Clinical, electrophysiologic, and pathologic evidence for sensory abnormalities in ALS
Clinical, electrophysiologic, and pathologic evidence for sensory abnormalities in ALS
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DOI：
10.1212/01.wnl.0000286948.99150.16
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发表时间：
2007-12-11
期刊：
影响因子：
9.9
通讯作者：
Benatar, M.
中科院分区：
文献类型：
--
作者：
Hammad, M.;Silva, A.;Benatar, M.
背景：肌萎缩侧索硬化症(ALS)是一种进行性的上下运动神经元退行性疾病。关于肌萎缩侧索硬化症感觉神经受累的性质和频率的报道各不相同。方法：我们回顾了1997-2004年间埃默里大学运动神经元病登记处，以确定103名没有可能导致感觉异常的并存疾病的肌萎缩侧索硬化症患者，并对其进行了电诊断研究。神经生理学研究基于我们实验室的年龄调整的标准数据进行解释。结果：32%的ALS患者存在感觉症状或体征，27%的患者腓肠感觉神经动作电位波幅异常，91%的患者存在病理异常。大口径有髓纤维主要受累(73%)，小口径有髓纤维受累较少(23%)。95%的活检组织中有稀疏髓鞘纤维，77%的活检组织中可见再生纤维团。梳理纤维分析显示轴突变性和再生的频率增加，以及过度的髓鞘不规则。形态计量学证实了大口径纤维的丢失。结论：这些数据表明，三分之一的肌萎缩侧索硬化症患者报告了感觉症状，在类似比例的受试者中，腓肠神经感觉反应幅度降低。91%的接受腓肠神经活检的患者存在感觉神经病理的病理证据。电生理和病理结果显示轴突丢失模式，主要影响大口径有髓纤维。
Background: Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease of upper and lower motor neurons. Reports of the nature and frequency of sensory nerve involvement in ALS have varied.Methods: We reviewed the Emory University motor neuron disease registry between 1997 and 2004 to identify 103 patients with ALS without coexisting diseases that might cause sensory abnormalities and for whom electrodiagnostic studies were available for review. Neurophysiologic studies were interpreted based on age-adjusted normative data from our laboratory. Twelve control biopsies were evaluated alongside 22 samples from patients with ALS to ensure blinded evaluation of pathologic specimens.Results: Sensory symptoms or signs were present in 32% of patients, sural sensory nerve action potential amplitudes were abnormal in 27%, and pathologic abnormalities were present in 91% of patients. Large-caliber myelinated fibers were predominantly affected (reduced in 73%) and small-caliber myelinated fibers were affected less often (23%). Thinly myelinated fibers were present in 95% and regenerating clusters in 77% of the biopsies. Teased fiber analysis showed an increased frequency of axonal degeneration and regeneration as well as excessive myelin irregularity. Morphometry confirmed the loss of large-caliber fibers.Conclusions: These data indicate that one third of patients with amyotrophic lateral sclerosis report sensory symptoms and sural sensory response amplitudes are reduced in a similar proportion of subjects. Pathologic evidence of sensory nerve pathology was present in 91% of patients who underwent sural nerve biopsy. The electrophysiologic and pathologic findings indicate a pattern of axonal loss that predominantly affects large-caliber myelinated fibers.