Poliakova N F, Lebezeva I V, Kisel' S A, Oknin V Iu
Zh Nevropatol Psikhiatr Im S S Korsakova. 1991;91(3):28-9.
Three cases of a rare and little studied disease which is characterized by progressive atrophy of the tissues of one half of the face. The muscular layer gets injured to a less extent. Dysembryogenesis underlies the disease while the hypothalamic formations are primarily damaged. The animal nervous system does not experience gross changes. The cases described may be referred to the idiopathic form of the syndrome. In one of the cases, the process progressed to the transition to the hand and body, in the other two ones, the process was combined with vegetovascular dystonia. All the patients were examined for the status of the vegetative nervous system according to the evoked cutaneous sympathetic potentials, the time of the pupillary cycle and the cardiovascular tests. The data obtained failed to reveal any lesions of the peripheral vegetative nervous system, which is likely to point to the interest of the suprasegmental vegetative structures.
三例患有一种罕见且研究较少的疾病，其特征为面部一侧组织进行性萎缩。肌肉层受损程度较轻。疾病的基础是胚胎发育异常，而下丘脑结构首先受到损害。动物的神经系统未出现明显变化。所描述的病例可能属于该综合征的特发性形式。其中一例病情进展至手部和身体，另外两例病情合并有植物神经血管性肌张力障碍。根据诱发的皮肤交感神经电位、瞳孔周期时间和心血管测试对所有患者的植物神经系统状况进行了检查。所获得的数据未能显示外周植物神经系统有任何病变，这可能表明节段上植物神经结构受到影响。
