Kohyama J
Division of Human Ontogeny and Childhood Development, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan.
Brain Dev. 2001 Nov;23(7):523-7. doi: 10.1016/s0387-7604(01)00309-6.
In this brief review, the sleep studies on patients with West syndrome (WS) were summarized. In addition to the previously reported common finding for sleep in WS--reduction of the amount of rapid-eye-movement (REM) sleep--weakness of phasic suppression of chin muscle activity in WS patients has recently been found. The degree of this weakness is quantified by the phasic inhibition index (PII), which has been found to reflect a patient's prognosis as to convulsions. PII is proposed to be a useful parameter for assessing the prognosis of WS. Since the pontine tegmentum is involved in the production of the REM-related phasic loss of muscle activity in REM sleep, WS patients are hypothesized to have a functional instability of the pontine tegmentum. After adrenocorticotropin (ACTH) treatment, PII decreased significantly in all WS patients examined. Taken together with the effects of corticosteroids on PII, and the incidence of phasic chin muscle activity in patients with congenital adrenal hyperplasia and nephrotic syndrome, ACTH is hypothesized to suppress the spasms in WS patients not only through corticosteroids, but also through a direct action on the pontine tegmentum. Since PII has been reported to be elevated in patients with an autistic tendency, the appearance of an autistic tendency is also hypothesized to be involved in the functional disturbance of the pontine tegmentum.
在这篇简短的综述中，总结了对韦斯特综合征（WS）患者的睡眠研究。除了先前报道的WS患者睡眠的常见发现——快速眼动（REM）睡眠量减少——最近还发现WS患者下巴肌肉活动的相位抑制减弱。这种减弱的程度通过相位抑制指数（PII）进行量化，已发现该指数可反映患者惊厥的预后。PII被认为是评估WS预后的一个有用参数。由于脑桥被盖参与了REM睡眠中与REM相关的肌肉活动相位丧失的产生，因此推测WS患者存在脑桥被盖功能不稳定。在促肾上腺皮质激素（ACTH）治疗后，所有接受检查的WS患者的PII均显著下降。结合皮质类固醇对PII的影响，以及先天性肾上腺皮质增生和肾病综合征患者下巴肌肉活动相位的发生率，推测ACTH不仅通过皮质类固醇，还通过对脑桥被盖的直接作用来抑制WS患者的痉挛。由于据报道有自闭症倾向的患者PII会升高，因此也推测自闭症倾向的出现与脑桥被盖的功能障碍有关。
