Günther Peter Albrecht, Kühn Hans-Juergen, Villmann Thomas, Hermann Wieland
Clinic of Neurology, University of Leipzig.
Ann Indian Acad Neurol. 2009 Jan;12(1):28-34. doi: 10.4103/0972-2327.48849.
Fine motor skills disorders belong to the neurological manifestation of Wilson's disease. The aim of this study is to investigate if fine motor performance changes during the course of the disease and with therapy.
In 15 neurological patients with Wilson's disease, severity of neurological symptoms was assessed with a neurology score. A test battery consisting of the hand writing of a test sentence, lines of "double-I" and retracing a circle was carried out for analysis. By means of a computer-aided analysis of the patient's handwriting, 10 kinematic parameters of the writing trace were calculated. These parameters were determined once at the very beginning of the study and then again after 7 years.
Improvement of clinical symptoms was observed after onset of therapy only within the first 2 years. In contrast to the standard population, a reduced degree of automation could be detected both at the beginning and at the end of the 7-year interval. There was no significant change in 8 out of the 10 kinematic parameters during the observation period, 2 deteriorated.
The absence of a significant increase in fine motor disturbances proves, on the one hand, the efficacy of the therapy regime applied. On the other hand, the end point of a possible reversibility had been reached. A computer-aided analysis of the patient's handwriting allows for a sensitive detection of the "functional scar" in the extrapyramidal control and can subsequently prompt a timely correction of therapy in case of progression.
精细运动技能障碍属于威尔逊病的神经学表现。本研究旨在调查在疾病过程中以及治疗期间精细运动表现是否发生变化。
对15例患有威尔逊病的神经科患者，用神经学评分评估神经症状的严重程度。进行了一组测试，包括书写一个测试句子、画“双I”线和画圆并进行回溯，以进行分析。通过对患者笔迹的计算机辅助分析，计算出笔迹轨迹的10个运动学参数。这些参数在研究开始时测定一次，然后在7年后再次测定。
仅在治疗开始后的头2年内观察到临床症状有所改善。与正常人群相比，在7年期间开始和结束时均检测到自动化程度降低。在观察期内，10个运动学参数中有8个没有显著变化，2个恶化。
精细运动障碍没有显著增加，一方面证明了所应用治疗方案的有效性。另一方面，可能的可逆性终点已经达到。对患者笔迹的计算机辅助分析能够敏感地检测锥体外系控制中的“功能瘢痕”，并随后在病情进展时促使及时调整治疗。
