Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disease. Its clinical symptoms includes rapidly progressive dementia, gait disturbance, and myoclonous etc. We report a 72-year-old woman admitting due to hyperglycemia. Later, evalation of progressive loss of consciousness and deterioration of mentality with myoclonus and generalized convulsion led to the discovery of such a rare disease. We reviewed the literature and discussed the incidence, diagnosis, infectivity, decontamination and infection control of sporadic Creutzfeldt-Jakob disease (CJD). The clinician should pay attention to it and know how to deal with after it was diagnosed.