Introduction
Acute Disseminated Encephalomyelitis (ADEM) is a rare but serious autoimmune demyelinating disorder of the central nervous system (CNS), primarily affecting children. It is usually triggered by infections or vaccinations, leading to widespread inflammation and demyelination in the brain and spinal cord. Prompt recognition and treatment are essential to prevent long-term neurological sequelae.
First-Line Treatment
1. High-Dose Corticosteroids
Methylprednisolone (IVMP) 30 mg/kg/day (max 1 g/day) for 3–5 days is the first-line treatment. This helps reduce inflammation and immune-mediated damage.
✅ Benefits:
- Rapid symptom improvement
- Reduces CNS inflammation
- Prevents disease progression
➡️ Followed by Oral Prednisolone (1-2 mg/kg/day) for 4–6 weeks, with a gradual taper over 4–6 weeks to prevent relapse.
Second-Line Treatment (Refractory Cases)
If no improvement is seen after 3–5 days of IV steroids, second-line therapies should be considered:
2. Intravenous Immunoglobulin (IVIG)
Dose: 2 g/kg over 2–5 days
✅ Used in steroid-resistant ADEM
✅ Modulates immune response
✅ Reduces inflammation
3. Plasma Exchange (PLEX)
- Indicated for severe or steroid-refractory cases
- Removes pathogenic autoantibodies
- Usually given as 5–7 exchanges over 10–14 days
4. Rituximab or Cyclophosphamide (Rarely Used)
- Considered in severe, relapsing, or refractory ADEM
- Rituximab (anti-CD20) depletes B-cells
- Cyclophosphamide suppresses immune response
Supportive Care
✅ Symptom Management:
- Antiepileptics (if seizures occur)
- ICU care if respiratory distress develops
- Physiotherapy for motor recovery
✅ Neurorehabilitation:
- Speech, occupational, and physical therapy for long-term recovery
✅ Long-Term Monitoring:
- Follow-up MRIs to assess demyelination resolution
- Neuropsychological assessment for cognitive deficits
Prognosis
- Most children recover fully within weeks to months
- 10-20% may have residual neurological deficits
- Differentiation from Multiple Sclerosis (MS) is crucial (ADEM is usually monophasic; MS is relapsing)
