Hironori Matsufuji

Low-dose carbamazepine therapy for benign infantile convulsions

Benign infantile convulsions (BIC) are characterized by: (1) onset at up to 2 years of age, (2) normal development, (3) mostly brief, often clustered convulsions, and (4) normal electroencephalography during the interictal stage. BIC follow a favorable course and disappear before 2-3 years of age, although convulsions for which diazepam is ineffective frequently develop. We treated 15 children (3-16 months of age) diagnosed as having BIC, excluding convulsions associated with mild gastroenteritis, with a once-daily dose of 5mg/kg of carbamazepine until up to 2 or 3 years of age. The serum concentration of carbamazepine was as low as below the effective range in six patients, but the treatment was dramatically effective in all the BIC children. Seizures did not recur in any patients during oral administration of carbamazepine. The treatment was finished in 12 patients at age 2 years, two at age 3 years, and one at 16 months-old. Therefore, we recommend the administration of a once-daily dose of 5mg/kg of carbamazepine until up to 2 or 3 years of age as a treatment for BIC.

Absence of small-vessel abnormalities in alternating hemiplegia of childhood

OBJECTIVE To investigate whether Japanese patients with alternating hemiplegia of childhood (AHC) have the similar small-vessel abnormalities in skin reported in European patients with AHC. METHODS Electron microscopic observation of biopsied skin specimens were carried out in six Japanese patients with AHC. All patients (aged 5-17, all boys) had been diagnosed with AHC through their typical clinical courses and symptoms. RESULTS No abnormal findings in both endothelial cells and smooth muscle cells in skin small-vessels were obtained in the present study, either in the five flunarizine responders or in the one non-responder. CONCLUSIONS From our observations, we hypothesized that there may be some subtypes of AHC. The diverse clinical courses in patients with AHC and the differing efficacy of flunarizine treatment could be explained because of the heterogeneity of AHC subtypes.