偏头痛伴皮层下梗死和白质脑病的常染色体显性遗传性脑动脉病临床分析

目的探讨国人伴皮层下梗死和白质脑病的常染色体显性遗传性脑动脉病合并偏头痛的可能性。方法对1例临床表现为偏头痛、记忆力减退和情感障碍的患者进行临床MRI、皮肤活检电镜检查。结果患者有家族性偏头痛史，记忆力减退、情感障碍IMRI显示多发性腔隙梗死、白质疏松；皮肤血管内皮细胞增厚，血管平滑肌嗜锇颗粒阳性。结论中国人伴皮层下梗死和白质脑病的常染色体显性遗传性脑动脉病也有可能伴有偏头痛。皮肤活检的血管变化与脑动脉病的关系尚不明。

One Case Study on the Cerebral Arteriopathy Due to Autosomal Dominant Implicated in Subcortical Infarcts and Leukoencephalopathy, and with the Migraine

Objective To evaluate the possibility of migraine in Chinese with the cerebral arteriopathy due to autosomal dominant implicated in subcortical infarcts and leukoeneephalopathy. Methods Clinical data of MRI and electron-microscopy of skin biopsy were collected from one case of Chinese with the cerebral arteriopathy due to autosomal dominant implicated in subcortical infarcts and leukoencephalopathy. Results The clinical features presented migraine with mild cognitive deficiency and slightly mood disorder; cerebral MRI showed multi-infarct and leukoaraiosis; ultra-structural changes in skin biopsy appeared obvious thickening of arteriole intima and basal lamina of endothelial cells; osmophilic materials were found on the surface of vascular smooth muscle cells. Conclusions Family history of migraine, depression and mild cognitive impairment occurs in Chinese patients of the cerebral arteriopathy due to autosomal dominant implicated in subcortical infarcts and leukoencephalopathy. The exact relationship between the cerebral arteriolpathy and ultra-structural changes in the skin biopsy is unknown.