Corticobasal degeneration preceded by cognitive impairment and apathy: An autopsy case report

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Background: 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. Case Presentation: 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. Conclusion: 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.

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