Acute Disseminated Encephalomyelitis in Children: Discordant Neurologic and Neuroimaging Abnormalities and Response to Plasmapheresis

• Published in: Pediatrics (Evanston) 2005-08, Vol.116 (2), p.431-436
• Main Authors: Khurana, Divya S, Melvin, Joseph J, Kothare, Sanjeev V, Valencia, Ignacio, Hardison, H. Huntley, Yum, Sabrina, Faerber, Eric N, Legido, Agustin
• Format: Article
• Language: English
• Subjects: Adolescent; Biological and medical sciences; Brain - pathology; Cerebrospinal fluid. Meninges. Spinal cord; Child; Children & youth; Corticosteroid drugs; Corticosteroids; Diagnosis; Dosage and administration; Drug therapy; Encephalomyelitis; Encephalomyelitis, Acute Disseminated - diagnosis; Encephalomyelitis, Acute Disseminated - therapy; Female; General aspects; Glucocorticoids - therapeutic use; Humans; Immunoglobulins; Immunoglobulins, Intravenous - therapeutic use; Inflammatory diseases; Magnetic Resonance Imaging; Male; Medical sciences; Medical treatment; Methylprednisolone - therapeutic use; Nervous system (semeiology, syndromes); Neurology; Pediatrics; Plasmapheresis; Spinal Cord - pathology
• ISSN: 0031-4005; 1098-4275
• DOI: 10.1542/peds.2004-2038

To describe our experience with acute disseminated encephalomyelitis (ADEM), focusing on (1) the relationship between clinical course and MRI findings and (2) the response to plasmapheresis in a subgroup of patients. A retrospective record review was conducted of 13 children who were admitted as inpatients with the diagnosis of ADEM during the period 1998-2003. Diagnosis was established by clinical signs and symptoms, cerebrospinal fluid changes and multifocal involvement of deep gray and white matter based on MRI. Initial therapy was high-dose methylprednisolone and intravenous immunoglobulin in 12 patients. One child improved spontaneously. Six of 12 children did not improve with corticosteroid treatment. All 6 had an acute progressive course neurologically, and 5 of them also showed a delay in the onset of neuroimaging changes, eventually developing lesions in the deep gray matter and brainstem. This latter group received 5 sessions of plasmapheresis and recovered over the course of several months with varying degrees of residual neurologic deficits. Presentation of ADEM with delayed development of MRI lesions in deep gray matter and brainstem may herald a prolonged clinical course and lack of response to glucocorticoid therapy. Plasmapheresis might be an effective therapeutic intervention in these patients. The role of plasmapheresis versus corticosteroids and intravenous immunoglobulin as a primary treatment of ADEM needs to be investigated further.