慢性特发性假性肠梗阻(下称CIIP)是肠道推动力减低所引起的临床综合征。肠道推动力受肠道平滑肌、神经和激素的相互作用所支配。由肠肌丛病变引起的CIIP有三种不同临床表现。一种发生在二个同胞中,伴有轻度植物神经功能不全和多发性神经系统异常,神经元含有核内包涵体且整个肠道神经元和神经纤维减少。其二是发生在四个同胞中,有智力发育迟缓,基底 Chronic idiopathic pseudo-intestinal obstruction (CIIP) is a clinical syndrome caused by a decrease in intestinal motility. Intestinal motility is governed by intestinal smooth muscle, nerve and hormonal interactions. CIIP caused by myenteric plexus lesions have three different clinical manifestations. One occurs in two siblings with mild autonomic dysfunction and multiple neurological abnormalities, neurons with nuclear inclusions and reduced total intestinal neurons and nerve fibers. The second is occurring in four sibs, with mental retardation, basal