All issues
Volume 21, Issue 6
Displaying 1-17 of 17 articles from this issue
- |<
- <
- 1
- >
- >|
-
[in Japanese]1989 Volume 21 Issue 6 Pages 516
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (120K) -
Part 1. Prevalence of the Rett SyndromeKayo Fujino, Toshiaki Hashimoto1989 Volume 21 Issue 6 Pages 517-522
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe Rett syndrome (RS) is currently diagnosed by its characteristic clinical features and course since specific biochemical markers have not been established. These features include psychomotor regression after early periods of normal development, autistic appearances, hand stereotypy and loss of purposeful hand skills. Since these features may be seen in other children with neurological disorders, it is possible that many patients who admitted to residential facilities or schools have RS but have not been diagnosed as such. We undertook a survey of the schools and residential facilities for handicapped children at Tokushima Prefecture to determine the prevalence of RS in the Japanese. The prevalence of RS in Tokushima prefecture was 0.36/10, 000 girls at age 15 and under (0.37/10, 000 girls at 7-15 years). The prevalence of RS in our study was lower than the Europian studies.View full abstractDownload PDF (747K) -
Reiko Morita, Kazutoshi Nakano, Yukiko Hirano, Tatsuro Izumi, Yoshito ...1989 Volume 21 Issue 6 Pages 523-528
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA high-dose intravenous gamma-globulin (IVGG) therapy was given to two children ; one with intractable dermatomyositis (case 1) and the other with polymyositis (case 2). Case 1, a 3-year-6-month-old girl, only poorly responded to the standard oral prednisolone therapy and a pulse therapy with high-dose methylprednisolone. This patient showed a complete remission after a course IVGG therapy, 100 mg/kg/day iv. for 5 days, which has been maintained for 6 months up to the present. Case 2, a 13-year-6-month-old girl, also showed little improvement after the oral prednisolone therapy, and transient improvement during plasma exchange therapy. This patient showed fairly good response after three repeated courses of IVGG therapy, but not complete remission.View full abstractDownload PDF (584K) -
Takahisa Fuse, Takuji Takagi, Mitsuhito Mase, Hajime Nagai, Tatsuo Ban ...1989 Volume 21 Issue 6 Pages 529-536
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSMagnetic resonance imaging (MRI) was conducted on infants with lumbosacral lipomas. Based on Chapman's classification (1982), these MRIs were classified into three representative types: caudal, dorsal and transitional. As confirmed by intraoperative observations, the relation between the lipomas and the spinal cord was clearly disclosed in the preoperative MRI. However, in the dorsal type, the intraoperative view of the margin between the lipomas and spinal cord was different from the MRI findings. Postoperative MRI disclosed the existence of residual lipomas and the change in the low conus medullaris. Moreover, preoperative and postoperative changes in the hydromyelia could be observed.View full abstractDownload PDF (11702K) -
Miki Yasujima, Yukuo Konishi, Masanori Kuriyama, Masakatsu Sudo, Katsu ...1989 Volume 21 Issue 6 Pages 537-542
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSCT and MRI in 13 patients with infantile spasms were analyzed. Cortical atrophy was found in 8 cases and ventricular dilatation in 9 cases. The patients with severe cortical atrophy or ventricular dilatation had poorer prognosis than the patients with normal CT findings. In addition to cortical atrophy and ventricular dilatation which were shown in CT, MRI revealed poor differentiation of gray and white matters in all cases and periventricular hyperintensity area in 9 patients. Six patients with the periventricular hyperintensity area of gradeffi and Arhad severe developmental delay. With regard to the prognosis, MRI plays a more useful role in detecting intracranial pathology in infantile spasms.View full abstractDownload PDF (3632K) -
Histological and Biochemical AnalysisMakoto Suzuki, Hideo Sugie, Satoshi Tsurui, Reiko Miyamoto, Youko Sugi ...1989 Volume 21 Issue 6 Pages 543-549
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a 3-week-old girl with profound generalized weakness, requiring assisted ventilation. There was no consanguinity or family history of any neuromuscular disorders. Serum levels of GOT, GPT and CK were slightly elevated. Although the clinical manifestations mimicked those of Werdnig-Hoffmann disease, a lack of the respiratory muscle involvement and the presence of high serum lactate levels suggested an underlying metabolic disorder. During the observation over the months, she gradually improved clinically and assisted ventilation was discontinued at 19 months. Muscle biopsies were performed at 4 and 19 months of age. The first biopsy showed an excessive mitochondrial aggregation and numerous ragged-red fibers. The second biopsy revealed rare ragged-red fibers. However there were definite neurogenic changes including type grouping and angulated fibers. Cytochrome c oxidase (CCO) stain was positive in less than 5% of fibers in the first biopsy but in all fibers in the second biopsy. Biochemical analysis using muscle specimens showed a decreased CCO activity; 18.5% of the control level in the first biopsy and 53.3% in the second biopsy. Immunocytochemistry showed the presence of immunologically reactive enzyme protein in both biopsies. Clinical manifestations of our patient were almost identical to those of two cases reported by DiMauro, et al (1983).
The enzyme defect in this case was reversible in contrast to that in the fatal infantile form of CCO deficiency.
Histochemical and biochemical bases for these differences were discussed.View full abstractDownload PDF (9436K) -
Makiko Kaga, Yoshisato Tanaka, Masaru Takamizawa, Haruko Naitoh, Kenji ...1989 Volume 21 Issue 6 Pages 550-556
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSOne hundred and forty-seven out of 1, 600 infants and children showed no detectable ABR. ABRs were evoked by clicks. As to hearing acuity, 121 out of 147 patients had sensorineural hearing loss and 19 had no hearing impairment of high frequency (3-4 kHz) and high sound intensity (85-90 dBnHL). Eight patients could not be diagnosed whether they had hearing impairment or not.
Among 19 patients who did not have sensorineural hearing loss in those ranges described above, 5 patients had cerebral palsy with severe mental retardation, 4 had mitochondrial encephalomyopathy, 3 had degenerative disease of unknown etiology, 2 patients had Down syndrome, 1 was a low birth weight infant with respiratory distress syndrome, 1 was a neonate born to a diabetic mother, 1 experienced severe neonatal asphyxia, 1 patient had acute lymphoblastic leukemia and 1 infant was later confirmed as a healthy girl. Of these, four patients showed transiently negative response in ABR.
Nine patients remained undiagnosed as to hearing because they were in deep coma, had profound brain damage, or died in early neonatal period. They had major neurological diseases: severe neonatal asphyxia, holoprocencephaly, microcephaly, degenerative diseases of the central nervous system of unknown origin, metachromatic leukodystrophy, trisomy of chromosome 18, anoxic encephalopathy, small for gestational age newborn with intracranial bleeding and posthemorrhagic hydrocephalus .
When ABR cannot be recorded using high sound intensity clicks, it usually suggests presence of hearing impairment of high frequency range. However, there are patients who have no hearing impairment in those sounds. Most of such patients have underlying neurological disorders. Improvement in ABRs without otological involvement was characteristic of pediatric patients.View full abstractDownload PDF (877K) -
Seiichi Shimada, Hiroshi Tamai, Masahisa Funato1989 Volume 21 Issue 6 Pages 557-562
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSChildren who require prolonged respiratory support often have to spend their whole lives in the hospital. It is important, however, for these children to have time with their families in their own homes. Home mechanical ventilation can make it possible. In Japan, the concept of home mechanical ventilation has just begun to open up. We reported five cases of home mechanical ventilation for children by using a portable respirator (Bennett, Companion 2800). Two cases of these were patients with hypoxic ischemic encephalopathy. The others were cases with neuro-muscular diseases (Werdnig-Hoffmann disease, mitochondrial myopathy, myotubular myopathy). Before taking the patients out, parents received a trainingin nursing procedures and in skillful use of the ventilator and equipment.
The patients went out on excursions 17 times, and were released for home stay a total of 25 times. Initially, 12 excursions were undertaken with doctors or nurses in attendance later, however, all of the home stays and 5 of the excursions were carried out successfully by the family only. They went to places such as their own home, a park, a zoo, an aquarium and a museum. The average time was 4.6 hours (3-8 hours) for excursions, and 1.8 days (1-7 days) for home stays. Questioning of family revealed that home mechanical ventilation accelerated the social and emotional development of these children whose condition necessitated respiratory support, especially patients with neuro-muscular diseases. It also revealed that family could accept the patient as a member with more familiarity and with more ease. In terms of more humane medicine and of quality of life, home mechanical ventilation of children should be emphasized in Japan.View full abstractDownload PDF (4216K) -
Report of a Case and Summary of Reported Patients in JapanJun Kohyama, Fumio Niimura, Chikao Fukuda1989 Volume 21 Issue 6 Pages 563-568
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA 13-day-old male new-born was admitted to our hospital because of prolonged fever from eleventh day of his life. Physical examinations were unremarkable with good feeding ability, but an increase of the white cell count in the cerebrospinal fluid was noted. In spite of the intravenous administration of antibiotics and gamma-globulin, intractable focal seizures were developed on the fifth day of hisillness, and his consciousness level was decreased. Herpes simplex virus (HSV) encephalitis was strongly suggested by the results of brain computed tomography (low density on the right temporal region) and electroencephalography (right temporal sharp waves recorded in concordance with clonic seizures of the left arm), and acyclovir was started on the sixth day of his illness. His condition was remarkably improved, and then he could drink milk again on the tenth day of his illness. The changes of the antibody titers supported our diagnosis. Among the previously reported twenty-nine patients of neonatal HSV encephalitis in Japan, seven died and twelve were severely handicapped. These grave prognoses may be improved by the early administration of antiherpetic agents to the febrile neonates with intrctable (focal) seizures regardless of theabsence of mucocutaneous lesions.View full abstractDownload PDF (3314K) -
Jun-ich Oki, Nobuhiko Sasaki, Yuichi Kusunoki, Kazuhiko Cho1989 Volume 21 Issue 6 Pages 569-573
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA case of 10-year-old female with hereditary progressive dystonia with marked diurnal fluctuation (HPD) was described. Her development was normal. Then at the age of eight, she developed dystonia of the left lower extremity that became noticeable toward evening but subsided aftertaking sleep. And the velocity of height gain decreased at the age of nine (from 5 cm to 3.6 cm per year). Before the diagnosis of HPD, small doses of haloperidol (0.015 mg/kg/day) were given to her. Thereafter she developed progressive dystonia of all extremities and could not walk. Administration of haloperidol wasdiscontinued, and an attempt to reverse the dystonia with L-Dopa was made. Prior to treatment with L-Dopa, a reduced concentration of cerebrospinal fluid homovanillic acid (10 ng/ml) was found. And her height was 130.5 cm (-1.33 SD) at the age of ten. Treatment with L-Dopa (18 mg/kg/day) markedly improved hersymptoms within four days. This improvement has been maintained for six years without a need for increasing the doses and without neurological side effects. At the age of sixteen her height was 162.3 cm (+1.0 SD). These findings suggest a diminished dopaminergic activity and a increased susceptibility to dopamine receptor blockade in HPD.View full abstractDownload PDF (2805K) -
Masashi Mizuguchi, Kazuhiko Komiya1989 Volume 21 Issue 6 Pages 574-578
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA typical case with progressive facial hemiatrophy was treated with a new therapeutic trial, stellate ganglion block.
The present case, a Japanese girl, suffered from progressive atrophy involving the soft tissue of the left buccal region, with onset at the age of 6 following a minor local trauma. She visited our hospital at the age of 9, and as soon as the diagnosis was made, left stellate ganglion block was initiated. Local injection of lidocaine was performed 53 times over the period of 1 year and 4 months. During this period of therapy and the subsequent follow-up period of 4 years, the state of atrophy remained unchanged and nonprogressive.
Stellate ganglion block as a therapy against progressive facial hemiatrophy was considered to be worth further evaluation, although it was impossible to judge, based solely on our experience, whether the arrest of progression was attributable to the therapy.
The rationale of this therapy was also discussed. It was based upon the assumption that atrophy may result from irritation of the cervical sympathetic nerve, one of the most popular theories regarding the pathomechanism of progressive facial hemiatrophy.View full abstractDownload PDF (3446K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1989 Volume 21 Issue 6 Pages 579-581
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese]1989 Volume 21 Issue 6 Pages 581-583
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese]1989 Volume 21 Issue 6 Pages 583-585
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese]1989 Volume 21 Issue 6 Pages 585-587
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (415K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1989 Volume 21 Issue 6 Pages 588-589
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese]1989 Volume 21 Issue 6 Pages 590-591
Published: November 01, 1989
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (301K)
- |<
- <
- 1
- >
- >|