Andrich Jürgen, Saft Carsten, Ostholt Natalie, Müller Thomas
Department of Neurology, St. Josef Hospital, Ruhr University Bochum, Gudrunstrasse 56, 44791 Bochum, Germany.
Neurosci Lett. 2007 Apr 18;416(3):272-4. doi: 10.1016/j.neulet.2007.02.027. Epub 2007 Feb 11.
Instrumental measurement of complex motion sequences with a peg insertion paradigm reflects impairment in patients with Huntington's disease (HD). Objectives were to study progress of HD symptoms and peg insertion results in 39 HD patients without symptomatic drug treatment over an interval of 3 years. Assessments were at baseline and 3 years later. Unified Huntington's Disease Rating Scale (UHDRS) total score, computed arm score and the specific rating outcomes for bradykinesia, chorea, dystonia and oculomotor symptoms significantly increased over a 3-year period. Motor test outcomes significantly worsened. Cognitive scores did not change significantly, but were significantly related to the peg insertion outcomes and to the various UHDRS rating results at both assessments. Peg insertion scores are nonspecific diagnostic marker for progress in HD. Peg insertion particularly reflects motor impairment and additionally higher cognitive and executive dysfunction. These higher cognitive functions are associated with frontostriatal pathology in HD. Our study results suggest that our motor test reflects these emerging deficits of higher cognitive and motor function abilities in HD. We conclude, that instrumental assessment of complex movement sequences is an additional simple method to follow impairment in HD patients in addition to clinical rating.
采用插栓范式对复杂运动序列进行仪器测量可反映亨廷顿舞蹈病(HD)患者的功能损害。本研究旨在对39例未接受症状性药物治疗的HD患者进行为期3年的随访,观察HD症状进展及插栓测试结果。评估在基线期和3年后进行。统一亨廷顿舞蹈病评定量表(UHDRS)总分、计算得出的手臂评分以及运动迟缓、舞蹈症、肌张力障碍和动眼神经症状的具体评定结果在3年期间显著增加。运动测试结果显著恶化。认知评分无显著变化,但在两次评估中均与插栓测试结果及UHDRS各项评定结果显著相关。插栓测试评分是HD病情进展的非特异性诊断指标。插栓测试特别反映运动功能损害,此外还反映较高水平的认知和执行功能障碍。这些较高水平的认知功能与HD患者的额纹状体病变有关。我们的研究结果表明,我们的运动测试反映了HD患者这些新出现的较高认知和运动功能能力缺陷。我们得出结论,除临床评定外,对复杂运动序列进行仪器评估是跟踪HD患者功能损害的另一种简单方法。