Ono S, Imai T, Yamauchi M, Nagao K
Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, Chiba, Japan.
J Neurol. 1996 Oct;243(10):693-9. doi: 10.1007/BF00873974.
We performed morphological studies of skin and measured glycosaminoglycans in the urine from patients with sporadic amyotrophic lateral sclerosis (ALS) and control subjects. The wide spaces separating collagen bundles reacted strongly with alcian blue stain in ALS patients and stained more markedly as ALS progressed. Staining with alcian blue was virtually eliminated by Streptomyces hyaluronidase. The urinary excretion of hyaluronic acid (HA) (mg/day) was significantly increased (P < 0.01) in ALS patients compared with that of control subjects, and there was a significant positive correlation between the excreted amount of HA and the duration of illness in advanced ALS patients with a duration of more than 2 years from clinical onset (r = 0.72, P < 0.02). We suggest that sporadic ALS includes a metabolic disorder of HA in which an accumulation of HA in the skin is linked to an increased urinary excretion of HA.
我们对散发性肌萎缩侧索硬化症（ALS）患者和对照受试者的皮肤进行了形态学研究，并测量了他们尿液中的糖胺聚糖。在ALS患者中，分隔胶原束的宽大间隙与阿尔辛蓝染色反应强烈，且随着ALS病情进展染色更明显。用透明质酸酶处理后，阿尔辛蓝染色几乎消失。与对照受试者相比，ALS患者的透明质酸（HA）尿排泄量（毫克/天）显著增加（P < 0.01），并且在临床发病超过2年的晚期ALS患者中，HA排泄量与病程之间存在显著正相关（r = 0.72，P < 0.02）。我们认为散发性ALS包括HA的代谢紊乱，其中皮肤中HA的积累与HA尿排泄增加有关。
