| Summary: | Abstract Introduction Among transmissible spongiform encephalopathies, Creuzfeldt-Jakob disease is considered a rare neurodegenerative disorder. The clinical features include rapid progressive dementia and myoclonic jerks, which progresses to death. Case description We report a case of a 65-year-old man, with progressive gait instability and impaired cognition with normal brain MRI. After 1 week, his symptoms became worse, EEG showed periodic sharp wave complexes, suggestive of Creuzfeldt-Jakob disease, and CSF was normal. One week later, he developed bradyphrenia and myoclonic fits. Brain MRI showed hyper-intensities mainly in the right frontal and occipital cortical gyri and caudate areas. After a few days, the patient developed akinetic mutism intractable fits, was admitted to the ICU, and was deceased after a few days. Discussion and evaluation Based on the 2010 CDC Criteria, our case was diagnosed as probable sporadic Creutzfeldt-Jakob disease (sCJD). The main findings were rapidly progressive dementia, ataxia, akinetic mutism, and myoclonus. EEG and MRI findings support the diagnosis. Conclusions Our case showed clinical, electrophysiological, and radiological features typical of probable sCJD—a rare, incurable, and fatal disease.
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