Creutzfeldt-Jacob Disease, As a Rare Cause of Dementia Should Not be Forgotten: Single-Center Experience

Abstract

Introduction: Dementia, which is an important public health problem, is divided into two groups as primary and secondary dementia. Creutzfeldt–Jakob disease (CJD), which is rarely seen, is a secondary cause of dementia with a rapidly progressive course. It is a fatal neurodegenerative disorder of infectious spongiform encephalopathy that can affect humans and animals. Sporadic CJD is the most common type that can present in four forms and has typical clinical presentations of progressive cognitive impairment, myoclonus, and ataxia. We aimed to discuss the demographic, clinical, and laboratory findings of CJD cases diagnosed in the neurointensive care unit. Methods: Sixteen patients were included in this retrospective study. Age, sex, complaints on admission, duration from symptom onset to mortality, neurologic examination, brain magnetic resonance imaging (MRI), electroencephalography (EEG), and the protein 14.3.3 status of the cerebrospinal fluid (CSF) were recorded. Results: The mean age was 61.18 ± 9.1 years (range, 37–73 years), and the most common complaints on admission were cognitive impairment, disturbance in consciousness, ataxia, and gait disturbance. CSF protein 14-3-3 was positive in 100% of patients. The most common findings were periodic sharp and wave complexes on EEG, cortical or putamen and caudate nucleus hyperintensity, cortical and cerebellar atrophy on MRI. One of the patients was diagnosed as variant CJD, and the others were diagnosed as the sporadic form. Conclusions: CJD should be kept in mind in patients with myoclonus or ataxia accompanied by progressive cognitive impairment. Neuroimaging, serial EEG recordings, CSF analysis, and histopathologic examination should be performed for diagnosis.