DYSKINETIC CEREBRAL-PALSY - A CLINICAL AND GENETIC-STUDY

The clinical features and family histories of 20 adults with dyskineti c cerebral palsy from 20 families were studied. The majority of the pa tients showed progressive neurological deterioration in adult life. In only three did the condition stabilise by 10 years of age and in seve n there was deterioration after the age of 30. Two patients developed a secondary cervical spondylotic myelopathy. Four patients had affecte d relatives and there were similar proportions of affected parents and siblings. The family data suggest genetic heterogeneity with autosoma l recessive and dominant variants. The existence of an X-linked form c annot be excluded, and the demonstration of an increased paternal age effect among single cases suggests that some of these may arise becaus e of fresh dominant genetic mutation.