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Differential diagnosis of acute central nervous system demyelination in children

Author
Timothy E Lotze, MD
Section Editors
Marc C Patterson, MD, FRACP
Francisco González-Scarano, MD
Deputy Editor
John F Dashe, MD, PhD

INTRODUCTION

Differential diagnostic considerations for acute central nervous system demyelination in children include acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), optic neuritis, transverse myelitis, neuromyelitis optica (Devic disease), and various infectious, metabolic, and rheumatologic conditions (table 1). Most of these conditions are thought to be caused by immune system dysregulation triggered by an infectious agent in a genetically susceptible host.

With the possible exception of the NMO-IgG autoantibody found in neuromyelitis optica, there are no disease-specific biomarkers for these conditions, making it difficult to distinguish among them at the time of the initial presentation. However, certain clinical features, laboratory results, and imaging findings can usually lead to the correct diagnosis. Multiple sclerosis is to a large extent still a diagnosis of exclusion, and therefore requires intense investigation for other conditions that might present in a similar manner.

ACUTE DISSEMINATED ENCEPHALOMYELITIS

Acute disseminated encephalomyelitis (ADEM) is characterized by acute or subacute onset of multifocal neurologic deficits with encephalopathy, often following a viral illness or vaccination. Additional typical signs associated with ADEM are headache, fever, or meningismus. Magnetic resonance imaging (MRI) demonstrates widespread asymmetric white and gray matter abnormalities (table 2). (See "Acute disseminated encephalomyelitis in children: Pathogenesis, clinical features, and diagnosis" and "Acute disseminated encephalomyelitis in children: Treatment and prognosis".)

In its classic form, ADEM is a monophasic disease, although the clinical features can fluctuate in severity and evolve in the first three months following disease onset [1]. However, relapsing ADEM (ie, multiphasic ADEM) has been reported in a minority. Multiphasic ADEM is defined as two episodes consistent with ADEM separated by three months, irrespective of glucocorticoid use, but not followed by any further events. The second ADEM event can involve either new or a re-emergence of prior neurologic symptoms, signs and MRI findings. Relapses beyond a second event are not consistent with ADEM according to current diagnostic criteria, but rather indicate a chronic disorder such as multiple sclerosis or neuromyelitis optica [1].

Acute hemorrhagic leukoencephalitis — Acute hemorrhagic leukoencephalitis is a rare entity that represents a more severe and fulminant form of ADEM and is associated with diffuse central nervous system hemorrhage. (See "Acute disseminated encephalomyelitis in children: Pathogenesis, clinical features, and diagnosis", section on 'Acute hemorrhagic leukoencephalitis'.)

             

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Literature review current through: Nov 2016. | This topic last updated: Fri Oct 31 00:00:00 GMT 2014.
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