Caccamo Martina, Urso Daniele, Nanni Alfredo Gabriele, Gnoni Valentina, Giugno Alessia, Vitulli Alessandra, Vilella Davide, Zecca Chiara, Dell'Abate Maria Teresa, Anastasia Antonio, De Blasi Roberto, Introna Alessandro, Logroscino Giancarlo
Department of Clinical Research in Neurology, Center for Neurodegenerative Diseases and the Aging Brain, University of Bari 'Aldo Moro', "Pia Fondazione Cardinale G. Panico", Tricase, Lecce, Italy.
Department of Translational Biomedicine and Neurosciences, Neurology Unit, Bari, Italy.
J Alzheimers Dis Rep. 2024 Jun 25;8(1):1023-1030. doi: 10.3233/ADR-230168. eCollection 2024.
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.
原发性进行性失语（PPA）中帕金森综合征特征的存在是一个正在进行研究的课题。这些特征通常在疾病的晚期更为明显，尤其是在非流利性/语法缺失亚型中，而在音韵性变异型（lvPPA）中极为罕见。在此，我们报告一例63岁男性病例，该患者以语言障碍为主，主要表现为命名和找词困难，并伴有左侧内部震颤。神经系统检查发现双侧以左侧为主的强直、运动迟缓及静止性震颤。值得注意的是，患者存在嗅觉减退和便秘。语言评估显示单字理解、物体知识保留，言语失用轻微，同时存在句子复述问题。神经影像学和生物标志物分析支持原发性进行性音韵性失语伴淀粉样蛋白病理改变并伴有显著且早期帕金森综合征的诊断。该病例强调了lvPPA中语言障碍、帕金森综合征和淀粉样蛋白病理改变之间的复杂关系。
