Acute encephalitis with refractory, repetitive partial seizures

Acute encephalitis with refractory, repetitive partial seizures (AERRPS) is a new epileptic syndrome described by Sakuma in Japan in 2001. The clinical manifestations, neuroimaging findings and outcome of AERRPS in Taiwan have not been reported.

From 2000 to 2006, we collected cases that fulfilled the diagnostic criteria of AERRPS and analyzed the clinical course, virology, medication, electroencephalographic findings, neuroimaging characters and prognosis retrospectively.

Fourteen children aged from 1 year and 2 months to 15 years and 6 months were enrolled. They presented with prodromic symptoms including fever (n=13, 92.9%), upper respiratory tract infection symptoms (n=12, 85.7%) and gastrointestinal tract discomfort (n=6, 42.9%). Seizures occurred 3-14 days after antecedent symptoms with patterns of generalized tonic-clonic seizures, focal seizures or myoclonic seizures. The seizures were refractory to combinations of antiepileptic drugs (AEDs). Prolonged fever (n=14, 100.0%), hypersensitivity to AEDs (n=11, 78.6%) and liver function impairment (n=8, 57.1%) were noted during the period of hospitalization. The follow-up EEG findings were similar to those of initial findings including epileptiform discharges and/or generalized background slow waves. Initial brain MRI scans were normal but later showed focal or multifocal abnormal signal intensity followed by generalized brain atrophy in more than 50% of cases. The general prognosis was poor with variable psychomotor retardation and persistence of refractory epileptic seizures.

The similarities of the clinical features support AERRPS as a new epileptic syndrome. More study is needed to specify the etiology of the syndrome as the first step for more effective treatment.