Shimauchi M, Kaji Y, Goya T, Kinoshita K
Department of Neurosurgery, Miyazaki Medical College.
No Shinkei Geka. 1989 Oct;17(10):981-4.
We report a case of fibromuscular dysplasia (FMD) presenting a transient ischemic attack (TIA) like Moyamoya disease. The patient, a 16-year-old woman, had recurrent attacks of right hemiparesis induced by hyperventilation. Neurological examinations revealed no abnormality. Angiography showed severe stenosis of both anterior cerebral arteries at the proximal portion with "string of beads" appearance in the left pericallosal artery, and tubular stenosis of the left internal carotid artery at the level of the second cervical vertebra. Renal artery stenosis was not seen. A cerebral-blood-flow (CBF) study with 133Xe SPECT showed marked reduction of CBF in the left cerebral hemisphere at times of hyperventilation. Extracranial-intracranial bypass surgery was performed and histological examination of the superficial temporal artery (STA) revealed intimal fibroplasia which was compatible with FMD. Postoperative angiography showed good filling of the middle cerebral arteries from the STA. The patient has had no recurrence of TIA for a year since the operation. CBF study showed mild low CBF in the region of the left anterior cerebral arterial circulation at hyperventilation. There is no report of intracranial FMD presenting an ischemic symptom induced by hyperventilation. Clinical diagnosis of Moyamoya disease should be made carefully when extracranial vascular lesion accompanies it.
我们报告一例纤维肌性发育异常（FMD），表现为类似烟雾病的短暂性脑缺血发作（TIA）。患者为一名16岁女性，因过度换气诱发右侧偏瘫反复发作。神经系统检查未发现异常。血管造影显示双侧大脑前动脉近端严重狭窄，左侧胼周动脉呈“串珠样”外观，左侧颈内动脉在第二颈椎水平呈管状狭窄。未发现肾动脉狭窄。用133Xe单光子发射计算机断层扫描（SPECT）进行的脑血流量（CBF）研究显示，过度换气时左侧大脑半球CBF明显降低。进行了颅外-颅内搭桥手术，颞浅动脉（STA）组织学检查显示内膜纤维增生，符合FMD。术后血管造影显示STA供血良好，大脑中动脉显影。自手术以来，患者TIA未再复发。CBF研究显示，过度换气时左侧大脑前动脉循环区域CBF轻度降低。目前尚无颅内FMD表现为过度换气诱发缺血症状的报道。当伴有颅外血管病变时，应谨慎做出烟雾病的临床诊断。
