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Acta neuropathologica. Supplementum最新文献

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Neuropathology of primary generalized epilepsies with awakening grand mal. 原发性全身性癫痫伴觉醒大发作的神经病理学。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_108
H J Meencke, D Janz, J Cervos-Navarro
{"title":"Neuropathology of primary generalized epilepsies with awakening grand mal.","authors":"H J Meencke, D Janz, J Cervos-Navarro","doi":"10.1007/978-3-642-81553-9_108","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_108","url":null,"abstract":"","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"378-80"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17836590","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: 11
Morphological diagnosis of Niemann-Pick disease type C by skin and conjunctival biopsies. 皮肤及结膜活检对C型尼曼-匹克病的形态学诊断。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_61
M L Arsénio-Nunes, F Goutières
{"title":"Morphological diagnosis of Niemann-Pick disease type C by skin and conjunctival biopsies.","authors":"M L Arsénio-Nunes,&nbsp;F Goutières","doi":"10.1007/978-3-642-81553-9_61","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_61","url":null,"abstract":"<p><p>Skin and conjunctival biopsies were performed in six children with mental deterioration, incoordination, involuntary movements and discrete visceromegaly. Two main types of inclusions were observed: 1) intracytoplasmic bodies containing membranous profiles loosely disposed in concentric or parallel arrays, lying in clear cells of eccrine sweat glands, hair follicles and cells of the basal layer of skin and 2) pleomorphous membrane bound bodies with a multivesicular aspect which are formed by vacuoles, stacks of lamellae and amorphous electron dense material. Subsequent biochemical studies showing a slight increase of sphingomyelin in liver and normal sphingomyelinase in leukocytes supported the morphological diagnosis of Niemann-Pick disease type C. The qualitative regional diversity of storage is stressed and the interest of both skin and conjunctival biopsies in the diagnosis of this affection is discussed.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"204-7"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17323455","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: 5
Melanosis cerebelli. 脑黑变病。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_114
C Bianchi, G Grandi, N Berti, L Di Bonito
{"title":"Melanosis cerebelli.","authors":"C Bianchi,&nbsp;G Grandi,&nbsp;N Berti,&nbsp;L Di Bonito","doi":"10.1007/978-3-642-81553-9_114","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_114","url":null,"abstract":"<p><p>Melanosis cerebelli (melanic pigmentation involving the dentate nuclei and the granular layer of the cerebellar cortex) is described in three patients, aged between 72 and 99 years. In two cases atrophic changes of the Alzheimer type co-existed in the cerebral cortex. One patient had a history of tremors, possibly cerebellar in origin.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"400-2"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17986100","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: 1
Ultrastructural study of peripheral nerve in Guillain-Barré syndrome presence of mononuclear cells in axons. 轴突单核细胞存在的格林-巴-罗综合征周围神经超微结构研究。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_73
C Brechenmacher, C Vital, L Laurentjoye, Y Castaing
{"title":"Ultrastructural study of peripheral nerve in Guillain-Barré syndrome presence of mononuclear cells in axons.","authors":"C Brechenmacher,&nbsp;C Vital,&nbsp;L Laurentjoye,&nbsp;Y Castaing","doi":"10.1007/978-3-642-81553-9_73","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_73","url":null,"abstract":"<p><p>As has been shown during the course of experimental allergic neuritis, in the Guillain-Barré syndrome there is an invasion of the myelin sheath in certain fibres by a mononuclear cell, with an intact axon. In a series of 45 cases we have been able to show this feature in 31 cases. In certain cases the cytoplasm prolongation of the invading cell was seen in the myelin sheath, forcing apart two neighbouring layers. In five cases we have furthermore observed the presence of mononuclear cells located either between the myelin and the axon which is deformed, or on the inside of the axon. Such an anomaly was frequent in one recent case and was occasionally found in four other cases.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"249-51"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17987806","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: 12
Polysaccharide (amylopectin-like) storage myopathy histochemical ultrastructural and biochemical studies. 多糖(支链淀粉样)储存肌病的组织化学、超微结构和生化研究。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_84
J F Pellissier, T de Barsy, J Bille, G Serratrice, M Toga
{"title":"Polysaccharide (amylopectin-like) storage myopathy histochemical ultrastructural and biochemical studies.","authors":"J F Pellissier,&nbsp;T de Barsy,&nbsp;J Bille,&nbsp;G Serratrice,&nbsp;M Toga","doi":"10.1007/978-3-642-81553-9_84","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_84","url":null,"abstract":"<p><p>A case of an adult polysaccharide myopathy is reported in a patient with progressive muscular atrophy and weakness of limb girdles. Histochemistry and electron microscopy showed in some muscle fibers, a storage material composed of amylopectin-like filaments. Biochemical results were normal and no enzyme deficiency was found. This case is compared with three other published cases. Pathological conditions with amylopectin or amylopectin-like storage material are reviewed.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"292-6"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17987815","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: 24
Glycogen storage disease in skeletal muscle. Morphological, ultrastructural and biochemical aspects in 10 cases. 骨骼肌中的糖原储存病。10例的形态学、超微结构及生化指标。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_85
A Cabello, T Benlloch, O Franch, J F Feliú, J R Ricoy
{"title":"Glycogen storage disease in skeletal muscle. Morphological, ultrastructural and biochemical aspects in 10 cases.","authors":"A Cabello,&nbsp;T Benlloch,&nbsp;O Franch,&nbsp;J F Feliú,&nbsp;J R Ricoy","doi":"10.1007/978-3-642-81553-9_85","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_85","url":null,"abstract":"<p><p>We analyzed clinical, histological and biochemical findings in 10 patients with glycogen storage disease in skeletal muscle. Four patients were deficient in acid-alpha-glucosidase (Glycogenosis type II), three of them with late infantile onset and one patient adult form. Five patients, two of them siblings, were deficient in myophosphorylase (glycogenosis type V, McArdle's disease). One patient was a newborn with phosphofructokinase deficiency (glycogenosis type VII, Tarui's disease). Of the study of our cases we would like to outline the following features: in the glycogenosis type II the deposit is fundamentally intralysosomal in the late infantile form, storage of mucopolysaccharides and deposit in interstitial fibroblasts were found, while in the adult form glycogen storage is minimal. In the glycogenosis type V the storage of glycogen is free and of a small amount. In two patients we have observed enzymatic activity in regenerating fibres. In glycogenosis type VII the storage is free, of considerable quantity and the interstitial cells are also affected; no storage is observed in the satellite cells.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"297-300"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17989131","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: 14
Pallido-luyso-nigral atrophy and amyotrophic lateral sclerosis. 苍白球-丘脑-黑质萎缩和肌萎缩性侧索硬化。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_99
F Gray, C De Baecque, M Serdaru, R Escourolle
{"title":"Pallido-luyso-nigral atrophy and amyotrophic lateral sclerosis.","authors":"F Gray,&nbsp;C De Baecque,&nbsp;M Serdaru,&nbsp;R Escourolle","doi":"10.1007/978-3-642-81553-9_99","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_99","url":null,"abstract":"<p><p>Clinical and neuropathological studies of a case of pallido-luyso-nigral atrophy and amyotrophic lateral sclerosis (ALS) in a young woman with a strong likelihood of a similar familial past medical history have been presented. Microscopic examination revealed neuronal loss and gliosis of globus pallidus, corpus luysii and substantia nigra. Pallor of the pyramidal tracts and neuronal loss in hypoglossal nuclei and anterior horns with gliosis were present. The rarity of the association of a pallido-luyso-nigral atrophy and an ALS, the occurrence of an ALS at such a young age and the fact that her grandmother died of Parkinson disease at age 30 suggest that this association may represent more than a coincidental occurrence.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"348-51"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17989142","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: 27
Histochemical and ultrastructural study of Gaucher cells. 戈谢氏细胞的组织化学和超微结构研究。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_62
M Elleder, A Jirásek
{"title":"Histochemical and ultrastructural study of Gaucher cells.","authors":"M Elleder,&nbsp;A Jirásek","doi":"10.1007/978-3-642-81553-9_62","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_62","url":null,"abstract":"<p><p>Findings are presented of the histochemical and electron microscopical studies of several bioptic specimens of a juvenile form of Gaucher's disease. The storage was restricted to the macrophages with the exception of occasional hepatocytes in which structures closely resembling the storage tubules were found. The cytoplasmic striations of GC were found to be composed both of lipid and a glycoprotein components which can be easily dissociated from each other by means of either organic solvent extraction or proteolytic digestion. According to the results obtained it is the glycoprotein component which is mainly responsible for the cytoplasmic striations. On the contrary the majority of the tubular storage material of the Krabbe cells was found to be removed with chloroform methanol. Results of the enzyme histochemical study (hydrolases and dehydrogenases) point to the considerably higher overall metabolic activity of GC when compared to the Niemann-Pick storage macrophages.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"208-10"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17989346","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: 18
Isolated leptomeninges of the spinal cord: an ideal tool to study inflammatory reaction in EAE. 脊髓分离的轻脑膜:研究EAE炎症反应的理想工具。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_54
K Kitz, H Lassmann, H M Wisniewski
{"title":"Isolated leptomeninges of the spinal cord: an ideal tool to study inflammatory reaction in EAE.","authors":"K Kitz,&nbsp;H Lassmann,&nbsp;H M Wisniewski","doi":"10.1007/978-3-642-81553-9_54","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_54","url":null,"abstract":"<p><p>Isolated and stretched leptomeninges have several advantages for the study of basic mechanisms in inflammation of the CNS. They offer a three dimensional view of the vascular tree which has basically the functional characteristics of cerebral vessels (the tight barrier, excluding macromolecules to enter the CNS compartment). The limited, small thickness of the specimen facilitates the performance of histochemical and immunohistochemical methods.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"179-81"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17989519","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: 13
Novel tubular inclusions in the bone marrow in multiple sclerosis: an ultrastructural study of early autopsy material. 多发性硬化症骨髓中新型管状包裹体:早期尸检材料的超微结构研究。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_56
J Kirk, E Dermott
{"title":"Novel tubular inclusions in the bone marrow in multiple sclerosis: an ultrastructural study of early autopsy material.","authors":"J Kirk,&nbsp;E Dermott","doi":"10.1007/978-3-642-81553-9_56","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_56","url":null,"abstract":"<p><p>Samples of bone marrow taken at early autopsy from patients who died with multiple sclerosis and from control cases, were examined ultrastructurally with the aim of detecting any infectious agents which might be present. No recognizable virus or mycoplasma was detected. However, rare bizarre cellular inclusions were found in 2 cases. The inclusions which are unlike anything previously described in MS consisted of fine (ca 17 nm) sinuous tubules occasionally showing dilated discoid ends. They occurred together with fragmentary electron opaque material in large membrane bound vacuoles in unidentified cells. Despite superficial resemblance to some viral nucleocapsids it is considered more likely that they have been formed as a result of degenerative phagocytic or autolytic activity. The specificity or otherwise of these inclusions to MS remains to be demonstrated.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"185-8"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17989521","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: 3
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