GuidelinesPEML (Paeds Hospital STG)
Acute Disseminated Encephalomyelitis (ADEM)
Acute Disseminated Encephalomyelitis (ADEM)
General and supportive measures
G04.0
DESCRIPTION
- Most common demyelinating disorder in childhood.
- Affects children with a peak incidence between 5 and 8 years.
- May occur following a systemic viral illness or vaccination.
- Demyelination of white matter in multiple areas of the brain and spinal cord.
DIAGNOSTIC CRITERIA
- ADEM is a diagnosis of exclusion.
- Consider and exclude differential diagnoses: SLE, infectious or autoimmune encephalitis, metabolic disorders, hypertensive encephalopathy.
Characterised by acute onset of encephalopathy with focal or multifocal neurological deficits.
- Seizures
- Cranial nerve palsies.
- Meningism
- Optic neuritis.
- Gait disturbances.
- Hemiparesis
- Pyramidal signs.
- Ataxia
- Aphasia
Symptoms usually peak between 2 and 5 days from onset, but may change or worsen for up to 3 months.
CSF
- Normal pressure.
- May have mild pleocytosis.
- Increased protein.
- Normal glucose.
Diagnostic criteria:
- A first, polyfocal clinical CNS event with a presumed inflammatory demyelinating cause.
- Encephalopathy (alteration in consciousness or behavior unexplained by fever, systemic illness or postictal syndrome).
- Brain MRI abnormalities consistent with demyelination during the acute (initial three-month) phase.
- No new clinical or MRI findings three months or more after clinical onset.
GENERAL AND SUPPORTIVE MEASURES
- Supportive care including ICU and mechanical ventilation may be necessary.
MEDICINE TREATMENT
Consider treatment with immune modulators and immunoglobulins in consultation with a neurologist.
REFERRAL
- As MRI is required for diagnosis, all patients should be referred prior to implementing treatment.
Click here to access NEMLC Report.
Standard Treatment Guidelines and Essential Medicines List for South Africa, Paediatric Hospital Level_Updated May 2025
Chapter 13: The Nervous System_13.8 Acute Disseminated Encephalomyelitis (ADEM)