Metzler-Baddeley Claudia, Cantera Jaime, Coulthard Elizabeth, Rosser Anne, Jones Derek K, Baddeley Roland J
Cardiff University Brain Research Imaging Centre (CUBRIC), School of Psychology, Cardiff University, Cardiff, UK Neuroscience and Mental Health Research Institute, Cardiff University, Cardiff, UK.
Music Factory, Bristol University, Bristol, UK.
J Huntingtons Dis. 2014;3(3):273-83. doi: 10.3233/JHD-140113.
Huntington's disease (HD) is an autosominal dominant neurodegenerative condition that leads to progressive loss of motor and cognitive functions. Early symptoms in HD include subtle executive dysfunction related to white and grey matter loss in cortico-striatal-thalamic loops. There is no cure for HD and hence a significant need for early intervention with the potential to delay the clinical onset of the disease.
The objective of the present pilot study was to devise a novel behavioural intervention involving drumming and rhythm exercises that targets early dysexecutive problems, such as difficulties in sequence and reversal learning, response speed, timing, and dual tasking.
One preclinical person and nine people with early to advanced stages of HD were recruited of whom five completed the two months intervention. The effects of rhythm exercise on executive function, basal ganglia volume, and white matter microstructure in the anterior corpus callosum, the anterior thalamic radiation, and the cortico-spinal tract were assessed post- relative to pre-training.
After two months training, improvements in executive function and changes in white matter microstructure, notably in the genu of the corpus callosum that connects prefrontal cortices of both hemispheres, were observed. No changes in basal ganglia volume were present.
This pilot study provides novel preliminary evidence that carefully targeted behavioural stimulation in HD can result in cognitive enhancement and improvements in callosal white matter microstructure.
亨廷顿舞蹈症（HD）是一种常染色体显性神经退行性疾病，会导致运动和认知功能逐渐丧失。HD的早期症状包括与皮质-纹状体-丘脑环路中白质和灰质损失相关的细微执行功能障碍。目前尚无治愈HD的方法，因此迫切需要进行早期干预，以延缓疾病的临床发作。
本初步研究的目的是设计一种新颖的行为干预方法，即击鼓和节奏练习，以针对早期执行功能障碍问题，如序列和逆向学习困难、反应速度、时间控制和双重任务处理困难。
招募了一名临床前个体和九名处于HD早期至晚期阶段的患者，其中五人完成了为期两个月的干预。在训练后相对于训练前，评估了节奏练习对执行功能、基底神经节体积以及胼胝体前部、丘脑前辐射和皮质脊髓束的白质微观结构的影响。
经过两个月的训练，观察到执行功能有所改善，白质微观结构发生了变化，特别是在连接两侧半球前额叶皮质的胼胝体膝部。基底神经节体积没有变化。
这项初步研究提供了新的初步证据，表明对HD进行精心设计的行为刺激可以提高认知能力，并改善胼胝体白质微观结构。
