Yoshiike Takuya, Ueda Satoshi, Takahashi Masahiko, Suda Kiyoko, Furuta Ko, Koyama Keiko
Seishin Shinkeigaku Zasshi. 2014;116(5):359-69.
Progressive supranuclear palsy (PSP) is a neurodegenerative disorder with diverse clinical phenotypes characterized by supranuclear gaze palsy, parkinsonism with postural instability, and frontal dementia. The early and accurate diagnosis of PSP remains difficult because of the variable combination of symptoms and frequent lack of gaze abnormalities early in the disease course. Moreover, a subset of PSP shows behavioral changes as the initial presentation, which considerably overlaps with the clinical picture of frontotemporal dementia (FTD). Thus, this subgroup possibly needs psychiatric assessments. Here, we describe a clinical case of PSP difficult to differentiate from FTD because the frontal presentation persisted without gaze palsy until the late stage of the clinical course. A 58-year-old man was admitted to our hospital for the reconsideration of a diagnosis of FTD. Disinhibited and gambling behaviors inconsistent with his previous personality first appeared at around the age of 45, with gradual progression, followed by memory deficits, executive dysfunction, and a slowing of mental processes. Recurrent sexual disinhibition led him to undergo psychiatric consultation at the age of 57. Downward gaze palsy and postural instability with recurrent falls emerged 8 months after the first psychiatric examination, and he was clinically diagnosed with PSP 13 years after the initial frontal presentation. PSP should be considered in the differential diagnosis of patients presenting with frontal lobe symptoms, even in psychiatric practice.
进行性核上性麻痹(PSP)是一种神经退行性疾病,具有多种临床表型,其特征为核上性凝视麻痹、伴有姿势不稳的帕金森综合征以及额颞叶痴呆。由于症状组合多样且在病程早期常缺乏凝视异常,PSP的早期准确诊断仍然困难。此外,一部分PSP以行为改变作为首发表现,这与额颞叶痴呆(FTD)的临床表现有很大重叠。因此,这一亚组患者可能需要进行精神科评估。在此,我们描述一例难以与FTD区分的PSP临床病例,因为该患者直至临床病程晚期一直以额叶表现为主,且无凝视麻痹。一名58岁男性因重新考虑FTD诊断而入院。其抑制解除和赌博行为与其先前性格不符,首次出现在45岁左右,呈逐渐进展,随后出现记忆缺陷、执行功能障碍和思维过程迟缓。反复出现的性抑制解除导致他在57岁时接受精神科会诊。首次精神科检查8个月后出现向下凝视麻痹和反复跌倒的姿势不稳,在首次出现额叶症状13年后,他被临床诊断为PSP。即使在精神科实践中,对于出现额叶症状的患者进行鉴别诊断时也应考虑PSP。