Kohyama J, Shimohira M, Kondo S, Fukuro S, Kouji T, Sugimoto J, Iwakawa Y
Department of Pediatrics, Faculty of Medicine, Tokyo Medical and Dental University, Japan.
Acta Neurol Scand. 1995 Jul;92(1):91-5. doi: 10.1111/j.1600-0404.1995.tb00473.x.
We investigated motor phenomena during rapid eye movement (REM) sleep in 13 patients with group A xeroderma pigmentosum aged from 11 to 39 months, and compared them with those obtained from 12 age-matched controls. At the time of sleep study, they had no abnormality on routine electrophysiological examinations. The amount of REM sleep and the incidence of motor phenomena during REM sleep in patients were similar to those in age-matched controls. However using the newly designated indices, we demonstrated disturbance on both the tonic motor inhibition occurring during the whole REM sleep period and the phasic one acting simultaneously with horizontal rapid eye movements in these patients. Since the motor inhibition during REM sleep is mediated by the subcortical structures, our study indicate that these structures are functionally impaired in group A xeroderma pigmentosum even during the early stage of the illness.
我们对13名年龄在11至39个月的A组着色性干皮病患者快速眼动（REM）睡眠期间的运动现象进行了研究，并将其与12名年龄匹配的对照组患者的相关现象进行了比较。在进行睡眠研究时，他们的常规电生理检查未发现异常。患者的REM睡眠量和REM睡眠期间运动现象的发生率与年龄匹配的对照组相似。然而，使用新指定的指标，我们证明这些患者在整个REM睡眠期发生的紧张性运动抑制以及与水平快速眼动同时出现的相位性运动抑制均受到干扰。由于REM睡眠期间的运动抑制是由皮质下结构介导的，我们的研究表明，即使在疾病早期，A组着色性干皮病患者的这些结构在功能上也已受损。
