Mayeux R, Stern Y, Herman A, Greenbaum L, Fahn S
Ann Neurol. 1986 Dec;20(6):727-31. doi: 10.1002/ana.410200613.
Functional disability in Huntington's disease usually results from a combination of the movement disorder, intellectual decline, and psychopathological changes, but the unique contribution of each element has never been investigated. The Shoulson-Fahn functional capacity rating scale measures independence in such daily activities as eating, dressing, and managing personal finances, and is used to stage the illness and follow its progression. To determine which problems contribute most to reduced functional capacity as the disease evolves, we reviewed the records of 48 consecutive patients who were evaluated for intellectual and emotional status and motor disability. Each patient was staged and rated for functional capacity at the time of the examinations. Thirty-three of these patients were followed over several years with repeat evaluations at 6-month intervals. Intellectual impairment and depression correlated significantly with reduced functional capacity. However, when the somatic symptoms of depression were eliminated from the analysis, its relationship to functional capacity was no longer significant. Duration of illness, motor disability, and age at onset also had little impact. Neuropsychological test performance and functional capacity deteriorated over time. Our data suggest that intellectual impairment is a major factor in reducing functional capacity in the early stages of Huntington's disease.
亨廷顿舞蹈症的功能残疾通常是由运动障碍、智力衰退和精神病理变化共同导致的，但尚未对各因素的独特作用进行过研究。舒尔森-法恩功能能力评定量表用于衡量诸如进食、穿衣和管理个人财务等日常活动中的独立性，也用于对疾病进行分期并跟踪其进展。为了确定随着疾病进展，哪些问题对功能能力下降的影响最大，我们回顾了48例连续患者的记录，这些患者接受了智力、情绪状态和运动残疾评估。在检查时，对每位患者进行了分期和功能能力评定。其中33例患者在几年内每隔6个月接受一次重复评估。智力障碍和抑郁与功能能力下降显著相关。然而，当从分析中去除抑郁的躯体症状后，其与功能能力的关系不再显著。病程、运动残疾和发病年龄的影响也很小。神经心理测试表现和功能能力随时间而恶化。我们的数据表明，智力障碍是亨廷顿舞蹈症早期功能能力下降的主要因素。
