Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease is a prion related disease, characterized by rapidly progressive dementia, associated with myoclonus and ataxia. A 55-year-old male, who initially developed depressive symptoms and obsessive-compulsive disorder one year before presentation, and in due course, developed ataxia, forgetfulness and disorientation in time and space followed by myoclonic jerks with increasing difficulty in walking, finally becoming bed bound and developing startle response to sound and touch, presented in mute and bed bound state. On the basis of typical clinical syndrome, suggestive electroencephalography and magnetic resonance imaging, and absence of any other cause, diagnosis of Creutzfeldt-Jakob disease was made.