Hamilton J M, Salmon D P, Corey-Bloom J, Gamst A, Paulsen J S, Jerkins S, Jacobson M W, Peavy G
Department of Neurosciences, University of California-San Diego, 9500 Gilman Drive, MC 0948, La Jolla, CA, USA.
J Neurol Neurosurg Psychiatry. 2003 Jan;74(1):120-2. doi: 10.1136/jnnp.74.1.120.
The independent and relative contributions of motor, cognitive, and behavioural deficits to functional decline in patients with Huntington's disease are examined. Twenty two patients with Huntington's disease were assessed with rating scales for motor dysfunction, cognitive measures of executive functions, and behavioural measures of apathy, executive dysfunction, and disinhibition. Their functional status was assessed with informant based and clinician based ratings of activities of daily living (ADL). A composite apathy/executive dysfunction behavioural index was strongly related to decline in ADL independently and after controlling for motor and cognitive deficits. These results suggest that behavioural dysfunction contributes to functional decline in patients with Huntington's disease and may impede their ability to utilise motor or cognitive skills that remain available in the early stages of the disease.
研究了运动、认知和行为缺陷对亨廷顿舞蹈症患者功能衰退的独立及相对影响。对22名亨廷顿舞蹈症患者进行了运动功能障碍评定量表、执行功能的认知测量以及冷漠、执行功能障碍和去抑制的行为测量。通过基于 informant 和临床医生的日常生活活动（ADL）评分来评估他们的功能状态。一个综合的冷漠/执行功能障碍行为指数在独立以及控制运动和认知缺陷后，与ADL的衰退密切相关。这些结果表明，行为功能障碍导致了亨廷顿舞蹈症患者的功能衰退，并可能阻碍他们利用疾病早期仍具备的运动或认知技能的能力。
