Differential diagnosis of 201 possible Creutzfeldt-Jakob disease patients

Abstract

Our objective was to describe the clinical signs of 'possible' Creutzfeldt-Jakob disease (CJD) and to investigate whether current diagnostic criteria can accurately differentiate between different forms of dementia. We studied clinical data of 'definite' CJD, Alzheimer's disease (AD), dementia with Lewy bodies (DLB), and vascular dementia (VD) patients. Two subgroups were used: the first consisted of patients with clinical signs compatible with 'possible' CJD but in whom another final diagnosis was made and a second group with a typical evolution of the respective dementia. More focal neurological deficits were observed in AD, DLB or VD patients initially classified as 'possible' CJD than in typical patients. A typical electroencephalogram showing periodic sharp wave complexes was observed in 26 (50%) CJD and 6% of other dementia patients. The 14-3-3 protein was detected in all CJD and 8% of other dementia patients. In patients with rapidly progressive dementia and focal neurological signs, CJD should be considered. When faced with the triad: dementia, myoclonus, and initial memory problems AD should be considered if the disease duration is longer than 1 year. The diagnosis of DLB is suggested, if Parkinsonism or fluctuations are present, whereas a focal onset and compatible brain imaging can indicate VD. Findings suggestive of CJD on EEG, brain imaging, and CSF do not exclude other dementias but make them very unlikely. These observations cannot only assist in the differential diagnosis of CJD but also with the identification of AD, DLB or VD patients with atypical clinical history.

Clinicopathological Correlations and Concomitant Pathologies in Rapidly Progressive Dementia: A Brain Bank Series

“…AD was the second most frequent aetiology, followed by DLB. Similar findings have been reported in previous studies [3,4,5,25,26,27]. Vascular encephalopathy was relatively frequent among non-prion RPD, as observed in other postmortem series [4,26].…”

Serum tau protein as a marker for the diagnosis of Creutzfeldt-Jakob disease

“…Previous studies have indicated that Alzheimer's disease (AD) is the most frequent alternative diagnosis for CJD [3][4][5][6][7]. Although, generally, there is little difficulty in making a differential diagnosis between CJD and AD, some atypical cases of CJD may present with slowly progressive clinical course mimicking AD [8].…”

A case of dementia with Lewy bodies that temporarily showed symptoms similar to Creutzfeldt-Jakob disease

“…Furthermore, three studies [13][14][15] showed that there were DLB cases diagnosed neuropathologically despite clinical diagnoses as CJD. Haïk et al 13 reported that there were 10 cases (2.2%) of DLB among 465 cases clinically diagnosed as suspected and possible CJD.…”

“…Van Everbroeck et al 15 noted that there were 16 cases (8.0%) of DLB among 201 cases clinically diagnosed as possible CJD.…”