[Acute non-herpetic encephalitis with severe refractory status epilepticus --its overwhelming ictogenicity, epileptogenicity, long-term prognosis and review of the literature].

In 1980s, the authors experienced 5 patients with "a peculiar form of acute encephalitis/encephalopathy "which is characterized by three features: 1. Complex partial seizures with secondary generalization recur incessantly or continue persistently without regaining consciousness for many days in spite of intensive diazepam (DZP) therapy; usually general anesthesia at ICU setting for 2-3 weeks becomes mandatory. 2. After weaning from long-run anesthesia, seizures of the same type still persist, though much less frequent, during the convalescent and chronic phases of the disease throughout. There is no seizure-free interval between the acute and chronic stages. 3. Etiology is totally unknown;extensive laboratory examinations mostly remain within normal ranges, though clinical features such as acute onset, frequent accompaniment of fever, etc., mimic those of acute encephalitis/encephalopathy. The synopsis of our 5 patients were; age ranged from 2 to 5 years old, no antecedent history of seizures, fever of moderate degree preceded seizures a few days and persisted in the acute stage; complex partial seizures with secondary generalization recurred several times every hour. Circulatory/respiratory compromise necessitated a drastic intervention with barbiturate coma at ICU. Etiology was unknown. Follow-up for 19 years in average revealed mental retardation and chronic epilepsies in all patients. Since our first report in 1987, 49 similar cases with ours have been sporadically reported. The majority of the reported cases had been submitted to longstanding general anesthesia and placed in barbiturate coma for weeks to 2 months. The death occurred in 7 patients. In all reported cases, the first line drugs such as DZP iv, phenytoin iv, etc did not work, and it was highly recommended to place the patient under barbiturate coma as early as possible.