原发性进行性言语失用症患者的死因。
Cause of death for patients who present with primary progressive apraxia of speech.
作者信息
Utianski Rene L, Meade Gabriela, Duffy Joseph R, Boland Sarah, Whitwell Jennifer L, Botha Hugo, Josephs Keith A
机构信息
Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Department of Neurology, Mayo Clinic, Rochester, MN, USA.
出版信息
Parkinsonism Relat Disord. 2025 Jul 21;138:107968. doi: 10.1016/j.parkreldis.2025.107968.
BACKGROUND
Apraxia of speech (AOS) can be the initial and sole manifestation of neurodegenerative diseases, termed primary progressive apraxia of speech (PPAOS). These patients frequently evolve to atypical parkinsonism. There is little information to inform end of life planning and expectations.
OBJECTIVE
This study explored causes of death in patients who presented with PPAOS.
METHODS
Data for 29 deceased PPAOS patients and for whom cause of death data were available were reviewed.
RESULTS
Median age at death was 76.25 years following a median disease duration of 9.78 years. Fifteen patients (52 %) succumbed to deterioration from the disease, ten (34 %) died from complications associated with dysphagia, and the remainder from a brain bleed associated with a fall (10 %) or medical aid in dying (3 %).
CONCLUSIONS
The findings inform counseling and underscore the need for comprehensive, interdisciplinary care addressing swallowing impairments and systemic disease to prevent or delay causes of death.
背景
言语失用症（AOS）可能是神经退行性疾病的首发且唯一表现，称为原发性进行性言语失用症（PPAOS）。这些患者常发展为非典型帕金森综合征。关于临终规划和预期的信息很少。
目的
本研究探讨了出现PPAOS的患者的死因。
方法
回顾了29例已故PPAOS患者且有死因数据的资料。
结果
中位病程9.78年后，中位死亡年龄为76.25岁。15例患者（52%）死于疾病恶化，10例（34%）死于吞咽困难相关并发症，其余死于跌倒相关脑出血（10%）或临终医疗协助（3%）。
结论
这些发现为咨询提供了依据，并强调需要全面的跨学科护理，以解决吞咽障碍和全身性疾病问题，预防或延迟死亡原因。
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