitis (ADEM) is an acute inflammatory demyelinating disease of the central nervous system that most commonly affects children and young adults. 2. It is usually a monophasic disease with an abrupt onset and can cause multifocal or focal neurological dysfunction. 3. Treatment involves high-dose intravenous corticosteroids, intravenous immunoglobulin, or plasma exchange depending on severity. Most patients fully recover, though some experience residual effects.

1. DEPATMENT OF INTERNAL MEDICINEWEL-COMEPowerPoint Presentation By Dr.P.L.John Israel

2. Acute Disseminated EncephalomyelitisAcute Disseminated Encephalomyelitis (ADEM and its variants (modified from Francis et al)

3. ADEM is an acute inflammatory demyelinating disease of the CNSIs usually a monophasic disease . Onset is acuteNeurological dysfunction is either multifocal or focal Most commonly effects young adults and childrenPrevalence 0.4 – 0.8 / 100,000 / year Sex Distribution – possible male preponderance

4. AHLEIs 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. ADEMUniphasic, para/postinfections or postvaccination inflammatory demyelinating disorder of CNSAHLEHyperacute from of ADEM, usually occuring after non-specific upper respiratory infections, more tissue destructive.

6. Site restricted uniphasic ADEM (postinfectious.Postvaccination)Transverse myelitisOptic neuritisCerebellitisBrain stem encephalitis

7. Chronic or recurrent forms of parainfectious or postvaccination encephalomyelitisCombined central and peripheral nervous system inflammatory demyelinating disorderPost vaccination : Rabies, influenzaPost infectios : Measles

8. Common Causes of ADEMPostinfectiousViralMeaslesVaricellaRubellaHerpes ZosterInfectious mononucleosisBacterialMyoplasmaGram- ve organismsSalmonella typhiProtozoalCerebral malaria

9. Post VaccinationViralAnti rabies vaccineInfluenza vaccineSmall pox( vaccina) vaccineJapanese encephalitis vaccine

10. PATHOLOGIC FEATURESA the Pathology of ADEM following infections and vaccines is indistinguishable in each otherGrossly the brain and spinal cord are congested and swollenThey even be normalSectioned brain on examination may show prominent vassals in the white matterThe Pathological hallmark on histology is white spread fossae of perivenous demyelination through out the brain and spinal cord

11. Clinical FeaturesHeadacheVomitingFeverConfusionMeningismFocal or multifocal brain and spinal cord signs may be present Seizures or coma may occurA minority of patients poor recover have further episodes

12. InvestigationsMRIShows 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

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. CSFMay be normal or show an increase in protein and lymphocytesOligoclonal bands may be found in the acute episode for do not persist upon recovery unlike in MSThe differential diagnosis from a first severe attack of MS may be difficult

18. ManagementThe 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 neccesarySupportive 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. ProphylaxisWith measles vaccine and frequent use human diploid vaccine has drastically reduced the incidence of ADEM in India

21. PrognosisRecovery may occur over 1- 6months 60-80% cases fully recover Rest of them may show residual neurological signs intellectual impairment and behavioral abnormalities

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