大脑小脑认知情感/施马曼综合征在脊脑小脑缩症中的规模
Louisa P Selvadurai1, Susan L Perlman2, Tetsuo Ashizawa3
1Department of Neurology, Ataxia Center, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Massachusetts General Hospital and Harvard Medical School, 100 Cambridge Street, Suite 2000, Boston, MA, 02114, USA.
Cerebellum (London, England)
|January 2, 2024
概括
大脑认知情感/施马曼综合征 (CCAS) 影响46%的脊髓大脑动症 (SCA) 患者,影响执行功能和语言. 该CCAS/Schmahmann尺度 (CCAS-S) 有效地识别了SCA中的CCAS,与运动性动力衰竭的严重程度相关.
科学领域:
- 神经科学是一个神经科学.
- 临床神经学 临床神经学
- 遗传学 是一个遗传学.
背景情况:
- 大脑小脑认知情感/施马曼综合征 (CCAS) 涉及执行控制,语言,视觉空间技能以及影响调节方面的缺陷.
- 虽然CCAS在脊髓小脑动症 (SCAs) 中被认可,但其患病率和CCAS/Schmahmann尺度 (CCAS-S) 的实用性仍未得到充分研究.
研究的目的:
- 通过使用CCAS-S来确定SCAS患者中CCAS的患病率.
- 评估CCAS-S在识别CCAS中的敏感性和选择性.
- 探索CCAS-S性能与SCA中的临床/遗传因素之间的相关性.
主要方法:
- 对309名有症状的SCA患者 (SCA1-8),26名症状前患者和37名对照患者的CCAS-S结果的分析.
- 在症状,预症状和对照组之间以及不同类型的SCA之间比较CCAS-S得分.
- CCAS-S表现与年龄,教育,遗传重复时间,发病年龄,疾病持续时间,运动性衰退,抑郁和疲劳的相关性.
主要成果:
- 确定的CCAS在46%的有症状的SCA个体中被确定,对照组中的虚假阳性率为5.4%.
- 症状患者的CCAS-S表现低于对照组,特别是在执行功能和语言领域.
- CCAS-S评分显示,与运动性动力衰竭严重程度的相关性最强.
结论:
- CCAS-S是识别SCA患者中CCAS高患病率的一个有价值的工具.
- CCAS代表了SCA的一个显著的临床特征,支持其作为ATAXIOLOGY的核心方面的作用.
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