Two cases of intracranial fibromuscular dysplasia whose repeated angiography disclosed progression of the lesion

Fibromuscular dysplasia (FMD) is well known owing to the characteristic angiographical finding of a "string of beads" appearance, but intracranial involvement with this disease is extremely rare. Moreover, to our knowledge, only seven cases that had repeated angiograms disclosed progression of FMD lesion in the literature. Such cases of intracranial FMD which showed progression in the follow-up angiography are reported. Case 1: A 8-year-old boy was referred to our hospital because of aphasia and right hemiplegia following right hemiconvulsion. Left carotid angiography on the 7th day from the onset revealed a "string of beads" appearance involving the left middle cerebral artery from M1 to M2 portion. He was treated with low molecular dextran, urokinase and steroid. After these drugs were administered, his speech was normalized. A repeat left angiogram performed two months later disclosed definite increase in the degree of stenosis associated with FMD. Perivascular sympathectomy around common and internal carotid artery and superior cervical ganglionectomy on the left side carried out on the 70th day from the onset. Postoperative left carotid angiogram showed improvement of the stenosis markedly, and the motor disturbance was improved gradually. Case 2: A 34-year-old woman presented with head dullness and disorientation suddenly. Left carotid angiogram on the third day from the onset showed a "string of beads" appearance from C1 to M1 portion. Follow-up angiography three days later revealed some progression of the stenosis. Furthermore a repeat left angiogram disclosed occlusion of left internal carotid artery at the C2 portion. Left STA-MCA bypass surgery was performed on the 61st day from the onset.(ABSTRACT TRUNCATED AT 250 WORDS)