Acute necrotizing encephalopathy: combined therapy and favorable outcome in a new case

Acute necrotizing encephalopathy (ANE) is a rare disease characterized by multiple, symmetrical brain lesions, affecting thalami, brainstem tegmentum, and cerebellar medulla; more inconstantly, other structures are involved, i.e., internal capsules, posterolateral putamen, and deep periventricular white matter.

The clinical picture consists of rapidly deteriorating acute monophasic encephalopathy preceded by prodromal febrile illness; the symptoms include hyperpyrexia, convulsions, recurrent vomiting, and coma within 24 h.

The outcome is usually poor and approximately 70% of the patients die within a few days from the onset of fever. There is no specific therapy for ANE but, in some patients, the clinical status improved with steroid treatment.