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acute necrotizing hemorrhagic encephalomyelitis

 

Definition: a fulminating demyelinating disorder of the central nervous system that affects mainly children and young adults. Almost always preceded by a respiratory infection, characterized by the abrupt onset of fever, headache, confusion, and nuchal rigidity, soon followed by focal seizures, hemiplegia, or quadriplegia, brainstem findings, and coma; the CSF shows evidence of an inflammatory process; due to the massive destruction of the white matter of one or both hemispheres, often accompanied by similar destruction of the white matter of the brainstem and cerebellar peduncles; of unknown etiology.

Synonym(s): acute hemorrhagic leukoencephalitis, acute necrotizing hemorrhagic leukoencephalitis, Hurst disease

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.

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