Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Skin collagen abnormalities in a Japanese patient with extracranial internal carotid artery dissection followed by extracranial vertebral artery dissection

Renpei Sengoku, M.D.1), Hironori Sato, M.D.1), Hidehiko Honda, M.D.1), Kiyoharu Inoue, M.D.1) and Seiitsu Ono, M.D.2)

1)Department of Neurology, The Jikei University School of Medicine
2)Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital

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.

(CLINICA NEUROL, 46: 140|143, 2006)
key words: extracranial carotid artery dissection, extracranial vertebral artery dissection, skin biopsy, skin collagen fiber

(Received: 13-Apr-05)