Kimura Araki, Kawakami Ito, Ikeda Kenji, Nagakura Akito, Niizato Kazuhiro, Oshima Kenichi, Kato Tadafumi, Hasegawa Masato
Dementia Research Project Tokyo Metropolitan Institute of Medical Science Setagaya Tokyo Japan.
Department of Psychiatry and Behavioral science Juntendo University Graduate School of Medicine Bunkyo Tokyo Japan.
PCN Rep. 2025 May 8;4(2):e70116. doi: 10.1002/pcn5.70116. eCollection 2025 Jun.
Pick's disease (PiD) is a subtype of frontotemporal lobar degeneration. However, the pathogenesis and symptomatic lesions remain unclear. We report a case of PiD with a short disease duration and compare it to a case series to reveal the association between degenerative patterns and clinical manifestations.
The patient showed a marked decline in motivation at the age of 54 years. He was admitted with a clinical diagnosis of depressive disorder at the age of 56 years. He exhibited only apathy and lacked typical behavioral symptoms. Specialist observation revealed behavioral symptoms such as disinhibition, a lack of empathy, and hyperorality that had previously unnoticed by the patient's family members. The patient died of acute heart failure 4 days after hospitalization. Postmortem examination revealed a brain weight of 1090 g, with focal atrophy of the bilateral frontal and temporal lobes. Neuropathological findings mainly presented as numerous Pick bodies (PBs), mainly in the frontal lobe and hippocampus. PBs were immunopositive for phosphorylated tau and 3-repeat tau but negative for 4-repeat tau. The pathological findings in this case corresponded to phase II of PiD staging as defined in a previous study.
The clinical symptoms in this case, primarily characterized by apathy with minimal behavioral symptoms, were consistent with the predominant pathological involvement of the dorsolateral frontal lobe. The present case was interpreted as early-phase PiD. A comparison of the case series suggested that early-phase PiD cases may help clarify the association between early clinical manifestations and focal degenerative lesions in the frontal lobe.
皮克病(PiD)是额颞叶变性的一种亚型。然而,其发病机制和症状性病变仍不清楚。我们报告一例病程较短的皮克病病例,并与一系列病例进行比较,以揭示退变模式与临床表现之间的关联。
该患者54岁时出现明显的动机减退。56岁时因临床诊断为抑郁症入院。他仅表现出冷漠,缺乏典型的行为症状。专科观察发现了一些行为症状,如脱抑制、缺乏同理心和口欲亢进,这些症状此前未被患者家属注意到。患者住院4天后死于急性心力衰竭。尸检显示脑重1090克,双侧额叶和颞叶局灶性萎缩。神经病理学发现主要表现为大量皮克小体(PBs),主要位于额叶和海马体。PBs对磷酸化tau和3重复tau免疫阳性,但对4重复tau免疫阴性。该病例的病理发现与先前研究中定义的皮克病分期的II期相符。
该病例的临床症状主要表现为以冷漠为主且行为症状轻微,与背外侧额叶的主要病理受累情况一致。本病例被解释为早期皮克病。病例系列的比较表明,早期皮克病病例可能有助于阐明早期临床表现与额叶局灶性退变病变之间的关联。