[Effectiveness and safety of long-term levetiracetam treatment in patients with refractory epilepsy].

Objective: To evaluate the long-term effects and tolerability of levetiracetam (LEV) in refractory epilepsy.

Methods: LEV was administered to 76 patients whose seizures were inadequately controlled by their current medications. The patients were followed for a minimum of 18 months but less than 2 years.

Nationwide survey of Arima syndrome: revised diagnostic criteria from epidemiological analysis.

Aim: We have never known any epidemiological study of Arima syndrome since it was first described in 1971. To investigate the number of Arima syndrome patients and clarify the clinical differences between Arima syndrome and Joubert syndrome, we performed the first nationwide survey of Arima syndrome, and herein report its results. Furthermore, we revised the diagnostic criteria for Arima syndrome.

[Effect of topiramate for patients with intractable epilepsy].

The effects of topiramate (TPM) were evaluated in 51 patients with intractable epilepsy. Callosotomy and hemispherotomy were performed in 16 patients and one patient before the administration of TPM, respectively. The 50% responder rate (50%RR) was recorded in 39% of the total patient population and in 58% of patients with symptomatic location-related epilepsy (SLE).

[The effect of parent training program on children with attention deficit/hyperactivity disorders and/or pervasive developmental disorders].

Mothers of 18 children with attention deficit/hyperactivity disorders (AD/HD) and 6 with pervasive developmental disorders (PDD) underwent a parent training (PT) program. After the program, the Beck Depression Inventory- II (BDI - II) score, which indicates parenting stress, significantly decreased from 15 to 8 (p=0.036).

[Case of drug-induced hypersensitivity syndrome due to lamotrigine: demonstration of sequential reactivation of herpesviruses].

Drug-induced hypersensitivity syndrome (DIHS) is a rare but severe multiorgan disorder. The reactivation of human herpesvirus-6 (HHV-6) and other human herpesviruses has been reported to be associated with its pathogenesis. We herein report a case of 14-year-old female who developed DIHS during the treatment with lamotrigine, a novel antiepileptic drug.

Novel mutations in the SIL1 gene in a Japanese pedigree with the Marinesco-Sjögren syndrome.

Marinesco-Sjögren syndrome (MSS) is a rare autosomal recessive disorder. Mutation in the SIL1 gene accounts for the majority of MSS cases. However, some individuals with typical MSS without SIL1 mutations have been reported.