[Spinal form of Ullrich's disease in monozygotic twins]

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.