Watanabe Hiroyuki, Ikeda Manabu, Mori Etsuro
Department of Behavioral Neurology and Neuropsychiatry, United Graduate School of Child Development, Osaka University, Suita, Japan.
Department of Psychiatry, Graduate School of Medicine, Osaka University, Suita, Japan.
Front Neurol. 2020 Feb 18;11:49. doi: 10.3389/fneur.2020.00049. eCollection 2020.
Dementia with Lewy bodies (DLB) is the second most common form of dementia in the elderly, and various clinical symptoms, including olfactory dysfunction, dysautonomia, depression, and rapid eye movement sleep behavior disorders (RBD), occur in patients with the prodromal state of DLB. We herein describe a case of a 72-years-old right-handed woman who exhibited primary progressive aphasia (PPA) as a prodromal state of DLB and took cholinesterase inhibitors (donepezil). At 4.5 years after aphasia onset, she exhibited all the core clinical features of DLB, including visual hallucinations, fluctuating cognition, RBD, and Parkinsonism, as well as progressive language impairment. She showed reduced dopamine transporter (DAT) uptake (assessed by DAT single-photon emission computed tomography imaging) in the striatum and decreased cardiac uptake (determined by I-metaiodobenzylguanidine myocardial scintigraphy), which are indicative biomarkers of DLB. Thus, this patient met all the criteria for probable DLB. Notably, the unique feature of this case was the presentation of PPA, which is seldom observed in typical DLB. Moreover, cholinergic enhancement (donepezil, 5 mg daily) improved her language function and global cognitive function, although mild aphasia remained. The findings provide valuable insights into the spectrum of the prodromal state of DLB and shed light on the development of the medication for PPA caused by cholinergic insufficiency.
路易体痴呆（DLB）是老年人中第二常见的痴呆形式，DLB前驱期患者会出现各种临床症状，包括嗅觉功能障碍、自主神经功能障碍、抑郁和快速眼动睡眠行为障碍（RBD）。我们在此描述一例72岁右利手女性，她表现出原发性进行性失语（PPA）作为DLB的前驱状态，并服用了胆碱酯酶抑制剂（多奈哌齐）。在失语发作4.5年后，她出现了DLB的所有核心临床特征，包括视幻觉、认知波动、RBD和帕金森症，以及进行性语言障碍。她的纹状体中多巴胺转运体（DAT）摄取减少（通过DAT单光子发射计算机断层扫描成像评估），心脏摄取减少（通过间碘苄胍心肌闪烁显像测定），这些都是DLB的指示性生物标志物。因此，该患者符合很可能的DLB的所有标准。值得注意的是，该病例的独特特征是出现PPA，这在典型的DLB中很少见。此外，胆碱能增强（多奈哌齐，每日5毫克）改善了她的语言功能和整体认知功能，尽管仍有轻度失语。这些发现为DLB前驱期的范围提供了有价值的见解，并为胆碱能不足引起的PPA的药物开发提供了线索。
