Parkinson and Movement Disorders Unit, IRCCS Foundation Carlo Besta Neurological Institute, Via Celoria 11, 20133, Milan, Italy.
Parkinson Institute, ASST Gaetano Pini-CTO, Via Bignami 1, 20126, Milan, Italy.
Neurol Sci. 2022 Jun;43(6):3695-3701. doi: 10.1007/s10072-021-05787-x. Epub 2022 Jan 24.
Apathy is the commonest psychiatric manifestation in Huntington's disease (HD). We investigated negative psychiatric symptoms-as determined by the Scale for the Assessment of Negative Psychiatric Symptoms (SANS)-in early and intermediate HD patients, hypothesizing that such symptoms would be prominent and constitute a more comprehensive and clinically relevant assessment than apathy alone. We also assessed relations between negative symptoms and disease stage, mood, motor, and cognitive disturbances.
Thirty-five stage 1 and twenty-nine stage 2 consecutive adult HD outpatients were administered SANS; the Scale for the Assessment of Positive Psychiatric Symptoms (SAPS); the motor section of the Unified Huntington's Disease Rating Scale (UHDRS); Total Functional Capacity (TFC); and instruments to assess cognition, anxiety, and depression.
The groups had similar age, education, and CAG length. Scores on the Hamilton depression and anxiety scales, and SAPS were similar. Negative symptoms were pervasive in the entire series. Illness duration, UHDRS, TFC, cognition, and SANS scores were significantly worse in stage 2. Mini Mental State Examination (MMSE) and SAPS scores were significantly (multiple regression) associated with SANS score, while Hamilton depression and UHDRS scores were not. SANS score was also associated with stage after removing the cognition-related domains of alogia and attention.
Negative symptoms are pervasive in HD but more severe in stage 2. The associations of SANS with MMSE and SAPS suggest impaired cognition and thinking as important in generating negative symptoms. SANS appears useful for revealing a wide range of negative symptoms in HD.
淡漠是亨廷顿病（HD）最常见的精神表现。我们研究了早期和中期 HD 患者的阴性精神病症状（由阴性精神病症状评定量表[SANS]确定），假设这些症状会突出并构成比单纯淡漠更全面和更具临床相关性的评估。我们还评估了阴性症状与疾病阶段、情绪、运动和认知障碍之间的关系。
对 35 名 1 期和 29 名 2 期连续成年 HD 门诊患者进行 SANS；阳性精神病症状评定量表（SAPS）；统一亨廷顿病评定量表（UHDRS）的运动部分；总功能能力（TFC）；以及评估认知、焦虑和抑郁的工具。
两组的年龄、教育程度和 CAG 长度相似。汉密尔顿抑郁和焦虑量表以及 SAPS 的评分相似。阴性症状在整个系列中普遍存在。疾病持续时间、UHDRS、TFC、认知和 SANS 评分在 2 期明显更差。简易精神状态检查（MMSE）和 SAPS 评分与 SANS 评分显著相关（多元回归），而汉密尔顿抑郁和 UHDRS 评分则不相关。在去除语言和注意力等认知相关领域后，SANS 评分也与阶段相关。
阴性症状在 HD 中普遍存在，但在 2 期更为严重。SANS 与 MMSE 和 SAPS 的关联表明认知和思维受损在产生阴性症状方面很重要。SANS 似乎有助于揭示 HD 中广泛的阴性症状。
