Hirose Shoma, Kobayashi Ryota, Hatakeyama Shigeki, Kawakatsu Shinobu, Suzuki Akihito, Kawanishi Chiaki, Utsumi Kumiko
Department of Psychiatry Sunagawa City Medical Center Sunagawa Japan.
Department of Neuropsychiatry Sapporo Medical University Graduate School of Medicine Sapporo Japan.
PCN Rep. 2025 Aug 3;4(3):e70174. doi: 10.1002/pcn5.70174. eCollection 2025 Sep.
Corticobasal degeneration (CBD) presents with a range of clinical phenotypes, making diagnosis challenging. We report the clinical course of a patient with autopsy-confirmed CBD who initially presented with cognitive impairment and apathy, without motor symptoms.
A 51-year-old man presented to our hospital with decreased motivation, amnesia, and executive dysfunction. Memory disturbance and frontal lobe dysfunction were evident on examination. No motor symptoms such as Parkinsonism, apraxia, or oculomotor abnormalities were apparent. Magnetic resonance imaging and brain perfusion imaging revealed mild atrophy and hypoperfusion in the left frontal lobe. Based on these findings, he was initially diagnosed with behavioral variant frontotemporal dementia. However, due to the presence of memory impairment, Alzheimer's disease was also considered in the differential diagnosis. At the age of 53 years, he began experiencing frequent falls, followed by rapid progression of extrapyramidal symptoms. Dopamine-transporter (DAT) imaging revealed a marked reduction in DAT availability in the striatum. The patient died of aspiration pneumonia at the age of 55 years. Neuropathological examination confirmed the diagnosis of CBD, showing abundant tau pathology in the frontal lobes and basal ganglia.
In this case, abundant tau pathology in the frontal lobe corresponded with early cognitive impairment, including frontal lobe dysfunction, whereas abundant tau pathology in the basal ganglia corresponded with rapid exacerbation of extrapyramidal symptoms and marked reduction in DAT availability. Clinicians should be aware that patients with CBD without motor symptoms may seek treatment at outpatient clinics specializing in dementia.
皮质基底节变性（CBD）具有一系列临床表型，这使得诊断具有挑战性。我们报告了一例经尸检确诊为CBD的患者的临床病程，该患者最初表现为认知障碍和淡漠，无运动症状。
一名51岁男性因动机减退、失忆和执行功能障碍前来我院就诊。检查发现存在记忆障碍和额叶功能障碍。未发现帕金森症、失用症或动眼神经异常等运动症状。磁共振成像和脑灌注成像显示左额叶轻度萎缩和灌注不足。基于这些发现，他最初被诊断为行为变异型额颞叶痴呆。然而，由于存在记忆障碍，鉴别诊断中也考虑了阿尔茨海默病。53岁时，他开始频繁跌倒，随后锥体外系症状迅速进展。多巴胺转运体（DAT）成像显示纹状体中DAT可用性显著降低。该患者55岁时死于吸入性肺炎。神经病理学检查确诊为CBD，额叶和基底节显示大量tau病理改变。
在本病例中，额叶大量tau病理改变与早期认知障碍相符，包括额叶功能障碍，而基底节大量tau病理改变与锥体外系症状迅速加重及DAT可用性显著降低相符。临床医生应意识到，无运动症状的CBD患者可能会在专门诊治痴呆的门诊就诊。
