Rusz Jan, Klempíř Jiří, Tykalová Tereza, Baborová Eva, Čmejla Roman, Růžička Evžen, Roth Jan
Department of Circuit Theory, Faculty of Electrical Engineering, Czech Technical University in Prague, Technická 2, 160 00, Prague 6, Czech Republic,
J Neural Transm (Vienna). 2014 Dec;121(12):1529-39. doi: 10.1007/s00702-014-1229-8. Epub 2014 May 9.
Although motor speech impairment is a common manifestation of Huntington's disease (HD), its description remains limited. The aim of the current study was therefore to estimate the occurrence and characteristics of speech disorder in HD and to explore the influence of antipsychotic medication on speech performance. Speech samples, including reading passage and monologue, were acquired from 40 individuals diagnosed with HD and 40 age- and sex-matched healthy controls. Objective acoustic analyses were used to evaluate key aspects of speech including vowel articulation, intensity, pitch and timing. A predictive model was constructed to detect the occurrence and most prominent patterns of speech dysfunction in HD. We revealed that 93% of HD patients manifest some degree of speech impairment. Decreased number of pauses, slower articulation rate, imprecise vowel articulation and excess intensity variations were found to be the most salient patterns of speech dysfunction in HD. We further demonstrated that antipsychotic medication may induce excessive loudness and pitch variations perceptually resembling excess patterns of word stress, and may also accentuate general problems with speech timing. Additionally, antipsychotics induced a slight improvement of vowel articulation. Specific speech alterations observed in HD patients indicate that speech production may reflect the pathophysiology of the disease as well as treatment effects, and may therefore be considered a valuable marker of functional disability in HD.
尽管运动性言语障碍是亨廷顿舞蹈症（HD）的常见表现，但其描述仍然有限。因此，本研究的目的是评估HD患者言语障碍的发生率和特征，并探讨抗精神病药物对言语表现的影响。从40名被诊断为HD的个体以及40名年龄和性别匹配的健康对照者中采集了言语样本，包括朗读段落和独白。采用客观声学分析来评估言语的关键方面，包括元音清晰度、强度、音高和时间。构建了一个预测模型，以检测HD患者言语功能障碍的发生率和最突出模式。我们发现，93%的HD患者存在某种程度的言语障碍。停顿次数减少、发音速度减慢、元音清晰度不准确以及强度变化过大，被发现是HD患者言语功能障碍最显著的模式。我们进一步证明，抗精神病药物可能会在感知上诱发过度的响度和音高变化，类似于单词重音的过度模式，并且还可能加剧言语时间方面的一般问题。此外，抗精神病药物会使元音清晰度略有改善。在HD患者中观察到的特定言语改变表明，言语产生可能反映了疾病的病理生理学以及治疗效果，因此可能被视为HD患者功能残疾的一个有价值的指标。
