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Acta neuropathologica. Supplementum最新文献

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Ultrastructure of a cerebral white matter lesion in a 41-year-old man with Leigh's encephalomyelopathy (LEM). 41岁男性Leigh型脑脊髓病(LEM)脑白质病变的超微结构。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_69
A P Anzil, A Weindl, A Struppler
{"title":"Ultrastructure of a cerebral white matter lesion in a 41-year-old man with Leigh's encephalomyelopathy (LEM).","authors":"A P Anzil,&nbsp;A Weindl,&nbsp;A Struppler","doi":"10.1007/978-3-642-81553-9_69","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_69","url":null,"abstract":"<p><p>A brain biopsy taken from a 41-year-old man with Leigh's encephalomyelopathy (LEM) was studied under the electron microscope. The sample of cerebral white matter revealed degenerated oligodendrocytes, reactive astrocytes and scavenger cells filled with miscellaneous debris. Myelin underwent splitting and vacuole formation as well as lamellar and vesicular dissolution. Degenerative and reactive changes were prominent in the axons, many of which were calcified. There was marked fibrous gliosis of the tissue and the extracellular space was enlarged. The small vessels were notably involved; evidence of capillary growth was also found. In sum, the overall picture was one of loss of tissue continuity, loss of axons and loss of myelin, opposite to which stood expansion of the extracellular space, accumulation of filament-filled astroglial process, hypertrophy and hyperplasia of the capillaries and increase of the tissue cellularity. The study has confirmed and defined at the ultrastructural level the well-known histological features of LEM. However, the formal, let alone the causal pathogenesis of the disease remains as obscure as ever.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"233-8"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17987803","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
An investigation in vitro of the exocytosis of lysosomal enzymes from C6 glioma cells. C6胶质瘤细胞溶酶体酶体外胞吐的研究。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_26
D McCormick, I Wallace
{"title":"An investigation in vitro of the exocytosis of lysosomal enzymes from C6 glioma cells.","authors":"D McCormick,&nbsp;I Wallace","doi":"10.1007/978-3-642-81553-9_26","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_26","url":null,"abstract":"<p><p>The rates of exocytosis on the lysosomal hydrolase n-acetyl-beta D-glucosaminidase and beta-glucuronidase were measured in monolayer cultures of the C-6 glioma cell line. A comparison of the kinetics of release of the enzymes and the effects of cytochalasin B suggest that either intralysosomal localisation or the mechanism of release may be different for each enzyme. Evidence was obtained that exocytosis is accompanied by receptor-mediated endocytosis of hydrolases and the importance of the microtubular system in the maintenance of the exocytosis equilibrium was indicated by the effect of colchicine on the rate of extracellular accumulation of the enzymes.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"85-7"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17988501","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: 0
Dysmaturative myopathy. Evolution of the morphological picture in three cases. Dysmaturative肌病。三例形态图的演化。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_90
J R Ricoy, A Cabello
{"title":"Dysmaturative myopathy. Evolution of the morphological picture in three cases.","authors":"J R Ricoy,&nbsp;A Cabello","doi":"10.1007/978-3-642-81553-9_90","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_90","url":null,"abstract":"<p><p>In a previous paper we grouped together several myopathic pictures as alterations of muscle maturation and we proposed the term of dysmaturative myopathy for them. In this report we shall describe the follow up on three of the original cases, studied 4 1/2, 7 and 6 years after the first biopsy. These cases demonstrate that the muscular picture is not static and that it evolves towards normality. During this evolution, it changes from one to another of the morphological pictures previously described as myotubular and/or centronuclear myopathy, hypotrophy of type I fibers with or without central nuclei and congenital disproportion of types.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"313-6"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17989136","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
Prospective neuropathologic study on the occurrence of Wernicke's encephalopathy in patients with tumours of the lymphoid-hemopoietic systems. 淋巴-造血系统肿瘤患者发生韦尼克脑病的前瞻性神经病理学研究。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_101
J De Reuck, G Sieben, W De Coster, H Vander Eecken
{"title":"Prospective neuropathologic study on the occurrence of Wernicke's encephalopathy in patients with tumours of the lymphoid-hemopoietic systems.","authors":"J De Reuck,&nbsp;G Sieben,&nbsp;W De Coster,&nbsp;H Vander Eecken","doi":"10.1007/978-3-642-81553-9_101","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_101","url":null,"abstract":"<p><p>In this prospective neuropathologic study, Wernicke's encephalopathy appears to be the most frequent intracranial complication of treated patients with tumours of the lymphoid-hemopoietic systems. The highest incidence of this encephalopathy is found in elderly and in patients with a prolonged survival. The chronic form of Wernicke's encephalopathy is most commonly observed.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"356-8"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17990138","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: 10
Marchiafava-Bignami disease. Marchiafava-Bignami疾病。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_102
M Dilmac, H Klein
{"title":"Marchiafava-Bignami disease.","authors":"M Dilmac,&nbsp;H Klein","doi":"10.1007/978-3-642-81553-9_102","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_102","url":null,"abstract":"","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"359-61"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17990139","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: 4
Thalamic degeneration. 丘脑的退化。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_103
P Pilz, P Erhart
{"title":"Thalamic degeneration.","authors":"P Pilz,&nbsp;P Erhart","doi":"10.1007/978-3-642-81553-9_103","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_103","url":null,"abstract":"<p><p>A man of 61 died after an illness of 20 years duration. The clinical picture displayed progressive dementia, loss of memory, apathy, inertia, emotional lability, temporal and local disorientation, visual hallucination and apraxia. Neuropathological findings predominated in the thalamus and displayed extensive nerve cell loss and gliosis in the anterior, medial, and posterior formations, whereas the lateral formations were somewhat better preserved. The lateral amygdala were severely affected. The cortex showed widespread moderate damage in the third or fourth layer which are interpreted as transneuronal degeneration. In addition moderate atrophy of the vestibular nuclei, the inferior olives and the cerebellum was noted. This case seems to be one of the very rare examples of isolated thalamic degeneration according to Martin (1975).</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"362-4"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17990141","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
The activity of some hydrolytic enzymes in the brain after administration of "Cynkotox". 脑内某些水解酶的活性在服用“脑毒”后。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_17
M B Kozik
{"title":"The activity of some hydrolytic enzymes in the brain after administration of \"Cynkotox\".","authors":"M B Kozik","doi":"10.1007/978-3-642-81553-9_17","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_17","url":null,"abstract":"<p><p>The effect of peroral administration of \"Cynkotox\" (diethyl-dithio-carbamate, zinc salt) on the histoenzymatic activity of the brain was examined. 1.0 g of Cynkotox was given daily to adult Wistar rats over a period of 10 days (the per os DL50 for rats is 5200 mg/kg of body weight). The results have shown that chronic ingestion of large doses of Cynkotox may cause degenerative changes of neurons in various brain regions as well as alterations in the activity of various enzymes. The cerebral activities TPPase and of acP were evidently increased, whereas those of AChE, BuTJ and ATPase were markedly reduced. In some regions of the experimental rat brains. NsE activity was elevated in other ones some loss of NsE activity was noticed.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"56-8"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17178541","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
Basophilic inclusions in skeletal muscle from two cases of hypothyroid myopathy. 两例甲状腺功能减退肌病骨骼肌中的嗜碱性包涵体。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_92
M Coquet, J M Vallat, J L Latapie
{"title":"Basophilic inclusions in skeletal muscle from two cases of hypothyroid myopathy.","authors":"M Coquet,&nbsp;J M Vallat,&nbsp;J L Latapie","doi":"10.1007/978-3-642-81553-9_92","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_92","url":null,"abstract":"<p><p>We have been able to examine muscular tissue from two females showing hypothyroid myopathy. Under light microscopy, certain fibres contained basophilic inclusions. Histoenzymology showed that these inclusions were found exclusively in certain type 1 fibres. Ultrastructural examination showed that the inclusions were accumulations without a limiting membrane. These accumulations contained straight fibrils which were randomly distributed. Their mean diameter was 70 A. The neighbouring myofibrils were usually unaltered. We have found similar images described for five cases of hypothyroid myopathies. They are more common in myocardium than in skeletal muscle. These images are similar to those observed in some case of type IV glycogenosis and Lafora's disease.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"320-2"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/978-3-642-81553-9_92","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17323458","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: 4
Systemic carnitine deficiency with peripheral nerve involvement morphological and biochemical study. 周围神经受累的系统性肉碱缺乏的形态学和生化研究。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_67
F Cornelio, D Peluchetti, M Rimoldi, D Testa, M Mora, S Negri, S DiDonato
{"title":"Systemic carnitine deficiency with peripheral nerve involvement morphological and biochemical study.","authors":"F Cornelio,&nbsp;D Peluchetti,&nbsp;M Rimoldi,&nbsp;D Testa,&nbsp;M Mora,&nbsp;S Negri,&nbsp;S DiDonato","doi":"10.1007/978-3-642-81553-9_67","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_67","url":null,"abstract":"<p><p>The patient, a 31-year-old woman, suffered from a weakness of the proximal muscles which subsequently worsened involving the spinal and neck muscles. During the hospitalization, the patient displayed a \"burning feet\" syndrome. Lab tests showed a moderate increase in CPK, Aldolase, SGPT, SGOT, lactic and pyruvic acids. Needle EMG was interpreted as \"myopathic\" but a marked reduction of sensitive action potentials was also detected. Muscle biopsy showed neutral lipid accumulation in muscle fibers, many small angular fibers, probably denervated ones were also evident. A liver biopsy showed fatty accumulation in hepatocytes. Free carnitine was extremely reduced in muscle and liver and just below normal level in plasma while there was a tenfold elevation of long-chain carnitine esters in plasma too. The mechanism by which carnitine deficiency can cause both neural and muscular alterations and in particular the role of carnitine in glucose metabolism is discussed.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"226-9"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/978-3-642-81553-9_67","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17323456","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: 4
Myopathological findings in progressive myoclonus epilepsy. 进行性肌阵挛性癫痫的病理表现。
Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI: 10.1007/978-3-642-81553-9_96
S Pierobon-Bormioli, C Angelini, M Armani, G F Testa
{"title":"Myopathological findings in progressive myoclonus epilepsy.","authors":"S Pierobon-Bormioli,&nbsp;C Angelini,&nbsp;M Armani,&nbsp;G F Testa","doi":"10.1007/978-3-642-81553-9_96","DOIUrl":"https://doi.org/10.1007/978-3-642-81553-9_96","url":null,"abstract":"<p><p>8 patients with progressive myoclonic epilepsy have been studied. Muscle biopsies showed polysaccharide accumulation in branched form, mitochondrial alteration, lipid droplets and lipofucsin bodies. \" Muscle Lafora bodies\" were present in two cases. A peculiar muscle cytoskeleton appearance has been seen, suggesting an abnormal physico-chemical state of the newly observed filaments that joins Z-bands among them and with the plasma membrane.</p>","PeriodicalId":75397,"journal":{"name":"Acta neuropathologica. Supplementum","volume":"7 ","pages":"334-7"},"PeriodicalIF":0.0,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17323459","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: 7
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