Ono S, Shimizu N, Imai T, Mihori A, Nagao K
Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, Japan.
Acta Neurol Scand. 2000 Jul;102(1):47-52. doi: 10.1034/j.1600-0404.2000.102001047.x.
Several abnormalities of skin have been described in patients with amyotrophic lateral sclerosis (ALS). Bunina bodies, which are small eosinophilic intraneuronal inclusions in the remaining lower motor neurons in ALS, are the only pathologically specific hallmark of ALS. It has been demonstrated that Bunina bodies were positive for anti-cystatin C serum. However, cystatin C in the skin of ALS has not been investigated.
We studied cystatin C immunoreactivity of skin in ALS patients, and the results were compared with those of control subjects.
The epidermis of ALS was immunohistochemically strongly positive for cystatin C as compared with that of controls. The optical density of cystatin C immunostaining reactivity in ALS patients was significantly higher than in controls, and was significantly increased with duration of illness.
These data suggest that a metabolic alteration of cystatin C may take place in the skin of ALS and the increased cystatin C in skin is likely to be related to the disease process in ALS.
已在肌萎缩侧索硬化症（ALS）患者中描述了几种皮肤异常情况。布尼纳小体是ALS中剩余下运动神经元内的小嗜酸性神经元内包涵体，是ALS唯一病理特异性标志。已证明布尼纳小体对抗胱抑素C血清呈阳性。然而，尚未对ALS患者皮肤中的胱抑素C进行研究。
我们研究了ALS患者皮肤中胱抑素C的免疫反应性，并将结果与对照组进行比较。
与对照组相比，ALS患者的表皮在免疫组织化学上对胱抑素C呈强阳性。ALS患者中胱抑素C免疫染色反应性的光密度显著高于对照组，且随病程显著增加。
这些数据表明，胱抑素C的代谢改变可能发生在ALS患者的皮肤中，皮肤中胱抑素C的增加可能与ALS的疾病进程有关。
