Atypical Presentation of Creutzfeldt-Jacob Disease (CJD): Early Incontinence and Delayed Imaging in a Rapidly Progressive Dementia

克雅氏病(CJD)的非典型表现:快速进展性痴呆伴早期尿失禁和影像学检查延迟

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Abstract

Creutzfeldt-Jacob disease (CJD) is a rare, fatal prion disease that can present with neuropsychiatric features such as behavioral changes, visual and auditory hallucinations, and cognitive impairment. It follows a fulminant course with limited survival after symptom onset, which contributes to the limited number of diagnosed and reported cases. We report a 73-year-old woman, previously healthy and active, who exercised at the gym four days weekly, presenting with dizziness and impaired balance, followed by abrupt cognitive deterioration. Urinary incontinence developed a few weeks after cognitive decline, notably preceding the terminal stage of the illness. Magnetic resonance imaging (MRI) of the brain, which was initially deferred because of preexisting metalwork, revealed an abnormal fluid-attenuated inversion recovery (FLAIR) signal associated with reduced diffusivity involving the bilateral caudate, ventral putamen, thalami, and hippocampi symmetrically.

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