Nishiyama K, Fuse S, Shimizu J, Takeda K, Sakuta M
Department of Neurology, Japanese Red Cross Medical Center.
Rinsho Shinkeigaku. 1992 Oct;32(10):1117-20.
A 25-year-old man developed Wallenberg syndrome (WS). At that time his carotid angiography was normal. When he was 28 years old, he suffered from retinal artery embolism in the left eye. At the age of 30 years, he had an acute onset of abducens nerve palsy in his right eye. The carotid angiography showed a giant aneurysm at the cavernous sinus portion in the right internal carotid artery. At his age of 38, the right oculomotor, trochlear and trigeminal nerves were involved. A vertebral angiography revealed a bead-like formation, and a diagnosis of fibromuscular dysplasia (FMD) was made. An intensive angiographic examination revealed many stenotic or dilated lesions in the carotid, vertebral, coronary, renal, and hepatic arteries. A sural nerve biopsy specimen revealed that the sural vein was involved. In Japan only one case of FMD presenting with WS is known. FMD should be under consideration as an underlying disease, when WS occurred in younger patients with few risk factors. In this patient an angiography revealed no abnormality in the cavernous sinus portion of the internal carotid artery, when he suffered from WS. However, eight years later he was proved to have a giant aneurysm in the cavernous sinus portion. In conclusion, we support the hypothesis that aneurysm may originate from angiographically normal arterial wall in FMD.
一名25岁男性患上了延髓背外侧综合征(WS)。当时他的颈动脉血管造影显示正常。28岁时,他左眼发生视网膜动脉栓塞。30岁时,他右眼急性发作展神经麻痹。颈动脉血管造影显示右侧颈内动脉海绵窦段有一个巨大动脉瘤。38岁时,他右侧动眼神经、滑车神经和三叉神经受累。椎动脉血管造影显示呈串珠样改变,诊断为纤维肌发育不良(FMD)。详细的血管造影检查发现颈动脉、椎动脉、冠状动脉、肾动脉和肝动脉有许多狭窄或扩张性病变。腓肠神经活检标本显示腓肠静脉也受累。在日本,仅已知1例FMD表现为WS。当WS发生在危险因素较少的年轻患者时,应考虑FMD作为潜在病因。该患者患WS时,血管造影显示颈内动脉海绵窦段无异常。然而,8年后证实他在海绵窦段有一个巨大动脉瘤。总之,我们支持这样的假说,即动脉瘤可能起源于FMD血管造影正常的动脉壁。