脳血管写追跡により病変進行を示したintracranial fibromuscular dysplasiaの2例

Two CASES OF INTRACRANIAL FIBROMUSCULAR DYSPLASIA WHOSE REPEATED ANGIOGRAPHY DISCLOSED PROGRESSION OF THE LESION

　抄録　脳血管写において，fibromuscular dysplasia （FMD）に特徴的とされる"string of beads"所見を頭蓋内血管に認め，しかも経時的脳血管写追跡により，FMD病変が進行していくことを捉えた2例を報告する。症例1は8歳男子，痙攣発作で発症し右半身完全麻痺，運動性失語が出現した。第7病日の左CAGで左M₁〜M₂部に"string of beads"所見を認め，第66病日のfollow-up CAGではM₁の狭窄が明らかに進行していた。本症例に対し，初めは低分子デキストラン，ウロキナーゼ，ステロイド投与を行ない，その後，左総頸動脈・内頸動脈周囲交感神経叢剥離術・上頸神経節切除術を施行したところ，症状の改善，脳血管写上の狭窄改善をみた。症例2は34歳女性。突然の右下肢脱力，失見当識で発症したが・症状は3時間後に消失した。第3病日の左CAGで左C₁〜M₁に"string of beads"所見を認め，第6病日でのfollow-up CAGで狭窄の進行を，更に第19病日には左内頸動脈閉塞を確認した。本症例に対し，STA-MCA吻合術を施行し，以後，神経症状の出現をみていない。頭蓋内血管でのFMD報告例は少なく，またstaticな病変とされるFMDの血管撮影追跡例も少ない。文献例と併せ，自験例を考察した。

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 progres-sion of FMD lesion in the literature.

Such cases of intracranial FMD which showed progression in the follow-up angiography are re-ported.

Case 1: A 8-year-old boy was referred to our hospital because of aphasia and right hemiplegia following right hemiconvulsion. Left carotid angio-graphy 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, uro-kinase and steroid. After these drugs were admi-nistered, his speech was normalized. A repeat left angiogram performed two months later disclosed difinite increase in the degree of stenosis associa-ted 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 ste-nosis. Furthermore a repeat left angiogram dis-closed occlusion of left internal carotid artery at the C₂ portion. Left STA-MCA bypass surgery was performed on the 61 st day from the onset. Postoperative course was uneventful and she left hospital without neurological deficit.

According to the previous report, prognosis for FMD was good and progression of FMD was rare. But the analysis of the intracranial FMD in the reported cases indicated that the patients were below 40 years of age, especially younger persons, and the lesion were ipsilateral in many cases, moreover that there were not always good prognosis (Table 1).

It was certain that clinical course were not always consist with the angiographical progression of the FMD lesion (Table 2).

Judging from our cases and analysis of the previous literature, it is suggested that some FMDcases which have been believed to be static lesion lead to angiographically dynamic chage and there may be cause of asymptomatic, completed occlu-sion of internal carotid artery or middle cerebral artery.