Abnormal Cerebral Vessels in von Recklinghausen Disease; Report of Two Cases

We report 2 cases of von Recklinghausen disease (VR) with cerebral arterial occlusion and stenosis. The first case was a 3-year-old girl with bilateral optic gliomas. With right CAG, the right middle cerebral artery (MCA) was seen to be completely occluded at the proximal portion, and blood was supplied through telangiectasia to the distal MCA, which was again occluded at the further distal portion. The second case was a 20-year-old man with craniodysplasia. He was admitted to our hospital with left exophthalmus and mental retardation. Left CAG showed stenosis of the left MCA and chiefly through telangiectasia, blood was supplied to the cortical portion of the left MCA. Twenty-two cases including ours have been reported and in 17 of them cerebral arterial occlusion was demonstrated before 20 years of age. Eleven patients were male and 11 were female. Thirty-eight points of vascular obstruction were observed in 22 patients : in the supraclinoid portion of the internal carotid artery in 20 cases, in the proximal MCA in 7, and in the carotid siphon in 4. Cerebral arterial occlusions in VR were similar to spontaneous occlusion of the circle of Willis in terms of clinical features, location, and radiological findings. We discussed the relationship between clinical signs and angiographical findings in 12 patients with VR admitted to our hospital. Two patients with arterial occlusion clearly showed mental retardation from infancy, and it was thought that the occlusion might have been the cause of the retardation. We should consider this vascular abnormality when we meet retarded patients with VR.