Spontaneous cerebral haemorrhage without hypertension in non-mosaic 45X Turner's syndrome.

Cerebral haemorrhage without hypertension, arteriosclerosis or clotting defect has not been reported in patients with Turner's syndrome before. In a 51 year old female patient with non-mosaic Turner's syndrome, acute aphasia and right-sided hemiplegia occurred, due to left-sided basal ganglia haemorrhage. The history for hypertension was negative, blood pressure was normal throughout hospitalisation as well as during 24 h monitoring, and all tests for secondary hypertension were negative. There was no indication of arteriosclerosis or a clotting defect. Since there were hypermobile joints, hyperextensible skin and ectatic ascending aorta and brachiocephalic trunk on angiography, a general connective tissue defect was assumed, making arteries more vulnerable to physiologically increased blood pressure and rupture of intracerebral arteries with consecutive bleeding.