Josiassen R C, Curry L, Roemer R A, DeBease C, Mancall E L
J Clin Neuropsychol. 1982 Jul;4(2):173-83. doi: 10.1080/01688638208401127.
The study investigated patterns of intellectual deficit in 13 patients with recently diagnosed Huntington's disease (mean duration of 2.3 years) and 46 offspring "at risk" for the illness using the Wechsler Adult Intelligence Scale (WAIS). The results support the idea that certain cognitive skills remain relatively intact at early stages of the disease while other skills are impaired. A consistent pattern of impairment was observed in the patient group which included the Arithmetic, Digit Span, Digit Symbol, and Picture Arrangement subtests. The most striking result was a demonstration of significantly increased variability among the "at risk" population of the Digit Span and Picture Arrangement subtests. These findings suggest that the WAIS may be a useful adjunct to neurological diagnosis for longitudinal monitoring of intellectual changes even at very early stages of the illness.
该研究使用韦氏成人智力量表（WAIS），对13例近期诊断为亨廷顿舞蹈症（平均病程2.3年）的患者以及46名有患病“风险”的后代的智力缺陷模式进行了调查。结果支持了这样一种观点，即疾病早期某些认知技能相对保持完好，而其他技能则受损。在患者组中观察到一种一致的损伤模式，其中包括算术、数字广度、数字符号和图片排列分测验。最显著的结果是，在数字广度和图片排列分测验的“有风险”人群中，变异性显著增加。这些发现表明，即使在疾病的极早期阶段，WAIS对于纵向监测智力变化可能是神经学诊断的一种有用辅助工具。
