Published online August 1, 2005
PEDIATRICS Vol. 116 No. 2 August 2005, pp. 431-436 (doi:10.1542/peds.2004-2038)
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Acute Disseminated Encephalomyelitis in Children: Discordant Neurologic and Neuroimaging Abnormalities and Response to Plasmapheresis

Divya S. Khurana, MD*, Joseph J. Melvin, DO*, Sanjeev V. Kothare, MD*, Ignacio Valencia, MD*, H. Huntley Hardison, MD*, Sabrina Yum, MD*, Eric N. Faerber, MD{ddagger} and Agustin Legido, MD, PhD*

* Section of Neurology, Departments of Pediatrics
{ddagger} Radiology, St Christopher's Hospital for Children, Drexel University College of Medicine, Philadelphia, Pennsylvania

Objectives. 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.

Methods. A retrospective record review was conducted of 13 children who were admitted as inpatients with the diagnosis of ADEM during the period 1998–2003.

Results. 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.

Conclusions. 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.

Key Words: MRI • demyelination • white matter • immunoglobulin • immunomodulation

Abbreviations: ADEM, acute disseminated encephalomyelitis • IVIG, intravenous immunoglobulin • CSF, cerebrospinal fluid • FLAIR, fluid attenuated inversion recovery • EDSS, Expanded Disability Status Scale • MS, multiple sclerosis • BDEM, biphasic disseminated encephalomyelitis

Accepted Nov 30, 2004.