Rapidly Progressive Encephalopathy: Initial Diagnosis of Creutzfeldt Jakob Disease in an Intensive Care Unit

Creutzfeldt-Jakob disease (CJD) is a rare, incurable and fatal condition that can only be confirmed through neuropathological investigation, such as brain biopsy or post-mortem study. However, a probable diagnosis can be made using clinical criteria. CJD manifests as rapidly progressive dementia with myoclonus and to a lesser extent visual impairment and cerebellar and pyramidal/extrapyramidal signs. We report the case of a previously independent adult male that met all the clinical criteria. Taken together, the investigation results suggested probable CJD.

Creutzfeldt-Jakob disease (CJD) is a rare cause of dementia.The rapidly progressive neurological signs and symptoms suggest the diagnosis.Mortality rates are very high even with surgical treatment in these complex patients.