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首页> 外文期刊>Neuropsychologia >Speech acoustic markers of early stage and prodromal Huntington's disease: A marker of disease onset?
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Speech acoustic markers of early stage and prodromal Huntington's disease: A marker of disease onset?

机译：早期和前驱性亨廷顿舞蹈病的语音声学标记：疾病发作的标记？

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Speech disturbances (e.g., altered prosody) have been described in symptomatic Huntington's Disease (HD) individuals, however, the extent to which speech changes in gene positive pre-manifest (PreHD) individuals is largely unknown. The speech of individuals carrying the mutant HTT gene is a behavioural/motor/cognitive marker demonstrating some potential as an objective indicator of early HD onset and disease progression. Speech samples were acquired from 30 individuals carrying the mutant HTT gene (13 PreHD, 17 early stage HD) and 15 matched controls. Participants read a passage, produced a monologue and said the days of the week. Data were analysed acoustically for measures of timing, frequency and intensity. There was a clear effect of group across most acoustic measures, so that speech performance differed in-line with disease progression. Comparisons across groups revealed significant differences between the control and the early stage HD group on measures of timing (e.g., speech rate). Participants carrying the mutant HTT gene presented with slower rates of speech, took longer to say words and produced greater silences between and within words compared to healthy controls. Importantly, speech rate showed a significant correlation to burden of disease scores. The speech of early stage HD differed significantly from controls. The speech of PreHD, although not reaching significance, tended to lie between the performance of controls and early stage HD. This suggests that changes in speech production appear to be developing prior to diagnosis.
机译：在有症状的亨廷顿舞蹈病（HD）个体中已经描述了语言障碍（例如，韵律改变），但是，基因预表达阳性（PreHD）个体中语音变化的程度在很大程度上尚不清楚。携带突变型HTT基因的个体的言语是行为/运动/认知标记，显示出作为早期HD发作和疾病进展的客观指标的某些潜力。从携带突变型HTT基因（13个PreHD，17个早期HD）和15个匹配对照的30位个体中获取语音样本。参加者阅读了一段话，发表了独白并说了星期几。对数据进行声学分析，以测量时间，频率和强度。在大多数声学测量中，该组都有明显的效果，因此语音表现随疾病进展而变化。各组之间的比较显示，对照组和早期高清组在计时（例如，语速）方面存在显着差异。与健康对照相比，携带突变型HTT基因的参与者讲话速度较慢，说话时间更长，单词之间和单词内的沉默更大。重要的是，语速显示出与疾病评分负担显着相关。早期高清的语音与对照组的语音有很大不同。 PreHD的语音虽然没有达到重要的意义，但往往介于控件的性能和早期HD之间。这表明在诊断之前，语音产生的变化似乎正在发展。

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