[Spinal form of Ullrich's disease in monozygotic twins]. / Spinal'naia forma bolezni Ul'rikha u monozigotnykh bliznetsov.

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.

[Lesions of the cervical region of the spinal cord in so-called infantile cerebral palsy]. / O porazhenii sheinogo otdela spinnogo mozga pri tak nazyvaemom detskom tserebral'nom paraliche

A EMG study of the brachial muscles in 41 children with spastic paralyses of the lower extremities, weakness of the hands concomitant with a mild hypotrophy of the muscles, disturbed functions of the III, VII and vestibular nerves and satisfactory mental development revealed the following: there were signs of motoneuron excitation in the cervical part of the spinal cord (motor units at rest in a maximum muscle contraction and in an elaboration of tenden reflexes), their destruction (a decreased amplitude of potentials of volitional contraction in combination with muscle hypotrophy) and a desinhibition of spinal mechanisms. Causes of such damage may be intrauterine pathology, perinatal hypoxia, delivery complicated by an incorrect position of the foetus and a mechanical trauma in an incomplete ossification of the spine, in premature infants.