Department of Neurodegeneration, Hertie Institute for Clinical Brain Research, Germany and Center for Neurology, University of Tübingen, University Hospital Tübingen, Tübingen, Germany.
Centre for Neuroscience of Speech, The University of Melbourne, 550 Swanston Street, Parkville, Melbourne, VIC, 3010, Australia.
J Neurol. 2019 May;266(5):1260-1266. doi: 10.1007/s00415-019-09258-4. Epub 2019 Mar 6.
We aimed to provide proof-of-principle evidence that intensive home-based speech treatment can improve dysarthria in complex multisystemic degenerative ataxias, exemplified by autosomal recessive spastic ataxia Charlevoix-Saguenay (ARSACS). Feasibility and piloting efficacy of speech training specifically tailored to cerebellar dysarthria was examined through a 4-week program in seven patients with rater-blinded assessment of intelligibility (primary outcome) and naturalness and acoustic measures of speech (secondary outcomes) performed 4 weeks before, immediately prior to, and directly after training (intraindividual control design). Speech intelligibility and naturalness improved post treatment. This provides piloting evidence that ataxia-tailored speech treatment might be effective in degenerative cerebellar disease.
我们旨在提供初步证据，证明强化家庭语言治疗可以改善以常染色体隐性痉挛性共济失调 Charlevoix-Saguenay（ARSACS）为代表的复杂多系统退行性共济失调的构音障碍。通过对 7 名患者进行为期 4 周的语言训练计划，专门针对小脑性构音障碍进行评估，检查了针对特定于小脑性构音障碍的语言训练的可行性和初步疗效，评估人员对清晰度（主要结果）和自然度以及言语的声学测量（次要结果）进行盲法评估，该评估在治疗前 4 周、治疗前即刻和治疗后即刻进行（个体内对照设计）。治疗后言语清晰度和自然度提高。这为针对共济失调的语言治疗可能对退行性小脑疾病有效的假说提供了初步证据。
