Aver'ianov Iu N, Il'ina N A, Mazaeva I V, Berezova N Iu
Zh Nevropatol Psikhiatr Im S S Korsakova. 1982;82(3):51-4.
The authors describe congenital Ulrich's arthrogryposis in two five-year-old boys who were monozygotic twins. Examinations of biopsy specimens from muscles revealed in both of them fascicular atrophy of the muscular fibres, the fact, that pointed to the neurogenic character of the disease. The EMG findings pointed to the spinal level of the affection. The results of the examination have given the authors grounds to regard the patients' disease as a spinal form of Ulrich's disease. This form is described in literature for the first time. A brief review of literature dealing with Ulrich's arthrogryposis is presented.
作者描述了两名5岁同卵双胞胎男孩患先天性乌尔里希关节挛缩症的情况。对肌肉活检标本的检查发现，两人的肌纤维均有束状萎缩，这一事实表明该疾病具有神经源性特征。肌电图检查结果指向病变的脊髓水平。检查结果使作者有理由将患者的疾病视为乌尔里希病的脊髓型。这种类型在文献中首次被描述。本文还对有关乌尔里希关节挛缩症的文献进行了简要综述。
