Amyloid neuropathies.

Amyloid neuropathies.
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DOI:
10.1002/msj.21352
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发表时间:
2012-11
影响因子:
--
通讯作者:
Robinson-Papp, Jessica
Robinson-Papp, Jessica
中科院分区:
其他
文献类型:
--
作者:
Shin, Susan C.;Robinson-Papp, Jessica

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周围神经病变是许多系统性淀粉样病变的常见并发症。虽然神经病变的原因尚不完全清楚,但可能与神经内淀粉样蛋白沉积有关。这可能导致局灶性、多灶性或弥漫性神经病变,包括感觉、运动和/或自主神经纤维。表现出的症状取决于受影响神经的分布。最常见的表型之一是感觉运动多神经病变,其特征是神经性疼痛,麻木,在晚期病例虚弱。症状从足部开始,最终发展到腿近端和手。最常见的局灶性神经病变是手腕的正中神经病变,临床上称为腕管综合征。腕管症状可能包括手掌和手指外侧的疼痛和感觉障碍;如果局灶性神经病变严重,可能会出现手部无力。自主神经病变可影响多种器官系统,如心血管、胃肠道和泌尿生殖系统。症状可能是非特异性的,这使得自主神经病变的诊断更加难以识别。然而,识别和区分自主神经病变与终末器官本身的疾病是很重要的。本章回顾了影响周围神经系统的遗传性和获得性淀粉样病变,包括家族性淀粉样多神经病变,以及原发性、继发性和老年性淀粉样变性。我们强调这些疾病的神经学方面的临床表现,体格检查结果,适当的诊断评估,治疗和预后。
Peripheral neuropathy is a common complication of many of the systemic amyloidoses. Although the cause of neuropathy is not entirely clear, it is likely related to amyloid deposition within the nerve. This may lead to focal, multifocal, or diffuse neuropathies involving sensory, motor and/or autonomic fibers. The presenting symptoms depend on the distribution of nerves affected. One of the most common phenotypes is sensorimotor polyneuropathy, which is characterized by symptoms of neuropathic pain, numbness, and in advanced cases weakness. Symptoms begin in the feet and ultimately progress to the proximal legs and hands. The most common focal neuropathy is a median neuropathy at the wrist, or clinically known as carpal tunnel syndrome. Carpal tunnel symptoms may include pain and sensory disturbances in the lateral palm and fingers; hand weakness may ensue if the focal neuropathy is severe. Autonomic neuropathy may affect a variety of organ systems such as the cardiovascular, gastrointestinal, and genitourinary systems. Symptoms may be non-specific making the diagnosis of autonomic neuropathy more difficult to identify. However, it is important to recognize and distinguish autonomic neuropathy from diseases of the end-organs themselves. This chapter reviews the inherited and acquired amyloidoses that affect the peripheral nervous system including familial amyloid polyneuropathy, and primary, secondary and senile amyloidosis. We emphasize the clinical presentation of the neurologic aspects of these diseases, physical examination findings, appropriate diagnostic evaluation, treatment and prognosis.
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发表时间: 2009-09
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影响因子: 3.4
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发表时间: 2006-05-01
期刊: MUSCLE & NERVE
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影响因子: 9.9
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DOI: 10.1001/archneur.1969.00480110054005
发表时间: 1969-01-01
影响因子: --
作者:
DYCK, PJ;LAMBERT, EH
通讯作者: LAMBERT, EH