Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
Experimental Psychology, Helmholtz Institute, Utrecht University, Utrecht, The Netherlands.
J Neurol. 2021 Sep;268(9):3456-3466. doi: 10.1007/s00415-021-10516-7. Epub 2021 Mar 20.
The cerebellar cognitive affective syndrome scale (CCAS-S) was recently developed to detect specific neuropsychological deficits in patients with cerebellar diseases in an expedited manner.
To evaluate the discriminative ability of the CCAS-S in an etiologically homogeneous cohort of spinocerebellar ataxia type 3 (SCA3) patients and to examine relationships between cognitive deficits and motor symptom severity.
The CCAS-S was administered to twenty mildly to moderately affected SCA3 patients and eighteen healthy controls matched for age, sex, and educational level. Disease severity was measured by the Scale for the Assessment and Rating of Ataxia (SARA), Inventory of Non-Ataxia Signs (INAS), 8 m walk test, nine-hole peg test (9HPT), and Patient Health Questionnaire-9 (PHQ-9).
SCA3 patients had a lower total CCAS-S score (p < 0.001) and higher number of failed tests (p = 0.006) than healthy controls. Patients displayed impairments in semantic fluency, phonemic fluency, category switching, cube drawing, and affect regulation. Total CCAS-S score showed high discriminative ability (area under the curve [AUC]: 0.96) and was associated with disease duration, SARA score, walking speed, and dominant hand 9HPT performance. No correlations were observed with INAS count, repeat length, and PHQ-9 score. Discriminative capacity of the number of failed tests was moderate (AUC: 0.76).
Essentially all SCA3 patients exhibited some form of cognitive impairment. The CCAS-S differentiates SCA3 patients from healthy controls, detects neuropsychological deficits early in the disease course, and correlates with relevant ataxia severity measures.
小脑认知情感综合征量表（CCAS-S）最近被开发出来，用于快速检测小脑疾病患者的特定神经心理学缺陷。
在一个病因学同质的 SCA3 患者队列中评估 CCAS-S 的鉴别能力，并研究认知缺陷与运动症状严重程度之间的关系。
对 20 名轻度至中度 SCA3 患者和 18 名年龄、性别和教育程度相匹配的健康对照者进行 CCAS-S 评估。疾病严重程度通过共济失调评估量表（SARA）、非共济失调体征量表（INAS）、8 米步行测试、九孔钉测试（9HPT）和患者健康问卷-9（PHQ-9）进行测量。
SCA3 患者的 CCAS-S 总分较低（p<0.001），失败测试的数量较多（p=0.006）。患者在语义流畅性、语音流畅性、类别转换、立方体绘制和情感调节方面存在障碍。CCAS-S 总分具有较高的鉴别能力（曲线下面积[AUC]：0.96），与疾病持续时间、SARA 评分、行走速度和惯用手 9HPT 表现相关。与 INAS 计数、重复长度和 PHQ-9 评分无相关性。失败测试数量的鉴别能力为中等（AUC：0.76）。
所有 SCA3 患者均存在某种形式的认知障碍。CCAS-S 可将 SCA3 患者与健康对照者区分开来，在疾病早期检测到神经心理学缺陷，并与相关的共济失调严重程度测量指标相关。
