Sengoku Renpei, Sato Hironori, Honda Hidehiko, Inoue Kiyoharu, Ono Seiitsu
Department of Neurology, The Jikei University School of Medicine.
Rinsho Shinkeigaku. 2006 Feb;46(2):140-3.
A 41-year-old man with hypertension and hyperlipidemia who complained of left hemiparesis after a temporal headache was admitted to our hospital. A cervical MRI with gadolinium enhancement revealed an intramural hematoma is compatible with right extracranial internal carotid artery dissection. Two weeks later, he complained of sudden onset of pain in the right side of his neck. The right extracranial internal carotid artery dissection followed by the right extracranial vertebral artery dissection was diagnosed. Spontaneous cervical artery dissection (SCAD) is one of the causes of stroke in young adults. The pathogenesis of SCAD remains unknown. Minor trauma like an excessive sneeze, migraine, and connective tissue disorders such as fibromuscular dysplasia and Ehlers-Danlos syndrome are well-known as risk factors for SCAD. Pathologically skin collagen abnormalities have been seen in German patients with SCAD without clinical evidence for any specific connective tissue disorder. We examined the ultrastructural morphology of the Japanese patient's dermal connective tissue components by electron microscopy. The patient's collagen fibers contained fibrils with highly variable diameters, and there were other ultrastructural abnormalities, including flower-like fibrils and large-diameter composite fibrils. This is the first report of a case of ultrastructural abnormalities of dermal connective tissue in a Japanese patient with SCAD.
一名41岁患有高血压和高脂血症的男性,在颞部头痛后出现左侧偏瘫,被收治入院。颈椎钆增强磁共振成像显示壁内血肿,符合右侧颅外颈内动脉夹层。两周后,他主诉右侧颈部突然疼痛。诊断为右侧颅外颈内动脉夹层继以右侧颅外椎动脉夹层。自发性颈动脉夹层(SCAD)是年轻成人卒中的病因之一。SCAD的发病机制尚不清楚。如过度打喷嚏、偏头痛等轻微创伤以及诸如纤维肌发育异常和埃勒斯-当洛综合征等结缔组织疾病是SCAD的已知危险因素。在德国患有SCAD的患者中,病理检查发现皮肤胶原异常,但无任何特定结缔组织疾病的临床证据。我们通过电子显微镜检查了该日本患者真皮结缔组织成分的超微结构形态。患者的胶原纤维包含直径高度可变的原纤维,并且存在其他超微结构异常,包括花状原纤维和大直径复合原纤维。这是首例关于日本SCAD患者真皮结缔组织超微结构异常的病例报告。