Symptomatic dolichoectasia of the vertebrobasilar system was found in 23 patients (16 males and 7 females, mean age: 62 years) during a 13-year period. Arterial hypertension was noted in 20 cases and associated aortic ectasia in 4. The malformation was identified in all patients on CT completed by angiography in 19, MRI in 7. Autopsy was performed in 5 cases. Fourteen subjects (group 1) presented with a vascular event (ischemic in 13) affecting the brainstem and/or cerebellum. Nine other patients (group 2) had a chronic symptomatology resulting from compression of the cranial nerves, central nervous system and/or CSF pathway. Two patients died of stroke within the first month (rupture of the ectasia in one and occlusion in the other one). The 21 survivors were followed for a mean period of 45.3 months. Eight patients had a stroke, with a significantly higher incidence in group 1 than in group 2 (p less than 0.05). Ten patients (5 in each group) developed progressive dementia possibly resulting from multiple cerebral infarction, hypertensive leucoencephalopathy, and/or hydrocephalus. Twelve patients died during the follow-up (4 of stroke, 6 of profound mental and motor deterioration, one from ruptured ectatic aorta, and the last one of unrecognized cause). The actuarial survival rate was 60% after 3 years of follow-up. Except for the incidence of stroke, inaugural manifestations (stroke vs nervous compression) did not seem to influence the long-term prognosis.
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