Feigin A, Kieburtz K, Bordwell K, Como P, Steinberg K, Sotack J, Zimmerman C, Hickey C, Orme C, Shoulson I
Department of Neurology, University of Rochester Medical Center, NY 14642-8673, USA.
Mov Disord. 1995 Mar;10(2):211-4. doi: 10.1002/mds.870100213.
We prospectively evaluated 129 patients with manifest Huntington's disease (HD) to determine the rate of illness progression and the clinical features that correlate with functional decline. A single examiner evaluated each patient using the HD Functional Capacity Scale. Standardized motor performance was also assessed in 94 of the patients (73%) using the HD Rating Scale. Total Functional Capacity declined at a rate of 0.63 +/- 0.75 U per year. As functional capacity worsened, chorea lessened, and dystonia intensified. There was no correlation between rate of functional decline and age at onset of HD, body weight, gender of affected parent, or history of neuroleptic use.
我们对129例显性亨廷顿舞蹈症（HD）患者进行了前瞻性评估，以确定疾病进展速度以及与功能衰退相关的临床特征。由一名检查者使用HD功能能力量表对每位患者进行评估。还使用HD评定量表对94例患者（73%）的标准化运动表现进行了评估。总功能能力以每年0.63±0.75单位的速度下降。随着功能能力恶化，舞蹈症减轻，肌张力障碍加剧。功能衰退速度与HD发病年龄、体重、患病父母的性别或使用抗精神病药物史之间无相关性。
