High-frequency rhythmic cortical myoclonus in a long-surviving patient with nonketotic hypergylcemia.

An 11-year-old girl with nonketotic hyperglycinemia who typically presented with a picture of early myoclonic encephalopathy in the neonatal period is presented in this article. Treated early with sodium benzoate and dextromethorphan, she became seizure-free, while myoclonus persisted. During examination, multifocal rhythmic myoclonic jerks in gamma frequency enhanced by motor activity were noted. Coherence analysis of the electroencephalography-electromyography relationship indicated a cortical origin of the myoclonic jerks. Observation of this case suggests that rhythmic cortical myoclonus may represent a late evolution of this rare disorder.

Severe vesicoureteral reflux and chronic renal failure: a condition peculiar to male gender? Data from the ItalKid Project.

Primary vesicoureteral reflux (VUR), one of the principal causes of chronic renal failure (CRF), occurs as a result of two distinct and sex-related mechanisms: congenital renal hypoplasia, which is prevalent in males, and acquired renal scarring in females. We used data from the ItalKid Project, a prospective population-based CRF registry of patients undergoing conservative treatment, to evaluate the gender distribution and severity of primary VUR, the age at diagnosis, and the diagnostic and therapeutic methods adopted in children with CRF. The prevalence of males (77.5%), the severity of VUR (grade IV-V), and the early age at diagnosis (18% prenatally) seem to suggest that congenital renal damage is the major cause of pediatric CRF.