Tokumaru Y, Hirayama K, Kita K, Kawamura M, Katayama K
Department of Neurology, School of Medicine, Chiba University.
Rinsho Shinkeigaku. 1990 Sep;30(9):933-8.
Two patients of ataxic sensory neuropathy associated with silicosis were studied. Case 1 is a 53-year-old (in 1979) man who was a stonecutter for 40 years and diagnosed as silicosis in 1973. Case 2 is a 64-year-old (in 1984) man who was a glasscutter for 30 years and had been treated for silicosis from 1980 to 1982. Both patients developed dysesthesias in the hands, feet and face asymmetrically and gait ataxia over a few months. Vibratory and joint position senses were profoundly diminished but were accompanied by only mildly decreased pain and temperature sensations. Their muscle power was almost unchanged. Both had absent muscle power was almost unchanged. Both had absent muscle stretch reflexes. Sensory nerve conduction velocities were absent and motor nerve studies were almost normal. Nerve biopsy in case 2 showed a severe loss of large myelinaed fibers, and no inflammatory infiltrates and onion bulb formations. Although these findings suggested the carcinomatous neuropathy, we could not find any malignancy. Both patients had elevated polyclonal gamma-globulin levels and rheumatoid factors and, in case 2 an increase of IgG in serum. Cerebrospinal fluid showed an albumino-cytogenic dissociation and steroid therapy was successful in both patients. Case 1 died of pneumonia in 1989. Though an autopsy was not performed, his condition had continued to improve without signs of malignancy during 10 years. The condition of case 2 has also continued to improve, although ataxias remain.(ABSTRACT TRUNCATED AT 250 WORDS)
对两名与矽肺相关的共济失调性感觉神经病患者进行了研究。病例1是一名53岁(1979年)男性,曾做过40年石匠,1973年被诊断为矽肺。病例2是一名64岁(1984年)男性,曾做过30年玻璃切割工,1980年至1982年接受过矽肺治疗。两名患者均在数月内不对称地出现手部、足部和面部感觉异常以及步态共济失调。振动觉和关节位置觉明显减退,但疼痛和温度觉仅轻度降低。他们的肌肉力量几乎未变。两人的肌肉牵张反射均消失。感觉神经传导速度消失,运动神经检查基本正常。病例2的神经活检显示大的有髓纤维严重缺失,无炎性浸润和洋葱球样结构形成。尽管这些发现提示为癌性神经病,但未发现任何恶性肿瘤。两名患者的多克隆γ球蛋白水平和类风湿因子均升高,病例2血清中IgG增加。脑脊液显示蛋白细胞分离,类固醇治疗对两名患者均有效。病例1于1989年死于肺炎。虽未进行尸检,但在10年期间其病情持续改善,无恶性肿瘤迹象。病例2的病情也持续改善,尽管仍存在共济失调。(摘要截选至250字)