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Volume 28, Issue 5
Displaying 1-16 of 16 articles from this issue
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[in Japanese]1996 Volume 28 Issue 5 Pages 378
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (144K) -
Masanobu Kojo, Teruyuki Ogawa, Naoki Fukushima, Keisuke Sato, Hirotomi ...1996 Volume 28 Issue 5 Pages 379-384
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe obtained the cardio-respiratory impulse response and noise contribution ratio to study the central respiratory control system in one case of Ondine's curse, one case of sleep apnea syndrome and 34 cases of preterm infants, using multivariate autoregressive analysis. In Ondine's curse, the noise contribution ratio decreased during sleep. In the sleep apnea syndrome, the noise contribution ratio before apnea and after apnea decreased. In the preterm infants, the noise contribution ratio increased with the conceptional age. From these results, we concluded that the central cardio-respiratory control system was disturbed in Ondine's curse, sleep apnea syndrome and preterm infants. The cardio-respiratory impulse response using a multiple autoregressive analysis was useful for prediction of sudden infant death syndrome.View full abstractDownload PDF (2748K) -
Masaki Yoshimura, Jun Tohyama, Yoshihiro Maegaki, Yukinori Maeoka, Tat ...1996 Volume 28 Issue 5 Pages 385-390
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSNine children with congenital rubella syndrome were examined with respect to the relation between clinical symptoms and the findings of computed tomography (CT, 9/9 cases) and magnetic resonance imaging (MRI, 7/9 cases). All patients had deafness, and three had relatively severe sequelae in the central nervous system (CNS), such as mental retardation (MR), cerebral palsy (CP) or microcephaly. In four patients, dilatation of the lateral ventricles was found by CT; in four patients, low-density areas were noted in the periventricular white matter and/or the subcortical white matter; one patient showed a spotty calcified area in the lenticula. No abnormal findings were found by CT in other three patients. MRI in seven children demonstrated areas of prolonged T1 and T2 relaxation times in the white matter in all of them.
In relation to clinical symptoms, five patients without dilatation of the lateral ventricles had no sequelae except deafness. On the other hand, in four patients with dilatation of the lateral ventricles, three had MR, CP or microcephaly. This study showed that there was a close relation between the ventricular dilatation and sequelae in CNS, whereas abnormal intensity areas in the white matter found by MRI were not related well to the sequelae of CNS.View full abstractDownload PDF (8789K) -
Norikazu Shimizu, Mariko Suzuki, Yukitoshi Yamaguchi, Tsugutoshi Aoki, ...1996 Volume 28 Issue 5 Pages 391-397
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA nation-wide survey for Wilson disease was performed from 1990 to 1991. We studied clinical features and copper content in liver for the neurologic and hepato-neurologic types. A questionnaire was sent to more than five thousand hospitals in Japan. Thirty-three percent of physicians completed the questionnaire. Four hundred and twenty-five cases were studied for the onset age, primary symptoms, prognosis and hepatic copper content. The onset age of neurologic and hepato- neurologic type of Wilson disease was usually 6 years or older. The most common initial symptom was dysarthria. Gait disturbance, flapping tremor and Kayser-Fleischer rings were also very common symptoms. We conclude that in patients with disarthria and/or extra pyramidal symptoms over 6 years of age, Wilson disease should be considered. The prognosis quod vitam of patients with neurologic and hepato-neurologic Wilson disease is not always fatal. However, many patients required prolonged treatment at either a hospital, sanatorium or at home due to irreversibility of their severe neurological defects. This result shows that early detection is the most important factor for a promising prognosis. Copper content in liver was examined for each type of Wilson disease. Neurologic type of Wilson disease had the highest copper content, followed by hepato-neurologic type. Hepatic type had the lowest copper level out of these three forms of the disease. The mechanism of onset for each type of Wilson disease should be studied using these results.View full abstractDownload PDF (976K) -
Yasuhiro Suzuki, Tomoko Kita, Toshiyuki Mano, Hiroshi Arai, Taro Matsu ...1996 Volume 28 Issue 5 Pages 398-402
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reviewed the outcome (seizures and development) of 25 cases with West syndrome in which antiepileptic drugs (valproate sodium, clonazepam) or high doses of vitamin B6, instead of ACTH therapy, were administered for the initial treatment. Five of 9 cryptogenic cases (56%) and 4 of 16 symptomatic cases (25%) showed complete cessation of spasms. In cryptogenic cases, response to vitamin B6, valproate sodium or clonazepam was not predicted by clinical features (age of onset, seizure type, initial EEG finding or treatment lag). On the other hand, response to these drugs was correlated with some clinical findings in symptomatic cases; all infants with neurocutaneous syndrome (tuberous sclerosis, neurofibromatosis type 1) had controlled infantile spasms, while none of patients with severe neonatal asphyxia or with prior other seizures responded. Twenty of 25 patients have been followed-up. The average age at follow-up was 5 years and 8 months. Prognosis of both cryptogenic and symptomatic “responders” was favorable; all had seizures controlled, and 50% had normal psychomotor development or only mild impairment (DQ>70). Symptomatic “nonresponders” had the worst prognosis. Our results suggest that choice of drug in the initial treatment of West syndrome should be determined by clinical features (especially etiology).View full abstractDownload PDF (718K) -
Tetsuzo Tagawa, Katsumi Imai, Kazumasa Otani, Yusuke Itagaki, Yuji Tan ...1996 Volume 28 Issue 5 Pages 403-408
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSSingle photon emission tomography (SPECT), MRI and EEG were studied in two infants with lissencephaly. One infant had generalized tonic convulsions at 7 month of age and continuous high voltage fast activities on EEG. The other infant developed infantile spasms at the age of 2.5 months, and showed low voltage disorganized background with pseudorhythmic high voltage sharp waves on EEG. Cranial CT and MRI in both infants revealed abnormally thick cortex, dilatation of the lateral ventricles, and smooth or relatively smooth cerebral surface. In SPECT studies, a diffuse increase of tracer uptake was shown at the cerebral cortex compared with the basal ganglia or cerebellum in both infants. The SPECT finding might reflect the abnormal cortical architectures in this cerebral malformation.View full abstractDownload PDF (7862K) -
Kimiko Nagata, Shigeko Shinomiya, Keiichi Takahashi, Toshiaki Masumura1996 Volume 28 Issue 5 Pages 409-417
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe frequency and power of EEG sleep spindles during sleep stage 2 were analysed in 56 subjects between the age of 3 to 25 years in order to define the maturational pattern of two types of spindles. Although 24 of the 56 subjects were medicated with a single anticonvulsant, there was no remarkable difference in spindle maturation patterns between the subjects who received monotherapy and unmedicated subjects. The frontal spindles matured differently than centro-parietal spindles. The frequency of centro-parietal spindles developed gradually with age, whereas frontal spindles matured with a rather sudden increase of frequency during puberty. Although the power of centro-parietal spindles showed little changes with age, the power of frontal spindles declined remarkably during the first decade and settled down to a constant level after puberty.The developmental characteristics of these two kinds of spindles may reflect a difference in synchronicity of the thalamo-cortical pathway maturation including the inhibitory system. Both centroparietal spindles and frontal spindles tended to develop slightly earlier in females than in males. The lag of spindle maturation in males might implicate developmental precedence in females. Separate observation of these two types of spindles is necessary for better understanding of sleep spindles as an indicator of central nervous system development.View full abstractDownload PDF (3012K) -
A Cognitive-Neuropsychological and Electrophysiological AnalysisAkira Uno, Makiko Kaga, Masumi Inagaki, Masato Kaneko, Noriko Haruhara ...1996 Volume 28 Issue 5 Pages 418-423
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a learning disabled child with a specific disorder involving Kanji writing. The patient was a 13-year-old boy. Although he manifested generally normal intellectual ability and his performance on WISC-R indicated that VIQ was 101 and PIQ 84, he could only write approximately 20% of Kanji, which he learned at age 6 to 7. On cognitive-psychological tests, he showed a visual cognition disorder that was different from that in a previously reported case during information processing. Electrophysiologically, he showed a disorder in visual task-relevant potentials. The mechanism underlying this patient appears to be a retrieval disorder for complex graphic forms in addition to a visual cognition disorder because of two reasons. One is that he could distinguish Kanji from similar graphic forms. The other is that he could read Kanji aloud.View full abstractDownload PDF (560K) -
Akihisa Okumura, Fumio Hayakawa, Kuniyoshi Kuno, Seijun Hayakawa, Yosh ...1996 Volume 28 Issue 5 Pages 424-429
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a case of type I lissencephaly/pachygyria which had cystic changes in the periventricular white matter and lentiform nuclei. The patient developed neonatal seizures and was referred to Anjo Kosei Hospital. His seizures were frequent and refractory to anticonvulsants. His development was severely retarded. CT and MRI revealed cystic changes in the periventricular white matter and lentiform nuclei as well as bilateral diffuse pachygyria and agenesis of corpus callosum. He died of unknown cause at 5 months of age and postmortem examination was performed. Type I lissencephaly/ pachygyria, almost complete agenesis of corpus callosum, leptomeningeal glioneuronal heterotopia and hypoplasia of corticospinal tract were seen pathologically. Marked gliosis and CD 68 positive macrophages were found around the cystic lesions in the periventricular white matter and lentiform nuclei, which suggests that these lesions were the secondarily destructive lesion. We considered that these secondary lesions were due to frequent seizures which could cause insufficient supply of blood and glucose in those areas.View full abstractDownload PDF (8815K) -
Masaaki Nishimura1996 Volume 28 Issue 5 Pages 430-433
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSTwo severely handicapped patients developed intractable hiccup during phenytoin therapy. In these two patients, serum phenytoin levels revealed 32.6μg/ml and 17.6μg/ml, respectively. Since large doses of phenytoin cause delayed latencies, and depressed or lost of brainstem auditory evoked potentials, it can be speculated that intractable hiccup could occur through dysfunction of brainstem by phenytoin.View full abstractDownload PDF (2655K) -
Shinjiro Akaboshi, Akiko Miyashita1996 Volume 28 Issue 5 Pages 434-437
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe report a 10-month-old boy with heat stroke because he was left in a car. He showed hyperthermia, coma and convulsions at the time of his admission. Liver dysfunction and coagulopathy were observed, but they were improved after several days. Consciousness was gradually recovered, but currently he shows neurological sequelae. Cranial CT showed brain edema until the 7th hospital day. Cranial MRI on the fortieth hospital day showed the finding of cortical laminar necrosis in the vascular boundary zones. This finding suggest that brain ischemia was related with the neurological involvement of heat stroke.View full abstractDownload PDF (5756K) -
Masaki Shimizu, Shinichiro Hamano, Takahiro Nara, Yoshikatsu Eto, Kihe ...1996 Volume 28 Issue 5 Pages 438-442
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported two cases of anterior spinal artery syndrome in childhood evaluated by MRI of the spinal cord. The T2-weighted MRI revealed high signal intensity in the anterior region of spinal cord in both cases. These findings might be related with edema of the ischemic region. Several years later, the regions of the spinal cord showed atrophic changes. The high signal intensity on the T2-weighted MRI was characteristic of the anterior spinal artery syndrome.View full abstractDownload PDF (8871K) -
Hiroko Wada, Hisahide Nishio, Shoko Nagaki, Hiroshi Yanagawa, Atsuko I ...1996 Volume 28 Issue 5 Pages 443-447
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA2-month-old girl had generalized weakness, profound muscular hypotonia, hepatomegaly and severe lactic acidosis. She needed ventilatory support. Muscle specimen taken at 2 months showed ragged-red fibers, abnormal mitochondria, and reduced cytochrome c oxidase (CCO) staining. Biochemical analysis showed CCO activity to be reduced to about 16% of the normal mean. She received carnitine and coenzyme Q10 supplementation from the age of 3 months and abnormal blood lactate values declined to near normal values during the first three weeks. Gradually her condition started to improved she held her head at 9 months, and walked alone at 15 months. The second biopsy specimen at 3 years and 8 months showed almost normal CCO staining and she was free of clinical signs. This case is an example of a rare benign infantile mitochondrial myopathy caused by CCO deficiency. Early diagnosis is crucial to provide intensive treatment until spontaneous clinical improvement appears. We concluded that carnitine and coenzyme Q10 supplementation was a useful treatment for clinical improvement in patients with a benign infantile mitochondrial myopathy caused by CCO deficiency.View full abstractDownload PDF (4052K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1996 Volume 28 Issue 5 Pages 448-450
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese]1996 Volume 28 Issue 5 Pages 450-453
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese]1996 Volume 28 Issue 5 Pages 460-465
Published: September 01, 1996
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (844K)
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