Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common disorder among the very rare prion diseases (human transmissible subacute spongiform encephalopathies). The diagnosis might be easy if a patient with characteristics features of myoclonus, progressive dementia and periodic electroencephalography activity. Diagnosis of some patients with unusual symptoms and signs may be challenging. The reported case is a 65 years old female patient who was finally diagnosed as CJD, after previously being misdiagnosed with depression, dementia and refractory nonconvulsive status epilepticus.

Keywords: Creutzfeldt-Jakob disease, nonconvulsive status epilepticus, prion disease