Abstract

Acute disseminated encephalomyelitis (ADEM) is a common acute multifocal autoimmune infl ammatory demyelinating disease of the central nervous system (CNS) with a predominant lesion of the white matter of the brain, brainstem and optic nerves, the conductors of the spinal cord, less often — gray matter of the CNS. ADEM is usually a self-limiting condition with spontaneous improvement and a favorable prognosis. Along with the possibility of a severe course in the classical form of ADEM, the development of hyperacute forms with a much more unfavorable prognosis is possible. Such variants of the course of ADEM are acute hemorrhagic leukoencephalitis (AHLE), acute hemorrhagic leukoencephalomyelitis and acute necrotizing hemorrhagic leukoencephalitis of Hurst, associated with the development of a cascade of hyperacute uncontrolled autoimmune reactions. The severity of these conditions (grouped under the term OHLE) is associated with rapid progression, brainstem involvement, large lesions is due to the development of acute edematous necrosis and hemorrhage with a high possibility of disability or death. The aggressive and severe course of АHLE requires the immediate initiation of decongestant and immunosuppressive therapy.

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