Cerebellar cognitive affective syndrome (CCAS; Schmahmann’s syndrome) is characterized by deficits in executive function, linguistic processing, spatial cognition, and affect regulation. Diagnosis currently relies on detailed neuropsychological testing. The aim of this study was to develop an office or bedside cognitive screen to help identify CCAS in cerebellar patients. Secondary objectives were to evaluate whether available brief tests of mental function detect cognitive impairment in cerebellar patients, whether cognitive performance is different in patients with isolated cerebellar lesions versus complex cerebrocerebellar pathology, and whether there are cognitive deficits that should raise red flags about extra-cerebellar pathology. Comprehensive standard neuropsychological tests, experimental measures and clinical rating scales were administered to 77 patients with cerebellar disease—36 isolated cerebellar degeneration or injury, and 41 complex cerebrocerebellar pathology—and to healthy matched controls. Tests that differentiated patients from controls were used to develop a screening instrument that includes the cardinal elements of CCAS. We validated this new scale in a new cohort of 39 cerebellar patients and 55 healthy controls. We confirm the defining features of CCAS using neuropsychological measures. Deficits in executive function were most pronounced for working memory, mental flexibility, and abstract reasoning. Language deficits included verb for noun generation and phonemic > semantic fluency. Visual spatial function was degraded in performance and interpretation of visual stimuli. Neuropsychiatric features included impairments in attentional control, emotional control, psychosis spectrum disorders and social skill set. From these results, we derived a 10-item scale providing total raw score, cut-offs for each test, and pass/fail criteria that determined ‘possible’ (one test failed), ‘probable’ (two tests failed), and ‘definite’ CCAS (three tests failed). When applied to the exploratory cohort, and administered to the validation cohort, the CCAS/Schmahmann scale identified sensitivity and selectivity, respectively as possible exploratory cohort: 85%/74%, validation cohort: 95%/78%; probable exploratory cohort: 58%/94%, validation cohort: 82%/93%; and definite exploratory cohort: 48%/100%, validation cohort: 46%/100%. In patients in the exploratory cohort, Mini-Mental State Examination and Montreal Cognitive Assessment scores were within normal range. Complex cerebrocerebellar disease patients were impaired on similarities in comparison to isolated cerebellar disease. Inability to recall words from multiple choice occurred only in patients with extra-cerebellar disease. The CCAS/Schmahmann syndrome scale is useful for expedited clinical assessment of CCAS in patients with cerebellar disorders.

中文翻译：

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小脑认知情感/ Schmahmann综合征量表

小脑认知情感综合症（CCAS； Schmahmann综合征）的特征是执行功能，语言处理，空间认知和影响调节方面的缺陷。目前的诊断依赖于详细的神经心理学测试。这项研究的目的是开发一个办公室或床边的认知筛查系统，以帮助鉴定小脑患者的CCAS。次要目标是评估可用的简短脑力测试是否能检测出小脑患者的认知功能障碍，孤立性小脑病变与复杂性小脑病变的患者的认知表现是否不同以及是否存在认知缺陷会引起小脑外危险信号病理。全面的标准神经心理学测试，对77例小脑疾病患者（36例孤立性小脑变性或损伤和41例复杂的小脑小脑病理）进行了实验测量和临床评定量表，并采用了健康的对照。将患者与对照区分开来的测试被用于开发包括CCAS基本要素的筛查仪器。我们在39名小脑患者和55名健康对照的新队列中验证了这种新的量表。我们使用神经心理学方法确认了CCAS的定义特征。执行功能的缺陷主要表现为工作记忆，心理灵活性和抽象推理。语言缺陷包括名词生成动词和语音>语义流利度。视觉空间功能的性能和视觉刺激的解释下降。神经精神病学特征包括注意力控制，情绪控制，精神病谱障碍和社交技能受损。从这些结果中，我们得出了10个项目的量表，提供了总的原始分数，每个测试的分界线以及确定“可能”（一个测试失败），“可能”（两个测试失败）和“确定” CCAS（三项测试均未通过）。当将CCAS / Schmahmann量表用于探索性队列并应用于验证队列时，其灵敏度和选择性分别确定为可能的探索性队列：85％/ 74％，验证队列：95％/ 78％；探索性队列：58％/ 94％，验证队列：82％/ 93％；明确的探索性队列：48％/ 100％，验证队列：46％/ 100％。在探索性队列中的患者中，迷你精神状态考试和蒙特利尔认知评估得分均在正常范围内。与孤立的小脑疾病相比，复杂的小脑脑疾病患者的相似性受损。仅在患有小脑外疾病的患者中无法回忆起多项选择中的单词。CCAS / Schmahmann综合征量表可用于加速小脑疾病患者的CCAS临床评估。