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原发性淀粉样变表现为上肢多发性单神经病。

Primary amyloidosis presenting as upper limb multiple mononeuropathies.

机构信息

Peripheral Neuropathy Research Laboratory, Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA.

出版信息

Muscle Nerve. 2010 May;41(5):710-5. doi: 10.1002/mus.21561.

Abstract

Peripheral neuropathy in primary (AL) amyloidosis is usually lower-limb predominant, length-dependent, symmetrical, and affects small (pain and autonomic) fibers, as much or more than large fibers. We report a patient with stepwise progressive, multiple upper limb mononeuropathies that were due to nerve biopsy-proven primary amyloidosis (lambda light chain), with no systemic or autonomic features. Recognition that light chain amyloidosis may be the cause of a multiple mononeuropathy pattern adds to the differential diagnosis of this clinical phenotype.

摘要

原发性(AL)淀粉样变的周围神经病通常以下肢为主,呈长度依赖性、对称性,影响小(疼痛和自主)纤维,与大纤维一样或更严重。我们报告了一例患者,其进行性多发性上肢单神经病,经神经活检证实为原发性淀粉样变(lambda 轻链),无系统性或自主神经特征。认识到轻链淀粉样变性可能是多发性单神经病的病因,这增加了这种临床表型的鉴别诊断。

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