Treatment of acute disseminated encephalomyelitis

Abstract

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease, characterized by an acute onset of polyfocal central nervous system (CNS) deficits, including encephalopathy, demonstrating multifocal lesions on MRI. ADEM is typically a monophasic disorder, but recurrent and multiphasic courses have been described. Furthermore, an ADEM presentation has been reported in neuromyelitis optica (NMO) and multiple sclerosis (MS), particularly in younger children. CNS infections, other autoimmune diseases, and neurometabolic disorders may mimic ADEM at manifestation. There is no single test confirming the diagnosis of ADEM, and diagnosis is based upon a combination of clinical and radiologic features and exclusion of diseases that resemble ADEM. Therefore, a broad workup including infectious, immunologic, and metabolic tests, as well as a systematic follow-up including MRI, is indicated to establish an accurate diagnosis as a prerequisite for an optimized treatment approach. There is a lack of evidence-based, prospective clinical trial data for the management of ADEM. Empiric antibacterial and antiviral treatment is standard of care until an infectious disease process is ruled out. Based on the presumed autoimmune etiology of ADEM, the common treatment approach consists of intravenous methylprednisolone at a dosage of 20 to 30 mg/kg per day (maximum 1 g/day) for 3 to 5 days, followed by an oral corticosteroid taper of 4 to 6 weeks. In case of insufficient response or contraindications to corticosteroids, intravenous immunoglobulin G (IVIG) at a dosage of 2 g/kg divided over 2 to 5 days is a therapeutic option. For severe or life-threatening cases of ADEM, plasmapheresis should be considered early in the disease course. Decompressive craniectomy has been reported as a life-saving measure for ADEM patients with intracranial hypertension. There is a lack of specific recommendations for the long-term management of recurrent and multiphasic ADEM. In children with relapsing demyelinating events, the diagnosis of a chronic autoimmune CNS disease like MS or NMO should be considered.