DeDios-Stern Samantha L, Gotra Milena Y, Resch Zachary J, Jennette Kyle J, Amin-Hanjani Sepideh, Charbel Fady T, Alaraj Ali, Testai Fernando D, Thulborn Keith R, Vargas Alejandro, Pliskin Neil H, Soble Jason R
Department of Neurology, National Hospital for Neurology and Neurosurgery, London , UK.
Department of Neuro Rehabilitation, Chelsea and Westminster Hospital NHS London Trust, London , UK.
Neurosurgery. 2024 Dec 1;95(6):1338-1348. doi: 10.1227/neu.0000000000003010. Epub 2024 Jun 5.
Moyamoya disease (MMD) is a rare noninflammatory disorder involving progressive intracranial vasculopathy and impaired cerebral blood flow in the anterior circulation, resulting in stroke and cognitive impairment. We aimed to characterize cognitive impairment and the possible predictive value of sociodemographic and clinical characteristics of adults with MMD.
This cross-sectional study examined neurocognitive performance in a group of 42 consecutive adult patients (mean age = 40.52 years; 69% female) referred for a presurgical neuropsychological evaluation. Neuropsychological functioning was assessed with a comprehensive battery, and cognitive dysfunction was defined as 1.5 SDs below the mean. Neurocognitive performance correlated with clinical/demographic characteristics and disease markers.
Most patients (91%) had a history of stroke, and 45% had cognitive deficits, most notably on measures of attention/speed (48%), executive functioning (47%), visuoconstruction (41%), and memory (31%-54%). Only higher educational attainment and poor collateral blood flow in the right hemisphere differentiated cognitively impaired (n = 19) and intact groups (n = 23), and MMD-related characteristics (eg, disease duration, stroke history) did not differentiate the 2 groups.
Consistent with previous work, frontal-subcortical cognitive deficits (eg, deficits in mental speed, attention, executive functioning) were found in nearly half of patients with MMD and better cognitive performance was associated with factors related to cognitive reserve. Angiographic metrics of disease burden (eg, Suzuki rating, collateral flow) and hemodynamic reserve were not consistently associated with poorer cognitive outcomes, suggesting that cognition is a crucial independent factor to assess in MMD and has relevance for treatment planning and functional status.
烟雾病（MMD）是一种罕见的非炎性疾病，涉及颅内血管病变的进展以及前循环脑血流受损，导致中风和认知障碍。我们旨在描述烟雾病成年患者的认知障碍情况以及社会人口学和临床特征的可能预测价值。
这项横断面研究检查了一组连续的42例成年患者（平均年龄 = 40.52岁；69%为女性）的神经认知表现，这些患者因术前神经心理学评估前来就诊。使用一套综合测试评估神经心理功能，认知功能障碍定义为低于平均值1.5个标准差。神经认知表现与临床/人口学特征及疾病标志物相关。
大多数患者（91%）有中风病史，45%有认知缺陷，最明显的是在注意力/速度（48%）、执行功能（47%）、视觉构建（41%）和记忆（31%-54%）方面的测试中。只有较高的教育程度和右侧半球侧支血流不良能区分认知受损组（n = 19）和未受损组（n = 23），而与烟雾病相关的特征（如病程、中风病史）并不能区分这两组。
与先前的研究一致，在近一半的烟雾病患者中发现了额叶 - 皮质下认知缺陷（如思维速度、注意力、执行功能方面的缺陷），较好的认知表现与认知储备相关因素有关。疾病负担的血管造影指标（如铃木分级、侧支血流）和血流动力学储备与较差的认知结果并非始终相关，这表明认知是烟雾病评估中一个关键的独立因素，对治疗计划和功能状态具有重要意义。
