Asian Journal of Research and Reports in Neurology

Creutzfeldt-Jakob disease is a rare, rapidly progressive prion disorder that most commonly presents with cognitive decline, myoclonus, cerebellar signs, and other neuropsychiatric manifestations. Atypical presentations may delay recognition, particularly when the initial clinical picture resembles a movement disorder. We report the case of a 70-year-old man with no significant past medical history who presented with a one-year history of asymmetric upper-limb tremor, gait instability, and progressive cognitive decline. Symptoms began with tremor and hesitant gait, followed by memory impairment, writing difficulty, disorientation, and worsening gait slowness. Neurological examination showed postural instability, a parkinsonian gait, action and postural tremor, and right-sided cogwheel rigidity. Cognitive evaluation revealed a Montreal Cognitive Assessment score of 23/30, with executive, visuospatial, memory, and writing deficits. Brain magnetic resonance imaging demonstrated cortical hyperintensity on diffusion-weighted imaging and FLAIR sequences involving the frontal, temporal, parietal, and occipital regions, while the basal ganglia and thalamus were normal. Brain PET-CT showed bilateral cortical hypometabolism involving the frontal, parietal, and occipital cortices. Cerebrospinal fluid analysis was positive for 14-3-3 protein, and electroencephalography revealed diffuse background slowing without epileptiform activity. Based on the clinical presentation and supportive investigations, probable sporadic Creutzfeldt-Jakob disease was diagnosed. The patient died four months after presentation. This case emphasises that sporadic Creutzfeldt-Jakob disease should be considered in patients with rapidly progressive parkinsonism associated with cognitive decline, even when basal ganglia abnormalities are absent on conventional neuroimaging.