Question

Acute disseminated encephalomyelitis (ADEM): Investigation and treatment.

Hint:

Early treatment with steroids is key in improving outcomes in ADEM, and most patients show good recovery, but early intervention is essential to prevent long-term disability.

Answer 1

Step 1: Investigations for ADEM.
Investigations for ADEM focus on confirming the diagnosis and excluding other conditions. Common investigations include:
(1) MRI of the Brain and Spine: To identify the characteristic demyelinating lesions, which are typically multiple, asymmetrical, and involve the brainstem, basal ganglia, and periventricular areas.
(2) CSF Analysis: Mild pleocytosis with an elevated protein level may be observed. The CSF usually lacks oligoclonal bands, which helps differentiate ADEM from multiple sclerosis.
(3) Blood Tests: To rule out infections, autoimmune conditions, or metabolic causes. Tests for viruses like Epstein-Barr virus (EBV), cytomegalovirus (CMV), and other infectious agents may be performed.
(4) Electroencephalogram (EEG): In cases with seizures, EEG can help assess for abnormal brain activity.
Step 2: Treatment of ADEM.
The treatment of ADEM focuses on controlling inflammation and managing symptoms. Key treatment options include:
(1) Corticosteroids: High-dose intravenous methylprednisolone is the first-line treatment. This helps to reduce inflammation and prevent further demyelination. Typically administered for 3-5 days, followed by oral prednisone tapering.
(2) Plasmapheresis: In severe or refractory cases, plasmapheresis may be considered to remove circulating antibodies and cytokines that contribute to the inflammatory process.
(3) Symptomatic Treatment: Antiepileptic drugs for seizure control, analgesics for headache, and physical therapy to address motor deficits and improve mobility.
(4) Supportive Care: Management in an intensive care unit (ICU) may be required for patients with severe symptoms or respiratory compromise.
Step 3: Prognosis and Follow-up.
Most children recover fully or with minimal residual neurological deficits within a few months. However, some may experience long-term neurological sequelae such as motor deficits or cognitive impairment. Regular follow-up is needed to monitor for recovery and address any ongoing symptoms.