Neuropadiatrie最新文献_第9页

An unusual case of phenytoin intoxication. 一例不寻常的苯妥英中毒病例。

Neuropadiatrie Pub Date : 1978-05-01 DOI: 10.1055/s-0028-1091478

M Albani

引用次数: 3

[Dichotic listening performance in children--development of a reliable test (author's transl)]. [儿童的二分性听力表现——开发一种可靠的测试[作者译]]。

Neuropadiatrie Pub Date : 1978-05-01 DOI: 10.1055/s-0028-1085415

I Roob, W Rost, A Rothenberger

引用次数: 0

Influences on the absorption of diphenylhydantoin preparations. 对二苯基苯妥英制剂吸收的影响。

Neuropadiatrie Pub Date : 1978-05-01 DOI: 10.1055/s-0028-1085416

A Windorfer, S Stünkel, H M Weimann

引用次数: 8

Leukocyte docosahexaenoic acid in juvenile form of ceroid-lipofuscinosis. 幼年型蜡样脂褐病中的白细胞二十二碳六烯酸。

Neuropadiatrie Pub Date : 1978-05-01 DOI: 10.1055/s-0028-1085417

R K Pullarkat, V K Patel, H Brockerhoff

引用次数: 19

Occipital 3-4/s-rhythms in childhood EEG. 儿童脑电图枕部3-4/s节律。

Neuropadiatrie Pub Date : 1978-05-01 DOI: 10.1055/s-0028-1085419

H Doose, H Gerken, G Koenig, E Völzke

{"title":"Occipital 3-4/s-rhythms in childhood EEG.","authors":"H Doose, H Gerken, G Koenig, E Völzke","doi":"10.1055/s-0028-1085419","DOIUrl":"https://doi.org/10.1055/s-0028-1085419","url":null,"abstract":"Report of follow-up studies in 65 children with occipital slow rhythms in the EEG (287 records). Two types of occipital rhythms can be differentiated: Paroxysmal bursts of 3--4/s-rhythms with high amplitude (I) accentuated by closing eyes and continuous usually regular occipital 3--4/s-rhythms (II). Type I is identical with the sinusoidal occipital 3/s-rhythms, which are observed especially frequently in childhood epilepsies. It could be demonstrated by follow-up studies, that the phenomenon usually disappears at latest during puberty. --The second type may also disappear during puberty or may even persist until the adult age. This persisting type is identical with what is quoted as \"Grundrhythmusvariante\" in the German literature. The continuous occipital rhythms could be demonstrated at earliest at age 3. The rhythms change their shape, amplitude, frequency and localisation during growth: In children prior to age 10 the rhythms show more often occipital accentuation in monopolar leads (against ear as reference); the amplitudes are higher on average; the frequency is often less than 4/s, and alpha groups preceding the rhythms after closing the eyes are less pronounced than in older children. Subharmonic waves are frequent. In the same manner as in adults a mostly right sided lateralisation can be seen. Genetic factors may be involved in the development of the occipital rhythms, although a simple mendelian transmission could not be confirmed. The EEG phenomenon is correlated with symptoms of psychic and vegetative lability. There is no correlation to epilepsy. The high incidence of anamnestic risk factors suggests that exogenous factors are at least contributing to the development of the rhythms. The continuous occipital 3--4/s-rhythms must be understood as the symptom of a disturbed development of central pacemaker systems. It can lead to persisting abnormalities or only occur transitorily in certain stages of the brain maturation.","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"9 2","pages":"140-56"},"PeriodicalIF":0.0,"publicationDate":"1978-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085419","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11783737","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 9

Positive ventriculography using water-soluble contrast media in infants with myelomeningocele and hydrocephalus. 小儿脊膜膨出和脑积水的水溶性造影剂脑室造影阳性。

Neuropadiatrie Pub Date : 1978-02-01 DOI: 10.1055/s-0028-1085409

K Kellermann, J A Bliesener, J M Kyambi

引用次数: 3

Juvenile type of generalized ceroid-lipofuscinosis (Spielmeyer-Sjögren syndrome) I. Clinical findings. 青少年型广泛性脂褐质病(Spielmeyer-Sjögren综合征)1 .临床表现。

Neuropadiatrie Pub Date : 1978-02-01 DOI: 10.1055/s-0028-1085407

G Schwendemann, K L Elze, P Koepp, I Lagenstein, H C Steinhausen, H J Colmant

引用次数: 1

Juvenile type of generalized ceroid-lipofuscinosis (Spielmeyer-Sjögren syndrome1 II. Biopsy findings. 少年型广泛性脂褐质病(Spielmeyer-Sjögren综合征)1。活组织检查结果。

Neuropadiatrie Pub Date : 1978-02-01 DOI: 10.1055/s-0028-1085408

G Schwendemann, H J Colmant, K L Elze, P Koepp, I Lagenstein, H C Steinhausen

{"title":"Juvenile type of generalized ceroid-lipofuscinosis (Spielmeyer-Sjögren syndrome1 II. Biopsy findings.","authors":"G Schwendemann, H J Colmant, K L Elze, P Koepp, I Lagenstein, H C Steinhausen","doi":"10.1055/s-0028-1085408","DOIUrl":"https://doi.org/10.1055/s-0028-1085408","url":null,"abstract":"Rectal biopsies were obtained from 11 children, clinically presumed to suffer from the juvenile type of generalized ceroid-lipofuscinosis. In addition, sural nerve biopsies were performed in 4 cases. Of the various staining procedures applied for light microscopy, Sundan Black B was found to be the most useful, revealing in each of the rectal biopsies at least some nerve cells with coarse black droplets as well as numerous mucosal macrophages with a more greyish, dustlike material. In sural nerve biopsies a slight reduction of the thick myelinated nerve fibers was seen in two cases, accompanied by regeneration phenomena. The latter were also detectable in one other case, the remaining sural nerve biopsy being histologically unremarkable. By electron microscopy neuronal perikarya revealed lipopigment bodies with a fingerprint-like pattern in each of the rectal biopsies. In one case only was this pattern combined with distinct curvilinear profiles. In 4 cases, however, the fingerprint-like deposits were intermingled with different lamellated bodies, some of them reminiscent of MCB's or zebra bodies. In two of the 4 cases, these were even seen exclusively in some neurons. In each of the rectal as well as the sural nerve biopsies, most of the non-neuronal cell types presented lipopigment bodies, likewise, displaying both curvilinear and fingerpring-like profiles, with some characteristics related to the various cell types. With regard to generalized ceroid-lipofuscinosis the diagnostic value of rectal biopsies, though being disputed in the literature, appears reestablished by the findings presented.","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"9 1","pages":"28-48"},"PeriodicalIF":0.0,"publicationDate":"1978-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085408","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11622248","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 2

Neurovisceral storage disorder simulating Niemann-Pick disease. A new form of oligosaccharidosis? 模拟尼曼-匹克病的神经内脏储存障碍。低聚糖病的新形式?

Neuropadiatrie Pub Date : 1978-02-01 DOI: 10.1055/s-0028-1085410

B Hagberg, M Haltia, P Sourander, L Svennerholm, M T Vanier, C G Ljunggren

{"title":"Neurovisceral storage disorder simulating Niemann-Pick disease. A new form of oligosaccharidosis?","authors":"B Hagberg, M Haltia, P Sourander, L Svennerholm, M T Vanier, C G Ljunggren","doi":"10.1055/s-0028-1085410","DOIUrl":"https://doi.org/10.1055/s-0028-1085410","url":null,"abstract":"Two sisters are described with demonstrable splenomegaly already from infancy and, after the age of 2--4 years, signs of slowly progressive encephalophy, vacuolated lymphocytes in the peripheral blood, and peculiar foam cells in the bone marrow aspirates. The died at 7 3/4 and 6 1/2 years. Widely spread in the brain, the nerve cell bodies were found to show extensive ballooning. It was most striking in the brain stem and spinal cord, while cerebellar structures were remarkably well preserved. The cytoplasm of the ballooned nerve cells was filled with finely granular storage material stainable as a readily soluble glycolipid. The spleen, liver and intestinal wall contained numerous foamy PAS-positive macrophages. Chemical assays showed a ten-fold increase of lactosylceramide and a modest one of minor gangliosides brain cortex. No accumulation sphingomyelin could be revealed, and the sphingomyelinase activity was found to be normal. The ganglioside GM1 beta-galactosidase activity of leucocytes was reduced to 20--25% of normal, which indicated a disturbance of the glycosaminoglycan metabolism. The tissue content of glycosaminoglycans was, however, normal, but an accumulation of lactose was demonstrated in the spleen. It is postulated that the primary enzymic defect is a disturbance of a lysosomal beta-galactosidase with a substrate specificity for lactose and other oligosaccharides with a terminal beta-galactosidic linkage.","PeriodicalId":76211,"journal":{"name":"Neuropadiatrie","volume":"9 1","pages":"59-73"},"PeriodicalIF":0.0,"publicationDate":"1978-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0028-1085410","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11783588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 20

[Acquired aphasia with electroencephalographic manifestation in children (author's transl)]. [儿童后天性失语伴脑电图表现(作者译)]。

Neuropadiatrie Pub Date : 1978-02-01 DOI: 10.1055/s-0028-1085412

U Petersen, P Koepp, M Solmsen, T Villiez

引用次数: 15