J F Pellissier, T de Barsy, J Bille, G Serratrice, M Toga
{"title":"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。","authors":"J F Pellissier, T de Barsy, J Bille, G Serratrice, M Toga","doi":"10.1007/978-3-642-81553-9_84","DOIUrl":null,"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":0.0000,"publicationDate":"1981-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"24","resultStr":"{\"title\":\"Polysaccharide (amylopectin-like) storage myopathy histochemical ultrastructural and biochemical studies.\",\"authors\":\"J F Pellissier, T de Barsy, J Bille, G Serratrice, M Toga\",\"doi\":\"10.1007/978-3-642-81553-9_84\",\"DOIUrl\":null,\"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\":0.0000,\"publicationDate\":\"1981-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"24\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Acta neuropathologica. Supplementum\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1007/978-3-642-81553-9_84\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Acta neuropathologica. Supplementum","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1007/978-3-642-81553-9_84","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Polysaccharide (amylopectin-like) storage myopathy histochemical ultrastructural and biochemical studies.
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.
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