Creutzfeldt-Jakob Disease with the presence of psychomotor agitation: a case report -

Background: Creutzfeldt–Jakob Disease (CJD) is a rare, prion-associated neurodegenerative disorder that characterized by rapidly progressive dementia and neuropsychiatric symptoms. Neuropsychiatric symptoms are prominently but infrequently manifest in the symptomatology of CJD. Conditions like confusion, agitation, mood and behavioral disturbances, psychosis or depression are reported in concurrence. This report aimed to present a case of sporadic CJD with psychomotor agitation that required hospitalization. Case: 62-year-old female patient presented with behavioral changes. She had uncontrolled behavior and amnesia for approximately one year. She has been coming to follow ups about her dementia. She had brought to emergency room by her relatives for increase in her uncontrolled behaviors and loss of communication for one and a half month. Magnetic resonance imaging (MRI) showed bilateral diffuse cortical diffusion restriction, bilateral increased signal of putamen and caudate.Electroencephalogram showed generalized periodic sharp wave complexes occurring every 1-2 seconds. Preliminary diagnosis of CJD was made and the patient was admitted to inpatient clinics for further evaluation. No myoclonus was seen while the patient was in clinic. The patient was agitated and low-dose risperidone was initiated. She was discharged after improvement in her behavioral disturbance with the neuroleptic treatment. Discussion: Rapidly progressive cognitive deterioration is the main neuropsychiatric symptom of CJD. Behavioral disturbances such as agitation and aggression were frequently described in dementia with the presence of psychotic features, however, non-psychotic psychomotor disinhibition disturbances were also recognized in different dementia syndromes. Agitation and repetitive vocalizations were commonly considered as startle reactions in CJD and often coexisted with stimulus-sensitive myoclonus, particularly in the latter stages of the disease. Structural and functional deficits in the specific brain regions such as anterior cingulate were blamed for agitated behavior. Behavioral disturbances reduce life quality in CJD and appropriate management of the symptoms would lessen the burden on caregivers and health-care professionals.