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Background: Guillain-Barré syndrome (GBS) is an acute acquired demyelinating polyneuropathy, presumed to be immune-mediated. Intravenous immunoglobulin (IVIg) has been used to treat GBS and was found to be effective. However, a well-controlled study of pediatric GBS has not been conducted in Japan. Therefore, to evaluate the efficacy of IVIg in the treatment of GBS, an open-labeled study was performed in pediatric patients., Methods: Participants in the study were required to be younger than 15 years old, and diagnosed as having moderate or severe GBS. IVIg (400 mg/kg per day) was administered to patients for five consecutive days. Predefined outcome measures were defined on a seven-point scale of motor function (Hughes' functional grade [FG])., Results: Eleven patients were treated with IVIg. The median time taken to improve by one grade on the FG scale was 10.0 days after initial treatment. Two weeks after initial treatment, 72.7% of patients treated with IVIg improved by one or more grades, and 36.4% improved by two or more grades, measured on the FG scale. After 4 weeks an improvement by one or more grades was observed in 81.8% of patients, and two or more grades in 63.6% of patients. These improvement rates were markedly greater than would occur with the natural course of GBS1. Adverse events (subjective symptoms or abnormal laboratory findings) were observed in four patients, although all were temporary and mild., Conclusions: The authors conclude that IVIg is a safe and effective treatment for childhood GBS, which shortens the time to recovery.

We recorded a P300 component of event-related potentials associated with auditory oddball tasks in nine absolute pitch (AP) possessors and seven non-AP possessors. The previous studies demonstrated that AP possessors did not appear to employ working memory during auditory oddball tasks because they have a fixed tonal template in their memories. However, the present findings showed that the AP possessors exhibited similar P300 as the non-AP possessors and did update the tonal context in the auditory oddball tasks. This result suggests that the AP possessors do not always refer to the fixed tonal template in their memories when executing the oddball tasks and they employ working memory properly according to the difficulty of the auditory tasks., (Copyright 2002 Elsevier Science Ltd.)

Spinal muscular atrophy is an autosomal recessive neurodegenerative disorder with progressive weakness and atrophy of voluntary muscles. The survival motor neuron gene (SMN) is present in two highly homologous copies (SMN1 and SMN2) on chromosome 5q13. Homozygous deletion of exons 7 and 8 of SMN1 is responsible for spinal muscular atrophy. In spinal muscular atrophy patients, SMN2 partially compensates for the lack of SMN1. Previously, we reported the relatively high incidence of a large deletion including the SMN1 region in Japanese spinal muscular atrophy type I patients. In order to further establish the genetic background of Japanese spinal muscular atrophy type I patients, we investigated the SMN1/SMN2 ratio in the carriers. In normal individuals, there is one copy of each gene on the chromosome (the SMN1/SMN2 ratio was 1). Among 15 carriers (14 parents and one carrier sibling of Japanese type I spinal muscular atrophy patients with homozygous deletion of exons 7 and 8 of SMN1), we found that the SMN1/SMN2 ratio was 0.5 or 1 in 11 (73.3%) carriers. The remaining four carriers had an SMN1/SMN2 ratio of 1/3. This finding supports the idea that deletion rather than conversion is the main genetic event in type I spinal muscular atrophy. In addition, the ratio of SMN1/SMN2 among Japanese carriers, which was thought to be higher than that of the Western population, was compatible with the results obtained in Western populations. For further insight into the characteristic genetic background of spinal muscular atrophy in Japanese, determination of the gene copy number is essential.

Development of medical knowledge and technology has greatly improved our understanding of mental retardation. However, clinically applicable treatment of mental retardation is still limited. As described in the definition of mental retardation by the American Association of Mental Retardation, functional maladaptations in various aspects of everyday life are the most integral part of disabilities in mental retardation. The role of pediatric neurologist in the medical care for children with mental retardation is not limited in medical treatment. Pediatric neurologists should play an important role as a coordinators in integrated care. Providing of medical care in special schools for children with mental retardation is among the most important issues, since children spend most of their time either at home or in school. The current situation of "medical care" in school, and its problems are discussed.

Ethical attitudes of Japanese physicians regarding life-sustaining treatment for children with severe neurological disabilities (SND) were investigated by mailing a translated questionnaire which the Child Neurology Society (CNS) of the United States used for their survey. The questionnaire was sent to 202 council members of the Japanese Society of Child Neurology (JSCN), and the answers of 147 respondents (72.8%) were analyzed. It was found that the majority (85. 0%) of respondents believed that the same level of care should be provided to children with SND as those without it. However, fewer respondents (15.6%) believed that cardiopulmonary resuscitation was indicated for children with progressive or degenerative brain disorders. With respect to the authoritative role of medical indications and family/guardian's wishes in clinical decision-making for children with SND, about 30% of respondents believed that medical indications should override family/guardian's wishes. However, almost as many respondents (29.9%) chose an ambivalent answer. If compared with the results of the preceding CNS survey, considerably more respondents gave ambivalent answers (average 26. 6%) than in the CNS survey (5.8%). About half of the respondents (49. 0%) acknowledged the need for ethical guidelines to help physicians make ethically difficult decisions. Although statistical comparison was not possible, there were considerable differences between the results of the current study and those of the CNS survey.

We conducted molecular analysis of two candidate genes for spinal muscular atrophy (SMA), the survival motor neuron gene (SMN) and the neuronal apoptosis inhibitory protein gene (NAIP), in 16 Japanese patients with SMA and compared the phenotypic features of SMA in these patients with the corresponding genotypes. Exons 7 and/or 8 of SMN were homozygously deleted in 11 SMA type I (Werdnig-Hoffmann disease) patients, two SMA type II patients and one SMA type III patient. Exons 5 and 6 of NAIP were homozygously deleted in six SMA type I patients. No patient had a deletion in NAIP without a deletion in SMN. Mechanical ventilation was required during the first 7 months of life in the SMA type I patients who had a deletion in both SMN and NAIP. Ventilatory support was initiated within 2 years after birth in patients who had a deletion in SMN but not in NAIP. We detected homozygous deletion of exon 5 of NAIP in the unaffected mothers of two SMA type I patients. In these families, the patients exhibited a deletion in both SMN and NAIP. The parents and unaffected siblings of these patients did not have a deletion in SMN. The present findings support the hypothesis that SMN deletion plays an important role in the development of SMA and suggest that combined deletion of both SMN and NAIP may be relevant for determining the disease severity.

Between April 1990 and March 1991, postoperative adjuvant chemotherapy for resected gastric cancer employing 5-fluorouracil, epirubicin and mitomycin C (FEM) was performed. Forty-two patients subjected to the therapy were considered to have positive serosal invasion and underwent curative operation. FEM therapy consisted of intraoperative intraperitoneal administration of mitomycin C (0.3-0.4 mg/kg) combined with 8 cycles of intravenous bolus injection of epirubicin (20 mg/body) every 2-3 weeks which was started 2 weeks after the operation. Daily oral administration of 5-fluorouracil (150-200 mg/body) was started 2 weeks after the operation and continued for more than 6 months. Thirty-four of the 42 cases were assessable. Major adverse effects were nausea, vomiting, and general fatigue. There were no cardiovascular symptoms. The cumulative two-year survival rate was 74.2%, and follow-up was still under way at this writing.

The Cooperative Program for Asian Pediatricians (CPAP) is a non-government organization established in 1989 to promote mutual understanding and friendship among young pediatricians in Asian countries. Unlike other government programs and non-government organizations, CPAP is solely facilitating mutual relationships among young inexperienced pediatricians who would otherwise have no chance to travel overseas. It has been funded by donations from members of the alumni association of the Department of Pediatrics at the University of Tokyo and many private companies and individuals. The Cooperative Program for Asian Pediatricians has so far invited 36 Asian pediatricians from 11 countries. By constructing a human network among Asian pediatricians, it is hoped that CPAP will contribute to making international cooperation in the Asian region easier and smoother.

In order to assess the changing pattern of pediatric neurologic disorders during this century, we retrieved and analyzed the medical records of hospitalized patients with neurologic disorders in our hospital from 1900 to 1980. It was demonstrated that bacterial meningitis had been by far the most common cause of death until 1950. After 1950, both the incidence and the mortality rate of bacterial meningitis declined rapidly probably because of the improved medical care and introduction of various antibiotics. We noticed several interesting features in the changing spectrum of bacterial meningitis as described below. First it was demonstrated that the incidence of tuberculous meningitis declined almost a decade later than those of other kinds of purulent meningitis. Second, the incidence of bacterial meningitis declined even before the introduction of antibiotics. Although the development of antibiotics was the main contributing factor in improving the prognosis for bacterial meningitis, it is suggested from our data that other factors such as improved general supportive care and carrying out of public health programs also played an important role in improving the overall prognosis for bacterial meningitis.