神经型地方性克汀病神经系统体征和脑CT研究

对80例神经型地方性克汀病人进行了神经系统检查,同时对其中10例进行了脑 CT 研究。结果发现病人神经系统变化的谱带很宽,除了智力、语言、听力障碍外,还有肢体痉挛和强直,尤以下肢近端为著。典型的锥体外系受损表现,额叶释放征,木僵状态,感觉迟钝等。脑CT 主要变化为,脑 CT 值偏低,不同程度皮质指压痕样切迹,蛛网膜下腔开大,脑池增宽,脑内有点状钙化等。此外,本文还结合动物实验,尸解材料,讨论了临床表现与脑损害的定位关系。

NEUROLOGICAL SIGNS AND BRAIN CT RESEARCH IN NEUROLOGICAL ENDEMIC CRETINISM

Neurological examinations were made of 80 neurological endemic cretinismand brain CT examination were aiso made of 10 cases.The findings showed,that the spectrum of clinical neurological disorders was wide.These include,to rarying degree:deafmutism or lesser degree oF hearing-loss or dysarthria;concomitant exotropia,miotic pupiles with diminished reaction to light;spasticity particularly involving the proximal lower extremites;mental def-iciency;lack of inhibition of blinking in response to glabellar lap,facile grin;frontal release sings,stopor status.Cerebellar function was largely spared,aswere functions of vegatative and autonomic function,exept in the most seve-rety involved.The results oF brain CT showed that dysgensis oF brain,wid-en of posterior cistern and subarachnoidal cistern,and calcific dot.