Epidemiology, immunopathogenesis and management of pediatric central nervous system inflammatory demyelinating conditions

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Purpose of review

Pediatric inflammatory demyelinating central nervous system diseases comprise monofocal and potentially monophasic disorders like optic neuritis and transverse myelitis, the multifocal, self-limiting disorder of acute disseminated encephalomyelitis, and multifocal chronic diseases like relapsing neuromyelitis optica and multiple sclerosis. This review discusses characteristics of these disorders with focus on epidemiology and treatment of acute disseminated encephalomyelitis, neuromyelitis optica and multiple sclerosis.

Recent findings

An international consensus group very recently defined diagnostic criteria for pediatric multiple sclerosis and acute disseminated encephalomyelitis. Immunological studies on pediatric inflammatory demyelinating disorders revealed possible disease-related humoral and T-cellular pathomechanisms. The recently identified biomarker for neuromyelitis optica, aquaporin-4-autoantibody, was detected in children with relapsing neuromyelitis optica with a similar frequency as in adult neuromyelitis optica patients. Clinically relevant, there is growing evidence that disease-modifying treatments are well tolerated and effective in the pediatric age group also.


Recent studies on pediatric inflammatory demyelinating central nervous system diseases have contributed to current awareness that these disorders are not restricted to the adult age group, and that some of them carry an unfavorable long-term prognosis. Growing knowledge will hopefully enable more timely diagnoses and more specifically tailored therapies in the near future, with the goal of improving outcomes in this young patient group.

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