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每个神经病理学家都需要知道的事:周围神经活检。

What Every Neuropathologist Needs to Know: Peripheral Nerve Biopsy.

机构信息

From the Department of Pathology, UT Southwestern Medical Center, Dallas, Texas.

Department of Pathology, University of Chicago, Chicago, Illinois.

出版信息

J Neuropathol Exp Neurol. 2020 Apr 1;79(4):355-364. doi: 10.1093/jnen/nlaa012.

Abstract

Peripheral neuropathy is a common disorder with many possible etiologies including metabolic diseases, inflammatory conditions, infections, malignancy, inherited diseases, drugs, and toxins. In most instances, diagnosis and treatment plan can be established based on clinical presentation, family history, laboratory results, genetic testing, and electrophysiological studies. But in some situations, a peripheral nerve biopsy remains a valuable tool. This is especially true in patients with rapidly progressive disease, with atypical presentation or for whom other approaches fail to yield a definitive diagnosis. The pathologic examination starts with basic decisions about specimen triage. A few basic questions help to provide an initial framework for the assessment of a nerve biopsy-is the specimen adequate; are there inflammatory changes; are there vascular changes; is there amyloid; are there changes to axonal density and the Schwann cell-myelin-axon unit. In the appropriate context and with such an approach peripheral nerve biopsies can still represent a clinically helpful test.

摘要

周围神经病是一种常见疾病,其病因很多,包括代谢性疾病、炎症性疾病、感染、恶性肿瘤、遗传性疾病、药物和毒素等。在大多数情况下,可以根据临床表现、家族史、实验室结果、基因检测和电生理研究来确定诊断和治疗方案。但在某些情况下,周围神经活检仍然是一种有价值的工具。对于快速进展性疾病、表现不典型或其他方法无法明确诊断的患者,尤其如此。病理检查首先要对标本进行分类,这是一个基本的决策。一些基本的问题有助于为神经活检评估提供初步框架:标本是否足够;是否有炎症改变;是否有血管改变;是否有淀粉样变性;轴突密度和施万细胞-髓鞘-轴突单位是否有改变。在适当的情况下,采用这种方法,周围神经活检仍然可以作为一种有临床帮助的检查。

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