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Acute necrotizing encephalopathy of childhood

ORPHA:263524· ICD-10 G31.8

Definition

A rare neurologic disease characterized by a rapid onset of seizures, an altered state of consciousness, neurologic decline, and variable degrees of hepatic dysfunction following a respiratory or gastrointesitnal infection (e.g. mycoplasma, influenza virus) in a previously healthy child. Brain MRI of patients reveals bilateral, multiple, symmetrical lesions predominantly observed in thalami and brainstem, but also in periventricular white matter and cerebellum in some cases.

Prevalence
Unknown
Inheritance
Not applicable
Age of onset
Childhood