Innovation Center for Neurological Disorders, Department of Neurology, Xuanwu Hospital, Capital Medical University, 45 Changchun Street, Beijing, 100053, People's Republic of China.
Department of Neurology, The First Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, People's Republic of China.
Neurol Sci. 2021 Jun;42(6):2487-2495. doi: 10.1007/s10072-020-04864-x. Epub 2020 Nov 2.
This study aimed to analyze the characteristics of cognitive impairment in adult-onset neuronal intranuclear inclusion disease (NIID).
Seven patients with adult-onset NIID were collected consecutively from the memory clinic of Xuanwu hospital from February to December 2019. These cases were diagnosed with skin biopsy triggered by DWI high-intensity signals in corticomedullary junction on brain MRI. We used a battery of neuropsychological scales to detect the patient's performance in each cognitive domain, and made a detailed analysis on the characteristics of cognitive impairment.
All seven cases had cognitive impairment, and four of them had met the criteria for dementia. The scores of Montreal Cognitive Assessment and Frontal Assessment Battery were abnormal in all patients. The executive dysfunction was confirmed by the abnormal scores of Trail Making Test (5/7, 71%) and Clock Drawing Test (4/7, 57%). Bad performance in Auditory Verbal Learning Test (6/7, 86%) demonstrated that the memory was also a very commonly impaired cognitive domain. The low score on the animal fluency (4/7, 57%), Boston Naming Test (3/7, 43%), and Pentagon and Cube Copying Test (4/7, 57%) indicated that the language and visuospatial skills were also impaired. Fazekas scores were significantly correlated to the global cognition, executive and language functions (r = 0.788-0.906, P < 0.05).
There is obvious impairment in multiple cognitive domains in adult-onset NIID, and both the executive dysfunction and memory deficit are very common. Leukoencephalopathy may be the main course of cognitive impairment in adult-onset NIID.
本研究旨在分析成人发病神经元核内包涵体病（NIID）患者认知障碍的特点。
连续收集 2019 年 2 月至 12 月宣武医院记忆门诊成人发病 NIID 患者 7 例。这些病例均由脑 MRI 皮质-髓质交界处 DWI 高信号触发的皮肤活检诊断。我们使用一系列神经心理学量表来检测患者在每个认知域中的表现，并对认知障碍的特点进行详细分析。
7 例患者均存在认知障碍，其中 4 例符合痴呆标准。所有患者的蒙特利尔认知评估和额叶评估量表评分均异常。轨迹生成测试（5/7，71%）和时钟绘制测试（4/7，57%）异常评分证实了执行功能障碍。听觉词语学习测试（6/7，86%）的较差表现表明记忆也是一个非常常见的受损认知域。动物流畅性（4/7，57%）、波士顿命名测试（3/7，43%）和五角和立方复制测试（4/7，57%）得分较低表明语言和视空间技能也受损。Fazekas 评分与整体认知、执行和语言功能显著相关（r = 0.788-0.906，P < 0.05）。
成人发病 NIID 存在多个认知域的明显障碍，执行功能障碍和记忆缺陷都很常见。脑白质病变可能是成人发病 NIID 认知障碍的主要发病机制。
