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周围神经病变的电诊断评估

Electrodiagnostic Evaluation of Peripheral Neuropathy

作者信息

Novello Briana J., Pobre Thomas

机构信息

Nassau University Medical Center

PMID:33085316
Abstract

Peripheral neuropathy, also known as peripheral polyneuropathy, is a general term for a broad range of disorders that cause damage and dysfunction of the nerves of the peripheral nervous system in several different patterns. Electrodiagnostic (EDX) testing can not only identify whether or not peripheral neuropathy may be present. Still, it can also help give the clinician information to determine the disorder's etiology, severity, and prognosis. Thorough history taking, physical examination, and EDX are all integral to the evaluation and treatment of patients presenting with symptoms of peripheral neuropathy. Although peripheral polyneuropathies are commonly found in patients with diabetes mellitus or excessive alcohol use, many medical conditions have associations with peripheral neuropathies. Peripheral polyneuropathies can be acquired, such as in diabetes mellitus, amyloidosis, HIV, or cis-platinum chemotherapy, while some may be inherited, as in Charcot-Marie-Tooth.  Common symptoms of most peripheral polyneuropathies involve paresthesias, numbness, or pain in the distal extremities, such as in the feet or hands. Some can present acutely, while many have a more insidious onset. A patient may describe difficulty with buttoning their clothes or tripping over their feet, which may be signs of worsening weakness and progression of the disease. Inherited polyneuropathies may present with ataxia and muscle cramping, while acquired polyneuropathies typically present as burning or paraesthesias.  EDX can be useful in finding an etiology and thus guiding treatment. Therefore, obtaining a detailed history, including medications, diet, social/occupational history, and family history is critical. Exposure to heavy metal toxins or certain medications, such as vincristine and amiodarone, or a medical history significant for hypothyroidism or vitamin b12 deficiency indicate a risk for developing peripheral polyneuropathies. Patients with a family history of hereditary motor and sensory neuropathies (HMSN) such as Charcot-Marie-Tooth Type 1 disease (HMSN I) or Refsum’s disease (HMSN IV) might point the clinician toward a hereditary cause for the neuropathy. Infectious diseases such as Lyme disease and HIV and autoimmune-mediated diseases such as acute inflammatory demyelinating polyneuropathy or chronic inflammatory demyelinating polyneuropathy can also present with symptoms of peripheral neuropathy.  The physical examination may also assist in identifying the etiology and characteristics of peripheral neuropathy. Patients with diabetic peripheral neuropathy often describe a “stocking-glove” distribution of numbness in the hands and feet. Deafness, cataracts, or musculoskeletal deformities point toward a hereditary cause. A predominant sensory neuropathy may have decreased light touch or vibration sensation, while distal muscle weakness and decreased deep tendon reflexes may indicate a motor-predominant neuropathy. For example, a patient on dapsone therapy may present with weakness and abnormal deep tendon reflexes and has electrodiagnostic findings of primarily axonal motor neuropathy. A patient who had high dose cis-platinum therapy may have preserved deep tendon reflexes and muscle strength but complained of abnormal sensation, as well as other drug side effects such as ototoxicity and gastrointestinal upset. In cis-platinum peripheral neuropathy, EDX often yields the presence of a primarily axonal sensory neuropathy.

摘要

周围神经病变,也称为周围性多发性神经病变,是一个通用术语,涵盖了多种不同类型的疾病,这些疾病会以多种不同模式导致周围神经系统的神经受损和功能障碍。电诊断(EDX)测试不仅可以确定是否可能存在周围神经病变,还可以帮助临床医生获取信息,以确定疾病的病因、严重程度和预后。全面的病史采集、体格检查和EDX对于评估和治疗出现周围神经病变症状的患者都至关重要。虽然周围性多发性神经病变常见于糖尿病患者或过度饮酒者,但许多病症都与周围神经病变有关。周围性多发性神经病变可以是后天获得性的,如糖尿病、淀粉样变性、艾滋病或顺铂化疗导致的;而有些则可能是遗传性的,如夏科-马里-图斯病。大多数周围性多发性神经病变的常见症状包括四肢远端(如足部或手部)的感觉异常、麻木或疼痛。有些症状可能急性出现,而许多症状的起病较为隐匿。患者可能会描述扣扣子困难或走路绊倒,这可能是病情加重、虚弱加剧和疾病进展的迹象。遗传性神经病变可能表现为共济失调和肌肉痉挛,而后天获得性神经病变通常表现为灼痛或感觉异常。EDX有助于找出病因,从而指导治疗。因此,获取详细的病史,包括用药情况、饮食、社会/职业史和家族史至关重要。接触重金属毒素或某些药物,如长春新碱和胺碘酮,或有甲状腺功能减退或维生素B12缺乏的病史,都表明有发生周围神经病变的风险。有遗传性运动和感觉神经病变(HMSN)家族史的患者(如1型夏科-马里-图斯病(HMSN I)或雷夫叙姆病(HMSN IV))可能提示临床医生该神经病变的遗传病因。莱姆病和艾滋病等传染病以及急性炎症性脱髓鞘性多发性神经病或慢性炎症性脱髓鞘性多发性神经病等自身免疫介导的疾病也可能出现周围神经病变的症状。体格检查也有助于确定周围神经病变的病因和特征。糖尿病周围神经病变患者常描述手脚麻木呈“手套-袜套”样分布。耳聋、白内障或肌肉骨骼畸形提示遗传病因。以感觉为主的神经病变可能会出现轻触觉或振动觉减退,而远端肌肉无力和腱反射减弱可能表明以运动为主的神经病变。例如,接受氨苯砜治疗的患者可能会出现无力和异常的腱反射,电诊断结果主要为轴索性运动神经病变。接受高剂量顺铂治疗的患者可能腱反射和肌肉力量保留,但主诉感觉异常,以及其他药物副作用,如耳毒性和胃肠道不适。在顺铂所致的周围神经病变中,EDX通常显示主要为轴索性感觉神经病变。

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