这篇综述首次包括对UnerTan综合征中人类四足动物的动力学系统分析,其特点是习惯性的四足动物,智力受损,和基本的演讲。第一个家庭是在伊斯肯德伦附近的一个小村庄发现的,后来在阿达纳和加济安泰普和恰纳卡莱附近的另外两个小村庄发现了家庭。在所有受影响的个体中,直立行走时动态平衡受损,他们习惯性地喜欢四肢行走。MRI扫描显示三个家庭的下位小脑小脑发育不全,脑回略简化,但在第四场表现正常.PET扫描显示小脑葡萄糖代谢活性降低,Vermis和,在较小程度上是大脑皮层,除了一个病人,MRI扫描也正常。所有四个家庭的血统都有近亲婚姻,提示常染色体隐性传播.该综合征具有遗传异质性。自从最初的发现以来,已经发现了更多的病例,这些表现出兼性四足运动,在一个案例中,儿童晚期发病。有人认为,人类的四足动物可能,至少,是反向进化的表型例子。从动态系统理论的角度来看,得出的结论是,在UnerTan综合征中,可能没有一个单一的因素可以预先确定人类的四足动物,但它可能涉及自我组织,大脑可塑性,重新布线,从神经元之间的许多分散和局部相互作用中,遗传,和环境子系统。
This review includes for the first time a dynamical systems analysis of human quadrupedalism in Uner Tan syndrome, which is characterized by habitual quadrupedalism, impaired intelligence, and rudimentary speech. The first family was discovered in a small village near Iskenderun, and families were later found in Adana and two other small villages near Gaziantep and Canakkale. In all the affected individuals dynamic balance was impaired during upright walking, and they habitually preferred walking on all four extremities. MRI scans showed inferior cerebellovermian hypoplasia with slightly simplified cerebral gyri in three of the families, but appeared normal in the fourth. PET scans showed a decreased glucose metabolic activity in the cerebellum, vermis and, to a lesser extent the cerebral cortex, except for one patient, whose MRI scan also appeared to be normal. All four families had consanguineous marriages in their pedigrees, suggesting autosomal recessive transmission. The syndrome was genetically heterogeneous. Since the initial discoveries more cases have been found, and these exhibit facultative quadrupedal locomotion, and in one case, late childhood onset. It has been suggested that the human quadrupedalism may, at least, be a phenotypic example of reverse evolution. From the viewpoint of dynamic systems theory, it was concluded there may not be a single factor that predetermines human quadrupedalism in Uner Tan syndrome, but that it may involve self-organization, brain plasticity, and rewiring, from the many decentralized and local interactions among neuronal, genetic, and environmental subsystems.