Abstracts

Rationale: To describe the clinical entity characterized by severe epilepsy and severe behavioral and cognitive dysfunctions following an acute febrile seizure disorder resembling encephalitis in previously normal children, probably due to an immune-mediated cerebral disorder.Methods: We selected, both prospectively and retrospectively, patients with intractable seizures and CNS signs of an ongoing immune-mediated encephalopathy.Results: We studied 8 patients with a mean age of 11.7+7 yrs. At the age of 6.7+6.2yrs (9m-17.6yrs, median 5 yrs) they presented with fever and stupor associated with frequent partial seizures which developed in epileptic status in seven cases and subsequent seizures in one. EEG at onset showed a diffuse slow activity associated with epileptiform abnormalities involving in all cases the temporal lobe, with extension in the frontal area in one, in parietal and occipital in one and in the central regions in two cases. Viral or bacterial infections have been excluded. Oligoclonal bands were present in four out of six tested patients. Brain MRs were normal at onset and during follow-up in three cases. In the other four MRs showed at the onset T2 and FLAIR bilateral hyperintensity over peri-insular regions in three and over frontal and mesial temporal regions in one. The mean follow-up period was 4.2+2.2 yrs. All patients developed without latency severe partial epilepsy with relapsing remitting course in six. A spectrum from attention deficits to dementia has been observed in association with intermittent and severe behavioral disorder. Treatment with corticosteroids and/or IVIG determined a reduction of seizure number and improvement in competences.Conclusions: In these patients severe partial epilepsy with a wide spectrum of behavioral and cognitive disorder is associated to signs of an immune-mediated cerebral ongoing process. The immune-mediated process is suggested by inflammatory CSF changes, reinforced by MR changes and sustained by the efficacy of immune-modulating therapy. The striking improvement in some patients after corticosteroids or IVIG, confirm that the recognition of this entity is highly crucial in the outcome.