Speech deterioration in amyotrophic lateral sclerosis (ALS) after manifestation of bulbar symptoms
Speech deterioration in amyotrophic lateral sclerosis (ALS) after manifestation of bulbar symptoms
复制标题
DOI：
10.1111/1460-6984.12357
复制
发表时间：
2018-03-01
影响因子：
2.4
通讯作者：
Palmio, Johanna
中科院分区：
文献类型：
--
作者：
Makkonen, Tanja;Ruottinen, Hanna;Palmio, Johanna
背景肌萎缩性侧索硬化症（ALS）的症状及其进展通常在确诊后进行研究。然而，许多ALS患者在诊断时已经有严重的构音障碍和语言障碍。针对渐冻症患者的交际需要，应及时进行有针对性的语言治疗干预。目的研究ALS患者的自然语言功能能维持多久，并确定其语言的变化。方法与程序对30例连续受试者进行研究，并根据ALS的初始类型分为两组，分别是球型和脊髓型。在2年的随访中对其言语障碍的严重程度、发音率和可理解性进行评估。结果和结果：60%的参与者的说话能力恶化到较差，需要辅助和替代沟通（AAC）方法。他们的语言能力在出现第一个球症状后的18个月内保持正常。在研究期间，几乎所有参与者的严重程度、发音率和可理解性都有所下降。一开始，语言能力的下降在球组中比在脊柱组中更严重，这种差异在整个随访过程中一直存在，但有一些例外。结论与意义：与ALS诊断或首次语言治疗评估相比，球部症状的出现预示着失语时间的缩短。在临床工作中，在确定AAC措施的紧迫性时，考虑ALS的初始类型是很重要的，因为球源性ALS患者更容易延迟评估和AAC干预。
BackgroundThe symptoms and their progression in amyotrophic lateral sclerosis (ALS) are typically studied after the diagnosis has been confirmed. However, many people with ALS already have severe dysarthria and loss of adequate speech at the time of diagnosis. Speech-and-language therapy interventions should be targeted timely based on communicative need in ALS.AimsTo investigate how long natural speech will remain functional and to identify the changes in the speech of persons with ALS.Methods & ProceduresAltogether 30 consecutive participants were studied and divided into two groups based on the initial type of ALS, bulbar or spinal. Their speech disorder was evaluated on severity, articulation rate and intelligibility during the 2-year follow-up.Outcome & ResultsThe ability to speak deteriorated to poor and necessitated augmentative and alternative communication (AAC) methods with 60% of the participants. Their speech remained adequate on average for 18 months from the first bulbar symptom. Severity, articulation rate and intelligibility declined with nearly all participants during the study. To begin with speech deteriorated more in the bulbar group than in the spinal group and the difference remained during the whole follow-up with some exceptions.Conclusions & ImplicationsThe onset of bulbar symptoms indicated the time to loss of speech better than when assessed from ALS diagnosis or the first speech therapy evaluation. In clinical work, it is important to take the initial type of ALS into consideration when determining the urgency of AAC measures as people with bulbar-onset ALS are more susceptible to delayed evaluation and AAC intervention.