Schady W, Sheard A, Hassell A, Holt L, Jayson M I, Klimiuk P
Department of Neurology, University of Manchester, Manchester Royal Infirmary, Salford.
Q J Med. 1991 Aug;80(292):661-75.
Peripheral neuropathy in patients with scleroderma is thought to be rare. We have undertaken a quantitative assessment of peripheral nerve function in 29 patients with either limited cutaneous scleroderma or progressive systemic sclerosis. Tactile thresholds were raised in the fingers in 28 per cent of patients and in the foot in 50 per cent. Two-point discrimination was abnormal in 10 patients, thermal thresholds were abnormal in five and vibration thresholds were abnormal in one. Nerve conduction studies showed abnormalities in six patients, five of whom had clinical signs of a mild peripheral neuropathy: the mean duration of disease in these six patients was 10 years longer than that in the remainder of the patients. There was electrophysiological evidence of a subclinical carpal tunnel syndrome in two patients. The sympathetic skin response was recorded in 16 patients who had not been subjected to sympathectomy for Raynaud's phenomenon, and was abnormal in four. These results indicate that peripheral nerve dysfunction in scleroderma, though mild, is not as uncommon as previously thought. The abnormal cutaneous sensory thresholds may be partly due to altered viscoelastic properties of the skin, but abnormal responses in the lower limbs to tests of tactile sensitivity, the clinical findings and the disturbances of nerve conduction argue in favour of an additional neuropathic process in some patients. Low grade distal nerve trunk ischaemia may be responsible.
硬皮病患者的周围神经病变被认为较为罕见。我们对29例局限性皮肤型硬皮病或进行性系统性硬化症患者的周围神经功能进行了定量评估。28%的患者手指触觉阈值升高,50%的患者足部触觉阈值升高。10例患者两点辨别觉异常,5例患者温度觉阈值异常,1例患者振动觉阈值异常。神经传导研究显示6例患者存在异常,其中5例有轻度周围神经病变的临床体征:这6例患者的平均病程比其余患者长10年。2例患者有亚临床腕管综合征的电生理证据。对16例未因雷诺现象接受交感神经切除术的患者记录了交感神经皮肤反应,其中4例异常。这些结果表明,硬皮病患者的周围神经功能障碍虽然轻微,但不像以前认为的那样不常见。皮肤感觉阈值异常可能部分归因于皮肤粘弹性的改变,但下肢对触觉敏感性测试的异常反应、临床发现以及神经传导障碍表明,部分患者还存在另外的神经病变过程。低度远端神经干缺血可能是其原因。