烟雾病认知功能障碍研究进展

Research advances in cognitive impairment in moyamoya disease

摘要:

烟雾病（MMD）是一种多发于东亚地区的慢性进展性脑血管疾病，其特点是双侧颈内动脉末端或大脑前动脉、大脑中动脉起始部进行性狭窄和闭塞，伴受累动脉供血区软脑膜和脑底部细小的烟雾状代偿血管形成。MMD患者的认知功能障碍发病率较高，是MMD的重要并发症之一，对患者造成严重的健康损害和社会负担，MMD并发认知功能障碍属于非痴呆血管性认知障碍，脑灌注不足造成的脑白质损伤与认知功能障碍密切相关，是MMD患者认知功能障碍的主要和始动原因。损害靶点主要位于额叶，左侧大脑半球损害在MMD患者认知能力下降中发挥着更重要的作用，临床上主要有3种表现，即智力受损、执行功能受损和记忆力受损，对儿童患者的影响程度高于成人患者，抑郁、焦虑和创伤后应激障碍的患病率较高，这3种精神障碍与MMD患者更严重的神经功能受损和更严重的认知缺陷相关。认知量表检测、影像学、相关的生物标志物可以作为诊断认知功能障碍的检查手段，目前无逆转MMD认知功能障碍病情进展的有效药，脑血运重建术是治疗MMD认知功能障碍的有效措施，康复治疗和饮食管控可以有一定的治疗作用。临床医师需要加强对此类患者的认识，建议在有条件的医疗单位对MMD患者开展认知功能障碍的普查，以期及早发现，及早诊断，及早治疗。

Abstract:

Moyamoya disease （MMD） is a chronic progressive cerebrovascular disease that often occurs in East Asia， and it is characterized by progressive stenosis and occlusion at the end of the bilateral internal carotid arteries or the origin of the anterior cerebral artery and middle cerebral artery， accompanied by small smoke-like compensatory vascularization of the leptomeninges and the base of the brain in the supply territory of the affected arteries. There is a high incidence rate of cognitive impairment in MMD， and cognitive impairment is one of the important complications of MMD and causes severe health damage and social burden to patients. Cognitive impairment of MMD is a type of non-dementia vascular cognitive impairment， and white matter injury caused by cerebral hypoperfusion is closely associated with cognitive impairment and is the main and initial cause of cognitive impairment in patients with MMD. The target of injury is mainly located in the frontal lobe， and damage to the left cerebral hemisphere plays a more important role in cognitive decline in patients with MMD， with the three main clinical manifestations of cognition impairment， executive function， and memory， which have a greater impact on pediatric patients than adult patients. There are higher prevalence rates of depression， anxiety， and posttraumatic stress disorder， and these three mental disorders are associated with more severe neurological disability and cognitive deficits in patients with MMD. Cognitive scale assessment， radiological examination， and related biomarkers can be used for the diagnosis of cognitive impairment. There are currently no specific drugs to reverse the progression of cognitive impairment in MMD， and cerebral revascularization is an effective treatment measure for cognitive impairment in MMD， while rehabilitation treatment and diet control also have a certain therapeutic effect. Clinicians need to enhance their understanding of such patients， and it is recommended to carry out the general screening of cognitive impairment in MMD patients in medical institutions with adequate resources， so as to achieve early identification， early diagnosis， and early treatment.