Ohnishi J, Kudo Y
Department of Psychiatry and Neurology, Kobe University School of Medicine.
Rinsho Shinkeigaku. 1990 Apr;30(4):444-7.
We reported a case of fibromuscular dysplasia (FMD) presenting lateral medullary syndrome accompanied with left truncal ataxia, left Horner's syndrome and superficial sensory deficit in the right extremities. He was 46-year-old man and had no remarkable risk factors for the cerebrovascular disease such as hypertension, diabetes mellitus and valvular heart disease. Cerebral angiography was performed and there was the string-of-beads-like shadow in the left vertebral artery, revealing a localized FMD. Wedge-shaped area of low signal intensity in the left lower medulla was recognized in magnetic resonance imaging (spin echo Tr/Te 600/30). It was suggested that the lesion of the lower medulla caused the patient's several symptoms. In Japan, there have been a few case reports of cervical or intracranial FMD, but we cannot find any report of FMD localizing in the unilateral vertebral artery. In general, it has been said that the etiology of lateral medullary syndrome under fifty years old differs from those of geriatric patients. The authors pointed out the significance of FMD as one of those risk factors causing lateral medullary syndrome and also discussed the mechanism of the sensory disturbance sparing face.
我们报告了一例纤维肌发育不良(FMD)患者,其表现为延髓外侧综合征,伴有左侧躯干共济失调、左侧霍纳综合征及右侧肢体浅感觉减退。患者为一名46岁男性,无高血压、糖尿病及瓣膜性心脏病等脑血管疾病的显著危险因素。行脑血管造影检查,发现左侧椎动脉呈串珠样阴影,提示为局限性FMD。在磁共振成像(自旋回波Tr/Te 600/30)中,左侧延髓下部可见楔形低信号区。提示延髓下部病变导致了患者的多种症状。在日本,有一些关于颈部或颅内FMD的病例报告,但我们未发现任何关于单侧椎动脉FMD的报告。一般来说,据说50岁以下的延髓外侧综合征的病因与老年患者不同。作者指出FMD作为导致延髓外侧综合征的危险因素之一的重要性,并讨论了面部感觉障碍得以幸免的机制。