Gemignani F, Manganelli P, Pavesi G, Marbini A
Institute of Clinical Neurology, University of Parma, Italy.
Funct Neurol. 1988 Jul-Sep;3(3):337-48.
A 38-year-old woman suffering from primary Sjögren's syndrome displayed a neurologic picture consisting of left tonic pupil, generalized tendon areflexia and left-sided hypohidrosis. Electrophysiological and pathological studies suggested a mild degree of peripheral nerve involvement. Moderate loss of large myelinated fibres and obliteration of small endoneurial vessels were seen in the sural nerve biopsy. Sympathetic skin response was absent and alterations of unmyelinated nerve fibres were found. A prevalently autonomic neuropathy with tonic pupils may represent a characteristic picture in the spectrum of peripheral nerve involvement in Sjögren's syndrome. Other similar cases reported in the literature are reviewed.
一名患有原发性干燥综合征的38岁女性表现出一系列神经系统症状，包括左侧强直性瞳孔、全身性腱反射消失和左侧多汗症。电生理和病理学研究提示存在轻度外周神经受累。腓肠神经活检显示有大量有髓纤维中度缺失和小神经内膜血管闭塞。交感神经皮肤反应缺失，且发现无髓神经纤维有改变。伴有强直性瞳孔的主要自主神经病变可能是干燥综合征外周神经受累谱中的一种特征性表现。本文回顾了文献中报道的其他类似病例。
