伴有淀粉样病理和早期帕金森症的原发性进行性失语症对数变异型并存

IF 2.8 Q2 NEUROSCIENCES Journal of Alzheimer's disease reports Pub Date : 2024-06-25 eCollection Date: 2024-01-01 DOI:10.3233/ADR-230168
Martina Caccamo, Daniele Urso, Alfredo Gabriele Nanni, Valentina Gnoni, Alessia Giugno, Alessandra Vitulli, Davide Vilella, Chiara Zecca, Maria Teresa Dell'Abate, Antonio Anastasia, Roberto De Blasi, Alessandro Introna, Giancarlo Logroscino
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引用次数: 0

摘要

原发性进行性失语症(PPA)中是否存在帕金森病特征是一个正在研究的课题。这些特征通常在疾病晚期更为明显,尤其是在非流利/语法亚型中,而在对数开放变异型(lvPPA)中则异常罕见。在此,我们报告了一例 63 岁男性患者的病例,患者表现为语言障碍,主要是命名和寻词困难,同时伴有左侧内震颤。神经系统检查显示,患者双侧、左侧主要表现为僵直、运动迟缓和静止性震颤。值得注意的是,患者还存在嗅觉障碍和便秘。语言评估显示,患者的单词理解能力、物体知识和轻微的语言障碍以及句子重复问题均得到了保留。神经影像学和生物标志物分析支持原发性进行性对数开放性失语症的诊断,淀粉样病理与突出的早期帕金森症并存。该病例强调了lvPPA患者语言障碍、帕金森病和淀粉样病理之间错综复杂的关系。
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Coexisting Logopenic Variant of Primary Progressive Aphasia with Amyloid Pathology and Early Parkinsonism.

The presence of parkinsonism features in primary progressive aphasia (PPA) is a subject of ongoing research. These features are usually more pronounced in the advanced stages of the disease, particularly in the non-fluent/agrammatic subtype, and are exceptionally rare in the logopenic variant (lvPPA). Here we report a case of a 63-year-old man presenting as language impairment, predominantly naming and word-finding difficulties, emerged alongside a left-sided internal tremor. Neurological examination revealed bilateral, left-side predominant rigidity, bradykinesia, and resting tremor. Notably, anosmia and constipation were present. Language assessments showed preserved single-word comprehension, object knowledge, and a minimal apraxia of speech, as well as sentence repetition issues. Neuroimaging and biomarker analysis supported a diagnosis of primary progressive logopenic aphasia with amyloid pathology co-existing with prominent and early parkinsonism. This case underlines the intricate relationship between language disorders, parkinsonism, and amyloid pathology in lvPPA.

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