Poncelet Ann N, Connolly M Kari
Department of Neurology, Box 0114, University of California, San Francisco, San Francisco, California 94143, USA.
Muscle Nerve. 2003 Sep;28(3):330-5. doi: 10.1002/mus.10439.
Because patients with scleroderma report neuropathic symptoms including numbness, paresthesias, and dysesthesias, we assessed peripheral nerve function in such patients. Fourteen scleroderma patients underwent complete neurologic examination, nerve conduction studies (NCS) and quantitative sensory testing (QST). Neurologic examination revealed reduced vibration (7) or pinprick (4) sensation in the upper or lower extremities, focal atrophy or proximal weakness (2), and decreased deep tendon reflexes (2). NCS showed reduced sensory nerve action potentials (1) and carpal tunnel syndrome (1). QST of the upper and lower extremity revealed increased cold or vibration detection thresholds in 8 of 14 patients. Our findings suggest that peripheral neuropathy occurs in patients with scleroderma at a higher frequency than previously appreciated. These findings cannot be ascribed to compression neuropathies, but rather involve large and small fibers in a non-length-dependent fashion. Larger, prospective studies using the more sensitive QST as well as pathologic studies of nerve, including cutaneous innervation, are needed to further assess the characteristics and etiology of the neuropathy.
由于硬皮病患者报告有包括麻木、感觉异常和感觉障碍在内的神经病变症状,我们对这类患者的外周神经功能进行了评估。14例硬皮病患者接受了全面的神经学检查、神经传导研究(NCS)和定量感觉测试(QST)。神经学检查发现,上肢或下肢的振动觉(7例)或针刺觉(4例)减退、局灶性萎缩或近端肌无力(2例)以及腱反射减弱(2例)。NCS显示感觉神经动作电位降低(1例)和腕管综合征(1例)。上下肢的QST显示,14例患者中有8例的冷觉或振动觉检测阈值升高。我们的研究结果表明,硬皮病患者外周神经病变的发生率高于以往的认识。这些发现不能归因于压迫性神经病变,而是以非长度依赖性方式累及大纤维和小纤维。需要使用更敏感的QST进行更大规模的前瞻性研究以及包括皮肤神经支配在内的神经病理学研究,以进一步评估神经病变的特征和病因。