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Y Yamatogi

Publications and source records attributed to Y Yamatogi.

32 records · Page 2Linked to original sources
PubMed1984

Chronic granulomatous disease of childhood and sea-blue histiocytosis. A pathologic study of an autopsy case.

A boy, who started remittent fever reaching 40 degrees C at the age of 4 years and 9 months and gradually developed hepatosplenomegaly, lymphadenopathy, pancytopenia, and hypergammaglobulinemia, is reported. Aspirated bone marrows contained sea-blue histiocytes, and nitroblue tetrazolium test was abnormal. X-ray examination revealed a tumor mass in the right lung, uremia occurred terminally, and he died at the age of 10 years and 3 months. Surgical specimens, obtained by splenectomy, and necropsy revealed the presence of characteristic sea-blue cells in the bone marrow, spleen, lymph nodes, liver, and kidneys, and pseudotuberculous granulomas with or without aspergillus in the lungs, epicardium, liver, and lymph nodes. Histochemically, sea-blue granules accumulating in the cytoplasm of the histiocytes were proved to be a lipogenic ceroid-like pigment. Electron microscopically, these granules were comprised of round or ovoid, membrane-bound or free lipid droplets of various sizes and electron densities, occasionally containing poorly oriented, lamellar structures. The nature and possible pathogenesis of the sea-blue histiocytosis in this case are discussed briefly.

Autopsy↗
PubMed1983

Psychotic symptoms in childhood epilepsy--an electroencephalographic study.

To investigate the significance of EEG findings relating to the appearance of psychic symptoms in epileptic children, a clinicoelectroencephalographic study was undertaken on 15 cases with psychotic episodes. 1) Psychotic episodes with hallucination and/or illusion were observed, though rarely, in childhood epilepsy. These seemed liable to occur in temporal lobe epilepsy. 2) Three of four patients in the hallucination and illusion group showed frequent epileptic discharges. However, no distinct relation existed between seizure discharges and psychotic episodes. 3) Eight of 11 patients in the dysphoria and excitement group were secondary generalized epilepsy. 4) In the dysphoria and excitement group, psychotic episodes occurred either as a result of the increase in epileptic discharges or conversely as a result of the suppression of epileptic discharges. The former was more frequent. Epileptic discharges tended to be suppressed in the Lennox syndrome and allied conditions relating to psychotic episodes. 5) A forced normalization-like phenomenon mostly resulted from the marked suppression of diffuse slow spike-waves. However, a complete suppression of epileptic discharges was not always noted.

Adolescent↗
PubMed1981

Age-dependent epileptic encephalopathy: a longitudinal study.

A longitudinal clinico-electroencephalographic study was conducted on 484 cases of age-dependent epileptic encephalopathy; early-infantile epileptic encephalopathy with suppression-burst (Ohtahara) (EIEE), the West and Lennox syndromes. 1) The three types had the same kind of etiologic heterogeneity. 2) An evolutional change with age was noted from EIEE to the West and from the West to the Lennox syndromes, with the change from suppression-burst to hypsarhythmia, and from hypsarhythmia to the diffuse slow spike-waves. 3) The core ictal EEG pattern of tonic spasms was the desynchronization in EIEE and the West syndrome, with the rapid synchronization and recruiting rhythm in the Lennox syndrome. In the same cases, the changes from desynchronization to hypersynchronization were observed with age. These facts suggest that the three syndromes are based on the same pathophysiology, strongly related to the subcortical mechanism, and that their clinico-electrical manifestations are modified by the degree of brain maturation.

Brain↗
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

[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↗
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↗
PubMedDate not supplied

Prenatal etiologies of West syndrome.

We investigated the etiology of West syndrome (WS) with special reference to prenatal factors in 180 cases. Prenatal cause was the most frequent diagnosis (77 cases, 42.8%), followed by perinatal (25 cases, 13.9%) and postnatal factors (12 cases, 6.7%); 48 cases (26.7%) were of uncertain etiology; eighteen cases (10.0%) were idiopathic. Of the three forms of age-dependent epileptic encephalopathy, prenatal cause was present in 12 of 15 cases (80.0%) of early-infantile epileptic encephalopathy with suppression-burst, 77 of 180 cases (42.8%) of WS, and 31 of 123 cases (25.2%) of Lennox-Gastaut syndrome (LGS). Prenatal factors of WS included tuberous sclerosis (23), chromosome abnormalities (10), cerebral dysgenesis (10), porencephaly (7), hydrocephalus (5), Aicardi syndrome (3), Aicardi syndrome associated with chromosome abnormality (1), and other causes (18). Chromosome abnormalities with WS consisted of 6 cases with 21 trisomy and one case each with 18q duplication, t(1;y) translocation, 7q duplication, and partial 2p trisomy. One patient with Aicardi syndrome also had a t(12;21) translocation. No significant difference was observed in the age of onset of WS among the five etiologic groups. The evolution from WS to LGS was not influenced by etiology, except for the idiopathic group. In patients followed for over 3 years, seizure remission occurred in 46.8% (22 of 47 cases) of the prenatal group. This was lower than the other four groups. Intellectual prognosis was also relatively poor in those with prenatal onset. Pyridoxal phosphate (PAL-P) treatment was effective in 9 of 70 (12.9%) prenatal cases and 5 of 18 (27.8%) idiopathic cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗