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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
26:48

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis

Published on: July 4, 2007

Acute disseminated encephalomyelitis

Silvia N Tenembaum1

  • 1Department of Neurology and Neurophysiology, National Paediatric Hospital Dr. Juan P. Garrahan, Buenos Aires, Argentina.

Handbook of Clinical Neurology
|April 30, 2013
PubMed

Insights

Acute disseminated encephalomyelitis (ADEM) is a childhood CNS disorder. Diagnosis relies on clinical and MRI findings, with favorable prognosis after steroid treatment, though differentiating from MS and NMO can be challenging.

Area of Science:

  • Pediatric Neurology
  • Neuroimmunology
  • Neuroimaging

Background:

  • Childhood white matter disorders have gained attention with advances in magnetic resonance imaging (MRI).
  • Pediatric inflammatory demyelinating diseases of the central nervous system (CNS) present with diverse clinical features, severity, progression, and prognoses.
  • Acute disseminated encephalomyelitis (ADEM) is a common, acute, immune-mediated CNS inflammatory disorder in children, characterized by encephalopathy and neurological deficits.

Purpose of the Study:

  • To summarize the current literature on childhood ADEM.
  • To discuss diagnostic challenges, particularly differentiating ADEM from multiple sclerosis (MS) and neuromyelitis optica (NMO) in relapsing cases.
  • To present proposed consensus definitions for monophasic and relapsing variants of ADEM.

Main Methods:

  • Diagnosis of ADEM is primarily based on clinical presentation and MRI evidence of demyelination, excluding other causes of acute encephalopathy.
  • Retrospective patient studies have focused on clinical and neuroimaging characteristics to establish diagnostic criteria.
  • Literature review of existing studies on ADEM in children.

Main Results:

  • ADEM is typically self-limiting, with the highest incidence in childhood.
  • Diagnostic difficulties arise in distinguishing relapsing ADEM from MS and NMO due to overlapping features.
  • High-dose steroid treatment generally leads to a favorable long-term prognosis for functional and cognitive recovery in ADEM patients.

Conclusions:

  • ADEM is a significant pediatric CNS inflammatory demyelinating disease.
  • Accurate diagnosis requires careful clinical evaluation and neuroimaging, especially when considering relapsing forms.
  • Established consensus definitions for monophasic and relapsing ADEM aid in classification and management.

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