[Abnormal findings of dichotic listening test in patients with childhood adrenoleukodystrophy].

Ten Japanese boys with childhood-onset adrenoleukoqdystrophy (ALD) were evaluated with dichotic listening test (DLT). Six cases showed abnormal findings especially of laterality index (L.I.

[Visual perception of Kanji characters and complicated figures. Part 3. Visual P300 event-related potentials in patients with attention deficit/hyperactivity disorders].

In order to evaluate visual perception, the P300 event-related potentials (ERPs) for visual oddball tasks were recorded in 11 patients with attention deficit/hyperactivity disorders (AD/HD), 12 with mental retardation (MR) and 14 age-matched healthy controls. With the aim of revealing trial-to-trial variabilities which are neglected by investigating averaged ERPs, single sweep P300s (ss-P300s) were assessed in addition to averaged P300. There were no significant differences of averaged P300 latency and amplitude between controls and AD/HD patients.

[Clinical neurophysiological study of verbal and non-verbal sound perception: normal development of P300 event-related potential to different sound stimuli].

Developmental changes of P300 event related potential were evaluated in healthy children and adults aged from 7 to 29 years old, adopting 2 pairs of oddball stimuli: a non-verbal sound (tone burst: TB) pair and a verbal sound (VS) pair. P300 was evident for the target stimuli. In adult subjects, P300 was dominant at Pz for both stimuli.

Identification of 16 novel mutations in the argininosuccinate synthetase gene and genotype-phenotype correlation in 38 classical citrullinemia patients.

Classical citrullinemia (CTLN1), a rare autosomal recessive disorder, is caused by mutations of the argininosuccinate synthetase (ASS) gene, localized on chromosome 9q34.1. ASS functions as a rate-limiting enzyme in the urea cycle.

[Visual perception of Kanji characters and complicated figures. II. Visual P300 event-related potentials in patients with mental retardation].

In order to objectively evaluate visual perception of patients with mental retardation (MR), the P300 event-related potentials (ERPs) for visual oddball tasks were recorded in 26 patients and 13 age-matched healthy volunteers. The latency and amplitude of visual P300 in response to the Japanese ideogram stimuli (a pair of familiar Kanji characters or unfamiliar Kanji characters) and a pair of meaningless complicated figures were measured. In almost all MR patients visual P300 was observed, however, the peak latency was significantly prolonged compared to control subjects.

[Visual perception of Japanese characters and complicated figures: developmental changes of visual P300 event-related potentials].

In order to evaluate developmental change of visual perception, the P300 event-related potentials (ERPs) of visual oddball task were recorded in 34 healthy volunteers ranging from 7 to 37 years of age. The latency and amplitude of visual P300 in response to the Japanese ideogram stimuli (a pair of familiar Kanji characters or unfamiliar Kanji characters) and a pair of meaningless complicated figures were measured. Visual P300 was dominant at parietal area in almost all subjects.