Acute disseminated encephalomyelitis in children: Pathogenesis, clinical features, and diagnosis
- Timothy E Lotze, MD
Timothy E Lotze, MD
- Associate Professor of Pediatrics and Neurology
- Baylor College of Medicine
- Donald J Chadwick, MD
Donald J Chadwick, MD
- Adjunct Instructor
- Minneapolis Clinic of Neurology
Acute disseminated encephalomyelitis (ADEM), also known as postinfectious encephalomyelitis, is a demyelinating disease of the central nervous system that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalopathy.
ADEM must be distinguished from other central inflammatory demyelinating conditions of childhood, including multiple sclerosis and clinically isolated syndromes that include optic neuritis, transverse myelitis, and neuromyelitis optica (Devic's disease). Most of these conditions are thought to be caused by immune system dysregulation triggered by an infectious or other environmental agent in a genetically susceptible host.
This topic will review the epidemiology, pathogenesis, clinical features, and diagnosis of ADEM in children. Treatment and prognosis of ADEM in children are discussed separately. (See "Acute disseminated encephalomyelitis in children: Treatment and prognosis".)
ADEM is an uncommon illness; the estimated incidence in California and Japan is 0.4/100,000 children per year [1,2], and in Canada is 0.2/100,000 per year . Approximately three to six cases are seen each year at regional medical centers in the United States, United Kingdom, and Australia [4-6]. There is no specific ethnic distribution . Several studies indicate a slight male predominance .
Postinfectious — ADEM is often preceded by a viral or bacterial infection, usually in the form of a nonspecific upper respiratory infection. In three series, an antecedent infection could be identified in 72 to 77 percent of patients [4,5,8]. A seasonal distribution also has been observed, with most cases presenting in the winter and spring [4,6]. In general, patients will present within one month of their illness.
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- Priming and activation phase
- Recruitment and effector phase
- CLINICAL FEATURES
- Clinical course
- Acute hemorrhagic leukoencephalitis
- Lumbar puncture
- Laboratory studies
- Diagnostic criteria
- - Monophasic ADEM
- - Multiphasic ADEM
- Differential diagnosis
- SUMMARY AND RECOMMENDATIONS