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S Ohtahara

Publications and source records attributed to S Ohtahara.

8 recordsLinked to original sources
PubMed1979

Treatment of the Lennox syndrome with ACTH: a clinical and electroencephalographic study.

In 45 cases of Lennox syndrome treated with ACTH, the immediate and long-term effects and the various factors affecting them were investigated by a follow-up study. 1) Regarding the immediate effect, 23 (51.1%) of the 45 cases became "seizure free" for over 10 days. 2) As to the long-term prognosis of these 23 cases, 10 cases relapsed into Lennox syndrome within 6 months and in the remaining 13 cases, seizures were suppressed for over 6 months; out of these 13, seizure relapse was observed in eight cases from 9 months to 7 years later, and the other five cases followed a very favorable course without relapse. 3) The favorable factors related to the effect of ACTH for Lennox syndrome are: a) age at ACTH treatment: up to 4 years old, b) time lag between onset of Lennox syndrome and initiation of ACTH treatment: the shorter the better; at least within 1 year, preferably within 3 months, c) presumptive causes and underlying diseases: idiopathic cases are best, d) mental defects before treatment: the slighter the better, e) seizure patterns: without tonic seizures, and f) EEG findings: disorganized diffuse slow spike-waves without asymmetry. 4) It is desirable to continue the ACTH treatment as long as possible, with the goal of the disappearance of seizure discharges, or at least the disappearance of diffuse seizure discharges.

Adrenocorticotropic Hormone↗
PubMed1978

Malignant pituitary chromophobe adenoma in an infant accompanying diabetes insipidus.

This report deals with a 1.5-year-old male infant terminating in viral pneumonia and "diabetes insipidus". The autopsy revealed malignant pituitary chromophobe adenoma, invading the wall of the third ventricle, extending to the periventricular-, dorsomedial- and ventromedial area, skipping to the lateral area adjacent to the optic nerve, and permeating into the subarachnoid space of the cerebrum. At the onset of disease polydypsia and polyuria were marked followed by interstitial pneumonia with high fever, and later generalized tonic convulsion terminated in death. This case is peculiar in at least two respects. Firstly, this is the youngest reported case of malignant chromophobe adenoma. Secondly, the manifestation of diabetes insipidus is rare in chromophobe adenoma. The malignant adenoma probably occurred from the primitive gland rest of chromophobe cells in the posterior lobe becoming malignant during the course of growth development and destroyed the cells of the posterior lobe, resulting in diabetes insipidus.

Adenoma, Chromophobe↗
PubMed1978

Farber's disease (disseminated lipogranulomatosis): the first case reported in Japan.

We report the first case in Japan, i.e., the first case among oriental subject of Farber's disease. This is a rare disorder of lipid metabolism in infancy subsequent to a genetically-determined defect in ceramide degradation. Main features are characterized clinically by hoarseness, joint swelling, subcutaneous nodules and retarded psychomotor development. Lipid analysis and pathological investigation on the material obtained from a subcutaneous nodule confirmed clearly the presence of ceramide and intracytoplasmic inclusion bodies characteristic for Farber's disease. In this case, we experienced also corneal opacity and striking abnormalities in electroencephalogram, which have apparently not been noticed in the 17 cases hitherto reported.

Child, Preschool↗
PubMed1978

[Diabetes insipidus in children--long-term follow-up study].

A follow-up study was carried out on 12 children with vasopressin sensitive diabetes insipidus. 1) Nine cases (75%) of 12 were finally diagnosed as having brain tumor in later course. There were 3 cases (25%) who could not be decided as having brain tumor during the follow-up period of more than 6 years. 2) There was one case who developed the overt signs of brain tumor 9 years after the onset of diabetes insipidus. Therefore, it seems necessary to follow-up cases with diabetes insipidus for at least 10 years before determining it as idiopathic type. 3) In cases with diabetes insipidus due to brain tumor, associated growth retardation, autonomic symptoms, behavior disorder, endocrine dysfunction and metabolic dysfunction were frequently observed. In the case where these symptoms become aggravated with lapse of time, these findings should be taken seriously as indicating brain tumor. 4) In the case showing either anterior or posterior focal slow waves in the EEG, if such focal slow waves aggravate with age, the findings should be considered as indicative of brain tumor. 5) We would like to emphasize the significance of brain tumor as the underlying pathology of childhood diabetes insipidus.

Astrocytoma↗
PubMed1977

[Apert's syndrome].

Explore the source record for details and available documents.

Acrocephalosyndactylia↗
PubMed1976

Prognosis of the Lennox syndrome-long-term clinical and electroencephalographic follow-up study, especially with special reference to relationship with the West syndrome.

The long-term clinical and electroencephalographic follow-up studies were carried out for more than three years, up to 14 years in the longest, on 116 cases with Lennox syndrome. And the results of s-ystematic study on changing patterns and out come have been reported. The follow-up examination was performed also on West syndrome which is closely related with Lennox syndrome; especially the relationship between both syndromes regarding prognosis has been clarified. (1) According to the long-term follow-up on 116 cases with Lennox syndrome, there were 98 cases (84.5%) having mes (61.2%) and persisted as Lennox syndrome except for one case. (2) Generally speaking, the cases with age of onset before two years old showed unfavorable outcome. (3) There were 42 cases (36.2%), which were converted from West syndrome and showed markedly unfaborable prognosis in regard to intelligence as well as the remainging of seizure. (31 cases, 77.5%) (4) On thehand, in 23 idiopathic cases, which showed no developmental retardation before onset of seizure, had favorable outcome, and the remaining of seizure was observed in eight cases (34.8%). However, even in such cases, it was noticed that those displaying mental defect at the follow-up attained 14 cases (60.9%). That is to say, it was clarified that persistence of even minor seizures induced mental deterioration. (5) The cases with favorable prognosis showed usually a typical slow spike-and-wave pattern electroencephalographically, whereas those with poor prognosis showed mostly an asymmetric or disorganized slow spike-and-wave pattern. (6) In many cases displaying signs of brain atrophy with pneumoencephalograpm and accompanying overt neurological signs at the initial examination, prognosis is obviously poor. (7) From the follow-up examination on 94 cases with West syndrome for three to 15 years transformed into Lennox syndrome Among them, those cases with the remaining of seizure at the time of follow-up were 44 (46.8%) out 94 cases, of which 37 cases (83.8%) had remaining seizure as Lennox syndrome. (8) From the above results, it is emphasized that Lennox and West syndromes show close relationship with each other and that a study should be done on the interrelation between their prognosis.

Adolescent↗