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Background Prion diseases are rare neurodegenerative diseases which can be sporadic, inherited, or acquired by infection. Sporadic Creutzfeldt Jakob Disease (sCJD) is the most well-known, accounting for approximately 90 percent of sporadic prion disease (Puoti, 2012). The Stern-Garcin variant presents with prominent extrapyramidal symptoms. Definitive diagnosis of sCJD is confirmed on histopathology, however the specificity of Real-Time quaking-induced conversion (RT-QuIC) assay may be as high as 98–100% (Foutz, 2017).Case A 64-year-old female with no chronic illnesses presented to a rural hospital with a 12-month history of gait failure with early falls, plus personality and cognitive decline. This was significantly impacting her quality of life and activities of daily living. Examination revealed bradykinesia, rigidity, bilateral resting tremor, hypophonia and impaired rapid alternating movements bilaterally, worse on the left. Grasp and snout reflexes were positive. She had postural instability and a shuffling gait with freezing on turns. Eye exam showed slow saccadic movements with vertical and horizontal nystagmus. She had minimal response to escalating doses of levodopa. Magnetic resonance imaging (MRI) showed T2 hyperintensity and restricted diffusion in bilateral basal ganglia and thalami, as well as a cortical ribbon sign in the high parietal region bilaterally. Cerebrospinal fluid was acellular with normal protein but returned a positive RT-QuIC assay. The 14–3-3 protein was negative.Conclusions/Discussion Stern-Garcin variant sCJD presents as rapidly progressive parkinsonism with predominant thalamic and basal ganglia involvement. This is highlighted in our case along with consistent MRI findings.References Puoti, Gianfranco, et al. Sporadic human prion diseases: molecular insights and diagnosis. The Lancet. Neurology 2012;11(7):618–28.Foutz, Aaron, et al. Diagnostic and prognostic value of human prion detection in cerebrospinal fluid. Annals of Neurology 2017;81(1):79–92.