言语改变可预测孤立性快速眼动睡眠行为障碍的表型转化：一项多中心研究

Spoken Language Alterations can Predict Phenoconversion in Isolated Rapid Eye Movement Sleep Behavior Disorder: A Multicenter Study.

作者信息

Šubert Martin, Novotný Michal, Tykalová Tereza, Hlavnička Jan, Dušek Petr, Růžička Evžen, Škrabal Dominik, Pelletier Amelie, Postuma Ronald B, Montplaisir Jacques, Gagnon Jean-François, Galbiati Andrea, Ferini-Strambi Luigi, Marelli Sara, St Louis Erik K, Timm Paul C, Teigen Luke N, Janzen Annette, Oertel Wolfgang, Heim Beatrice, Holzknecht Evi, Stefani Ambra, Högl Birgit, Dauvilliers Yves, Evangelista Elisa, Šonka Karel, Rusz Jan

机构信息

Department of Circuit Theory, Faculty of Electrical Engineering, Czech Technical University in Prague, Prague, Czech Republic.

Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic.

出版信息

Ann Neurol. 2024 Mar;95(3):530-543. doi: 10.1002/ana.26835. Epub 2023 Dec 9.

DOI:10.1002/ana.26835

PMID:37997483

Abstract

OBJECTIVE

This study assessed the relationship between speech and language impairment and outcome in a multicenter cohort of isolated/idiopathic rapid eye movement (REM) sleep behavior disorder (iRBD).

METHODS

Patients with iRBD from 7 centers speaking Czech, English, German, French, and Italian languages underwent a detailed speech assessment at baseline. Story-tale narratives were transcribed and linguistically annotated using fully automated methods based on automatic speech recognition and natural language processing algorithms, leading to the 3 distinctive linguistic and 2 acoustic patterns of language deterioration and associated composite indexes of their overall severity. Patients were then prospectively followed and received assessments for parkinsonism or dementia during follow-up. The Cox proportional hazard was performed to evaluate the predictive value of language patterns for phenoconversion over a follow-up period of 5 years.

RESULTS

Of 180 patients free of parkinsonism or dementia, 156 provided follow-up information. After a mean follow-up of 2.7 years, 42 (26.9%) patients developed neurodegenerative disease. Patients with higher severity of linguistic abnormalities (hazard ratio [HR = 2.35]) and acoustic abnormalities (HR = 1.92) were more likely to develop a defined neurodegenerative disease, with converters having lower content richness (HR = 1.74), slower articulation rate (HR = 1.58), and prolonged pauses (HR = 1.46). Dementia-first (n = 16) and parkinsonism-first with mild cognitive impairment (n = 9) converters had higher severity of linguistic abnormalities than parkinsonism-first with normal cognition converters (n = 17).

INTERPRETATION

Automated language analysis might provide a predictor of phenoconversion from iRBD into synucleinopathy subtypes with cognitive impairment, and thus can be used to stratify patients for neuroprotective trials. ANN NEUROL 2024;95:530-543.

摘要

目的

本研究评估了多中心孤立性/特发性快速眼动（REM）睡眠行为障碍（iRBD）队列中言语和语言障碍与预后之间的关系。

方法

来自7个中心、讲捷克语、英语、德语、法语和意大利语的iRBD患者在基线时接受了详细的言语评估。使用基于自动语音识别和自然语言处理算法的全自动方法，对故事叙述进行转录并进行语言注释，从而得出3种独特的语言和2种声学语言退化模式及其总体严重程度的相关综合指数。然后对患者进行前瞻性随访，并在随访期间接受帕金森病或痴呆症评估。采用Cox比例风险模型评估5年随访期内语言模式对表型转化的预测价值。

结果

在180例无帕金森病或痴呆症的患者中，156例提供了随访信息。平均随访2.7年后，42例（26.9%）患者发生了神经退行性疾病。语言异常严重程度较高（风险比[HR = 2.35]）和声学异常（HR = 1.92）的患者更有可能发生明确的神经退行性疾病，转化者的内容丰富度较低（HR = 1.74）、发音速度较慢（HR = 1.58）且停顿时间延长（HR = 1.46）。痴呆首发（n = 16）和伴有轻度认知障碍的帕金森病首发（n = 9）转化者的语言异常严重程度高于认知正常的帕金森病首发转化者（n = 17）。