Probable Creutzfeldt-Jakob disease—A case report at Suez Canal University Hospital, Egypt

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.