Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome

Daniela Pohl, Gulay Alper, Keith Van Haren, Andrew J. Kornberg, Claudia F Lucchinetti, Silvia Tenembaum, Anita L. Belman

Research output: Contribution to journalArticle

87 Citations (Scopus)

Abstract

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating CNS disorder with predilection to early childhood. ADEM is generally considered a monophasic disease. However, recurrent ADEM has been described and defined as multiphasic disseminated encephalomyelitis. ADEM often occurs postinfectiously, although a causal relationship has never been established. ADEM and multiple sclerosis are currently viewed as distinct entities, generally distinguishable even at disease onset. However, pathologic studies have demonstrated transitional cases of yet unclear significance. ADEM is clinically defined by acute polyfocal neurologic deficits including encephalopathy. MRI typically demonstrates reversible, ill-defined white matter lesions of the brain and often also the spinal cord, along with frequent involvement of thalami and basal ganglia. CSF analysis may reveal a mild pleocytosis and elevated protein, but is generally negative for intrathecal oligoclonal immunoglobulin G synthesis. In the absence of a specific diagnostic test, ADEM is considered a diagnosis of exclusion, and ADEM mimics, especially those requiring a different treatment approach, have to be carefully ruled out. The role of biomarkers, including autoantibodies like anti-myelin oligodendrocyte glycoprotein, in the pathogenesis and diagnosis of ADEM is currently under debate. Based on the presumed autoimmune etiology of ADEM, the current treatment approach consists of early immunotherapy. Outcome of ADEM in pediatric patients is generally favorable, but cognitive deficits have been reported even in the absence of other neurologic sequelae. This review summarizes the current knowledge on epidemiology, pathology, clinical presentation, neuroimaging features, CSF findings, differential diagnosis, therapy, and outcome, with a focus on recent advances and controversies.

Original languageEnglish (US)
Pages (from-to)S38-S45
JournalNeurology
Volume87
Issue number9
DOIs
StatePublished - Aug 30 2016

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Acute Disseminated Encephalomyelitis
Multiple Sclerosis
Myelin-Oligodendrocyte Glycoprotein
Oligoclonal Bands
Encephalomyelitis
Clinical Pathology
Leukocytosis
Brain Diseases
Demyelinating Diseases
Neurologic Manifestations
Basal Ganglia
Thalamus
Routine Diagnostic Tests
Neuroimaging
Autoantibodies
Immunotherapy
Nervous System
Spinal Cord
Epidemiology
Differential Diagnosis

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Pohl, D., Alper, G., Van Haren, K., Kornberg, A. J., Lucchinetti, C. F., Tenembaum, S., & Belman, A. L. (2016). Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome. Neurology, 87(9), S38-S45. https://doi.org/10.1212/WNL.0000000000002825

Acute disseminated encephalomyelitis : Updates on an inflammatory CNS syndrome. / Pohl, Daniela; Alper, Gulay; Van Haren, Keith; Kornberg, Andrew J.; Lucchinetti, Claudia F; Tenembaum, Silvia; Belman, Anita L.

In: Neurology, Vol. 87, No. 9, 30.08.2016, p. S38-S45.

Research output: Contribution to journalArticle

Pohl, D, Alper, G, Van Haren, K, Kornberg, AJ, Lucchinetti, CF, Tenembaum, S & Belman, AL 2016, 'Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome', Neurology, vol. 87, no. 9, pp. S38-S45. https://doi.org/10.1212/WNL.0000000000002825
Pohl, Daniela ; Alper, Gulay ; Van Haren, Keith ; Kornberg, Andrew J. ; Lucchinetti, Claudia F ; Tenembaum, Silvia ; Belman, Anita L. / Acute disseminated encephalomyelitis : Updates on an inflammatory CNS syndrome. In: Neurology. 2016 ; Vol. 87, No. 9. pp. S38-S45.
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