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淀粉样变相关性神经病

Amyloid-related neuropathies.

作者信息

Goebel H H, Bohl J, Störkel S

机构信息

Division of Neuropathology, University of Mainz, FRG.

出版信息

Zentralbl Allg Pathol. 1990;136(6):517-23.

PMID:2177943
Abstract

Formation of amyloid within peripheral nerves, resulting in amyloid-related neuropathies, may occur when myeloma-associated amyloid (AL) is deposited in an immune-related neuropathy or in familial amyloid polyneuropathy where prealbumin/transthyretin variants are marked by AF amyloid deposition. Refined histochemical and recent immunohistochemical techniques identify the correct type of amyloid and thus the nosologically precise form of amyloid-related neuropathy. Neuropathy is an inherent thought not obligate clinical and morphological component in two of the three systemic amyloidoses. Multiple causative factors in adult forms of neuropathy render a search for amyloid mandatory whenever respective biopsied nerve specimens are examined.

摘要

当骨髓瘤相关淀粉样蛋白(AL)沉积于免疫相关性神经病变或家族性淀粉样多神经病(其中前白蛋白/转甲状腺素蛋白变体以AF淀粉样蛋白沉积为特征)时,可在周围神经内形成淀粉样蛋白,导致淀粉样蛋白相关神经病。精细的组织化学和最新的免疫组织化学技术可识别淀粉样蛋白的正确类型,从而确定淀粉样蛋白相关神经病在分类学上的确切形式。神经病是三种全身性淀粉样变中两种类型的内在而非必然的临床和形态学组成部分。成人形式的神经病有多种致病因素,因此在检查相应的活检神经标本时,寻找淀粉样蛋白是必不可少的。

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