SEVERE ACUTE ENCEPHALOPATHY WITH EXTRAPYRAMIDAL INVOLVEMENT OF UNKNOWN ETIOLOGY

Objectives: Objectives: We describe the clinical features and evolution of six patients with acute encephalopathy (AE) with extrapyramidal involvement and of unknown etiology. Methods: We evaluated six patients with the diagnosis of AE with extrapyramidal involvement who were admitted to our center over the last 14 years. Results: The onset of the disease was at a mean age of five years (r: 1–11 years) in six previously healthy patients, four of whom started with severe and progressive behavioral disorders and two with partial seizures. Afterwards, extrapyramidal signs, aphasia and alteration of eye movements appeared in all six cases. Five patients presented: sleep and swallowing difficulties, psychomotor excitation, pyramidal involvement and impaired consciousness. Hospital stay was between 28 and 120 days. The clinical picture improved after 5 months of evolution in three patients, who currently present normal neurological examination and psychomotor development. One patient died. Blood and CSF (metabolic, infectious, toxic, immunological) lab studies were normal. Imaging studies were normal at onset and only one patient presented sequelae in the putamen at 4 months of evolution. The only patient who received gammaglobulin IV stayed less time in hospital than the others. Conclusions: We consider that the patients evaluated met the clinical diagnostic criteria of lethargic encephalitis, first described by Von Economo. In spite of the severe initial compromise and unresponsiveness to treatment, the majority of patients show a good evolution. Treatment with gammaglobulin may be a therapeutic option.