关键词: Primary progressive aphasia amyotrophic lateral sclerosis frontotemporal dementia longitudinal assessment neuropsychology

来  源:   DOI:10.1080/23279095.2024.2302833

Abstract:
UNASSIGNED: Approximately 50% of patients with amyotrophic lateral sclerosis (ALS) experience cognitive decline, with frontotemporal dementia (FTD) accounting for up to 15% of these cases. Despite this, there is considerable delay in diagnosis, which affects patient care.
UNASSIGNED: We report longitudinal results of neuropsychological evaluations in a patient diagnosed with non-fluent/agrammatic primary progressive aphasia (nfvPPA) and amyotrophic lateral sclerosis (ALS). The patient, Ms. X, presented with progressive speech difficulties starting in her late-60\'s. Initial diagnosis was nfvPPA. After 4-5 years of progressive swallowing difficulties, as well as facial weakness, her diagnosis was modified to PPA-ALS.
UNASSIGNED: Ms. X underwent neuropsychological evaluations three times over a period of five years. Results of evaluations were intact and stable over time, except for progressive loss of speech impacting her performance on a sentence repetition task.
UNASSIGNED: This case study provides valuable insight into the overlap between PPA-ALS from a neuropsychological standpoint. The results reflect preserved cognitive skills in the context of loss of speech and motor abilities. This case study also shows the length of time between onset of symptoms and clear diagnosis, which often requires an immense amount of health literacy and personal advocacy on the part of the patient.
摘要:
大约50%的肌萎缩侧索硬化症(ALS)患者认知功能下降,额颞叶痴呆(FTD)占这些病例的15%。尽管如此,诊断有相当大的延迟,影响病人护理。
我们报告了一名诊断为非流畅性/语法性原发性进行性失语(nfvPPA)和肌萎缩侧索硬化症(ALS)的患者的神经心理学评估的纵向结果。病人,X女士,从她60年代后期开始,她就出现了渐进的言语困难。最初诊断为nfvPPA。经过4-5年的渐进性吞咽困难,以及面部弱点,她的诊断被修改为PPA-ALS.
X女士在五年的时间里接受了三次神经心理学评估。评估结果是完整和稳定的,随着时间的推移,除了逐渐失去言语影响她在句子重复任务中的表现。
本案例研究从神经心理学的角度为PPA-ALS之间的重叠提供了有价值的见解。结果反映了在言语和运动能力丧失的情况下保留的认知技能。该案例研究还显示了症状发作到明确诊断之间的时间长度,这通常需要大量的健康素养和患者的个人倡导。
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