All issues
Volume 22, Issue 1
Displaying 1-18 of 18 articles from this issue
- |<
- <
- 1
- >
- >|
-
[in Japanese]1990 Volume 22 Issue 1 Pages 2
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (119K) -
Hideki Endo, Shigeichi Kobayashi, Terutoshi Nakamigawa, Hideo Shimoizu ...1990 Volume 22 Issue 1 Pages 3-8
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported two infants with hydranencephaly and chronic hypernatremia. Their plasma sodium concentration gradually increased during the first week and remained between 150-160 mEq/L thereafter. They showed no signs of thirst. A water deprivation test demonstrated low urine osmolality and low plasma ADH concentration despite markedly elevated plasma osmolality in both cases. Urine was significantly concentrated when vasopressin was given. Thus, it was concluded that both thirst mechanism and ADH secretion were disturbed in these two cases.
ADH producing cells, the thirst center and the osmoreceptor are all located in the hypothalamus. Radiographic measures showed dysplasia of the hypothalamus, providing the anatomical basis for their dysfunction.View full abstractDownload PDF (8600K) -
Hideto Yoshikawa, Norio Sakuragawa, Hatsumi Nitta1990 Volume 22 Issue 1 Pages 9-15
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported clinical features and CT, MRI findings on cerebellum and brain stem in nine patients with congenital cerebellar ataxia (CCA) which-was diagnosed by Hagberg's criteria. The patients were aged from 2 years to 14 years; five males and four females. CT and MRI were performed in nine cases and six cases, respectively. The patients were compared with sixty normal controls on CT, and with eight normal controls on MRI, aged from 1 year to 15 years.
Five cases without perinatal brain insults showed abnormal findings in the cerebellum on CT and MRI. One case out of five cases showed abnormal finding only by MRI and not apparent by CT. Four cases with neonatal asphyxia did not show abnormal findings in the cerebellum. But the measurements of CT and MRI disclosed slightly abnormal values. Cereberum and brain stem were thought to be almost normal except for cavum Vergae of one case. There were no close correlation between clinical symptoms and abnormal findings in the cerebellum on CT and MRI.
The etiology of CCA was thought to be heterogeneous, judging from CT and MRI. At least there were two causes; prenatal brain insults and perinatal brain insults. The degree of cerebellar lesion was thought to be various by its etiology.View full abstractDownload PDF (4626K) -
Part 2. Polysomnographic and Neuroendocrinological StudiesKayo Fujino, Toshiaki Hashimoto1990 Volume 22 Issue 1 Pages 16-23
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe biogenesis of the Rett syndrome (RS) is unknown although there have been several reports suggesting some biochemical defect of the monoaminergic system in CNS. We performed all night polysomnography and neuroendocrinological tests in three cases of RS discovered on a survaylance study in Tokushima. The following results were obtained; 1) dissociation of sleep elements (stage 1-REM, stage 2-REM), 2) no increase in REMs/min with development, 3) abnormal patterns of BMs, TMs of each sleep stage, 4) paradoxical secretion of growth hormone (GH) and excessive secretion of prolactine in TRH provocation test, the absence of sleep enhancement of GH secretion, 5) progressive disfunction of autonomic nervous system during sleep. These results indicated that RS involves the functional disturbances of brain stem and hypothalamic monoaminergic regulatory systems.View full abstractDownload PDF (888K) -
Toshiki Takeya, Kenzo Hamano, Koichiro Kawashima, Nobuaki Iwasaki, Tor ...1990 Volume 22 Issue 1 Pages 24-29
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSIt has been reported that cochlea is the lesion of hearing loss in FSH. However, the details of this lesion are not yet sufficiently known. We performed detailed audiologic studies to examine hearing loss in FSH.
We experienced 2 cases of FSH associated with hearing loss. Case 1 was a girl aged 5 years, and case 2 a boy aged 15 years. Clinical findings, EMG and muscle biopsy gave a diagnosis of FSH in both cases. Hearing loss was evaluated by pure tone audiography, speech audiography, tympanometry, stapedial reflex, auditory brain stem response and electrocochleography.
In case 1, pure tone audiograms revealed high tone hearing loss without an A-B gap. On speech audiography, the maximum articulation score was 100% and proved normal. The tympanogram was type A. Stapedial reflex was normal bilaterally. The threshold of the 5th wave increased markedly on auditory brain stem response. On electrocochleography, the H-curve of the input-output function curves of action potential was recorded, but the L-curve was absent.
There were no complaints of hearing loss in case 2, but pure tone audiograms revealed high-tone hearing loss without an A-B gap. The tympanogram was type A. Stapedial reflex was normal bilaterally. On auditory brain stem response, threshold was increased and latency was prolonged when intensity was lowered. The electrocochleograms were almost normal.
It has been reported that, in electrocochleography, the L-curve represents the function of the outer hair cells and the H-curve that of the inner hair cells. The electrocochleograms in case 1 showed damage to the outer hair cells. It was also reported that the outer hair cells have actin and tubulin, the important components of skeletal muscle tissue. Our result suggests that hearing loss and FSH have a common pathogenesis.View full abstractDownload PDF (5813K) -
Shinobu Kohsaka, Masako Kohsaka, Noriko Fukuda, Yuhichi Gotoh, Kimiaki ...1990 Volume 22 Issue 1 Pages 30-37
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe evaluated the efficiency of a newly developed cassette EEG recording device and our system for detecting and analyzing ictal EEGs in epileptic children.
We got 13 ictal records from 16 epileptic children. We could precisely differentiate and diagnose their classifications of seizures by comparative analyses of ictal records and clinical symptoms. We discussed the characteristics of our system, and described actual analysis methods in 3 cases as examples.
It was concluded that our system offered valuable information about ictal events in epileptic children.View full abstractDownload PDF (2261K) -
Chieko Shimomura, Masuko Funabashi, Hiroyuki Choh, Yasuyuki Suzuki, Ak ...1990 Volume 22 Issue 1 Pages 38-44
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSIn severely handicapped patients, chronic respiratory insufficiencies and recurrent hypoxemia especially during sleep are one of the major problems to take care of them. To clarify the pathophysiology of respiratory disorders during sleep, we examined 5 severely handicapped patients with no respiratory distress during wakefulness, but with signs of latent respiratory insufficiency by means of polygraphical all-night study including the arterial oxygen saturation (Sa02) monitoring. In the severely handicapped patients, sleep, hypoxemia occurred frequently. The value of Sa02 decreased in proportion to the intensity of apnea determined by polygraph. The relationship between the value of Sa02 and sleep stage was variable in each patient. Four patients had mainly obstructive respiratory disorders and one had a central type respiratory disorder. The density of REM stage decreased in the obstructive type. In the central type, the repeat of REM stage with normal REM density was not observed. In one patient with obstructive type and one with central type, hypoxemiawasimprovedwith an increased number of REM stage by O2 inhalation.View full abstractDownload PDF (864K) -
Hisaharu Suzuki1990 Volume 22 Issue 1 Pages 45-49
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSPrevalence rate of severely retarded children aged 6-14 years was studied in Tokyo. The definition of severely retarded children was those who neither walk nor have their IQs over 35. We visited total of 60 institutions and schools for retarded children in and around Tokyo. Because of the school system in Tokyo that enrolls all children regardless of their disabilities, case finding was exhaustive. The number of severely retarded identified was 830. The prevalence rate was 0.68/1, 000 on April 1, 1988.
We conclude that the prevalence rate of severe retardation is modestly lower than the rates previously reported from other areas of Japan.View full abstractDownload PDF (682K) -
Hisaharu Suzuki1990 Volume 22 Issue 1 Pages 50-54
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSPatterns of disabilities in 830 severely retarded children aged 6-14 years identified through the prevalence study in Tokyo were analyzed. Severely retarded children were defined as those who neither capable to walk nor have their IQs over 35. Sixty-four percent of the total children belonged to class 1 of Ooshima's classification (bedridden and IQs less than 20) and most of the others belonged to class 2 of Ooshima's classification (those who able to sit alone and IQs less than 20). The percentages of the complications were as follows epilepsy 84%, visual disturbance 40%. respiratory distress 30%, and feeding difficulty 46%. Sixteen percent of the total children were institutionalized.
We conclude that the proportion of the most severe redardation has become larger than before and that the day care service for such children at their homes will be more important.View full abstractDownload PDF (655K) -
Yumi Arai, Sawako Sumida, Makiko Osawa, Kyoko Hirasawa, Noriko Okada, ...1990 Volume 22 Issue 1 Pages 55-60
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe computed tomography (CT) and ultrasound (US) imaging studies were performed on skeletal muscles of two siblings (5-year-old boy and 10-year-old girl) with central core disease. The appearance of low-density areas in muscles was remarkable at the levels of the 3rd lumbar vertebra (L3), the midthigh and the thickest part of legs. The muscles at the levels of L3 and thigh were more severely affected than those of legs. Especially, paravertebral muscles, m. vastus, m. sartorius, m. gracilis tended to be more severely affected. The muscles of the legs except m. soleus were well preserved. US imaging of the thigh revealed a marked increase of echogenicity of rectus muscle as well as opaque, indistinct changes of fascia and bone. In contrast, the CT finding of the rectus muscle was relatively well preserved.View full abstractDownload PDF (7559K) -
Mihoko Kodama, Yumiko Kai, Shigeto Sugino, Narumi Inokuchi, Teruhisa M ...1990 Volume 22 Issue 1 Pages 61-65
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSTwo siblings with interstitial deletion of chromosome 14 not associated with ring formation were reported. Clinical features of the patients included failure to thrive, severe mental retardation, microcephaly, round face, hypertelorism, micrognathia and high-arched palate. They were common also in five previously reported cases.
Other peculiar finding was hyperthyrotropinemia in their neonatal periods ; mild hypothyroidism was found in the elder sister, and hyperthyrotropinemia was only transient in the younger sister. By a high-resolution G-banding analysis, the maternal origin of the chromosome with the deletion was noted.View full abstractDownload PDF (3332K) -
Yoh Miura, Shun Mizuta, Shinichi Yagi, Kaichirou Kobayashi, Naoki Kata ...1990 Volume 22 Issue 1 Pages 66-70
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThis report presented a 4-year-old girl who had atonic partial seizure of the right leg accompani ed byimpaired eqilibrium. This patient had a generalized tonic-clonic seizures before, and had been on anticonvulsant medication. Mild cataplexy of the right leg and flail trunk while standing occurred adruptly.
Based on clinical symptoms, physiological findings, and an electroencephalogram taken at the time of seizure, the cataplexy of the right leg was diagnosed as epileptic seizures. After the dosage of anticonvalsant drug was increased, all symptoms disappeared completely. Cases of atonic partial seizure have been reported only rarely. In our case, atonic partial seizure was associated with nonepileptic equilibrium impairment, probably due to cerebral cortex dysfunction. This is an extremely rare occurrence.View full abstractDownload PDF (612K) -
Yoshihiro Naganuma, Tohru Konishi, Kazuhisa Hongou, Miyako Murakami, M ...1990 Volume 22 Issue 1 Pages 71-76
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSCerebrovascular involvement is rare in progeria syndrome (Hutchinson-Gilford syndrome). A patient with progeria syndrome, who developed cerebral infarction, was reported. At 7 years of age, she suffered from right hemiplegia and transient ischemic attacks. X-ray CT showed multiple low density lesions in left frontal and parieto-occipital areas, which were enhanced with a contrast medium. Cerebral angiography demonstrated complete occlusion of left carotid artery and narrowing of vertebral artery.
Brain imaging using 123I-N-isopropyl-p-iodoamphetamine (1231-IMP SPECT), which expressed regional cerebral blood flow, showed extensive perfusion defect over the left cerebral hemisphere in early scans, and redistribution phenomena in late scans.
The symptoms improved gradually, which correlated well with 123I IMP-SPECT findings. This method will be useful to determine the prognosis as well as to understand changeable hemodynamic pathophysiology. A slowing of back ground on EEG also correlated well with clinical symptoms. It was necessary to examine the possibility of cerebrovascular involvement in progeria.View full abstractDownload PDF (8489K) -
Hideo Yamanouchi, Takayuki Yanagisawa, Michiko Takahashi, Shinzo Shimi ...1990 Volume 22 Issue 1 Pages 77-81
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSIn 12-month-old girl with Krabbe's disease, serial CT and MRI examinations showed characteristic findings, which were symmetric high densities in globus pallidus and thalami before and during the course of brain atrophy and decreased attenuation in white matter. The low densities in white matter were not so remarkable as other degenerative diseases affecting primarily white matter. The high densities in the globus pallidus changed into low densities with progression of the white matter hypodensities and brain atrophy. These findings on CT in Krabbe's disease may assist neurologists in narrowing the differential diagnosis and requesting appropriate laboratory tests.View full abstractDownload PDF (6143K) -
Yasuko Noda, Kaori Sakai, Megumu Tojo, Norio Sakuragawa, Masataka Arim ...1990 Volume 22 Issue 1 Pages 82-85
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA girl with severe infantile type of nemaline myopathy was reported, who is still alive at the age of 9 years and 3 months. Myopathy was so severe that antigravity movement has been seen only at fingers and toes since early infancy.
Skeletal muscles of extremeties and lumbogluteal regions were found to be replaced by fat on CT examination. Her daily activity has been improved by speech therapy and introduction of an electric wheel chair controlled with her chin. Cor pulmonale has gradually progressed by 8 years of age, although myopathic symptoms were apparently nonprogressive. Most patients of nemaline myopathy of this type were reported to have died of respiratory insufficiency by two years of age. Our present case was probably the oldest recorded in the literature, surviving up to the age of 9 years.View full abstractDownload PDF (3634K) -
[in Japanese]1990 Volume 22 Issue 1 Pages 86-87
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1990 Volume 22 Issue 1 Pages 88-90
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1990 Volume 22 Issue 1 Pages 90-92
Published: January 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS
- |<
- <
- 1
- >
- >|