Patients with Alzheimer's disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease

Abstract

Objectives-To describe the clinical presentation of patients with Alzheimer's disease (AD) or dementia with Lewy bodies (DLB) who were suspected of having Creutzfeldt-Jakob disease (CJD) and to investigate whether current clinical diagnostic criteria cover these atypical forms of AD and DLB. Methods-Brains from necropsy were examined for the diagnosis of CJD at the German reference centre for spongiform encephalopathies. Symptoms and signs in patients with suspected CJD in whom necropsy showed AD (n=19) or DLB (n=12) were analysed. Their data were compared with a group of patients with CJD (n=25) to determine overlapping and discriminating clinical features. All patients were classified according to clinical diagnostic criteria for CJD, AD, and DLB. Results-Demented patients were suspected of having CJD if disease was rapidly progressing and/or focal neurological signs appeared and/or an EEG showed sharp wave complexes. Myoclonus and limb rigidity were the most common neurological signs in all three dementias. DLB was not suspected in any patient, although patients with DLB showed parkinsonism (58%) and fluctuations (58%). Periodic sharp wave complexes (PSWCs) in EEG typical of CJD were found in five patients with AD and one patient with DLB. 14-3-3 Protein in CSF was detected in 20 patients with CJD, in two patients with AD, but not in any patient with DLB. Although most patients with DLB or AD met the clinical criteria for their respective diagnosis (74% and 90%), they also fulfilled criteria for CJD (42% and 58%). Conclusions-In patients with rapidly progressive dementia and focal neurological signs, CJD should be the first line diagnosis. Facing the triad dementia, myoclonus, and rigidity, AD should be considered if the disease course is longer and DLB is the diVerential diagnosis if parkinsonism or fluctuations are present. Findings on EEG or CSF typical of CJD do not exclude AD or DLB. (J Neurol Neurosurg Psychiatry 2001;71:33-39)

Expression of Cellular Prion Protein in the Frontal and Occipital Lobe in Alzheimer's Disease, Diffuse Lewy Body Disease, and in Normal Brain: An Immunohistochemical Study

“…In view of the recent reports suggesting that PrP c is upregulated in certain neuropathological conditions (Esiri et al 2000), including in AD (Ferrer et al 2001;Voigtländer et al 2001), we examined the expression of PrP c in frozen sections from the frontal and occipital lobe in AD and DLBD-two well-characterized neurodegenerative disorders that share certain clinical and pathological characteristics with CJD (DeArmond 1993; Budka et al 1995;Hsiao 1997;Haïk et al 2000;Tschampa et al 2001;Castellani et al 2004), and in normal control cases. This is significant because unlike routine paraffin-embedded materials, the tissues in our study had not been previously subjected to long-term fixation in aldehyde-based fixatives (notably formalin) and antigenicity was therefore well preserved by comparison.…”

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].…”

Diagnosis and treatment of rapidly progressive dementias

“…PSWCs, however, might also be seen in hepatic encephalopathy, HE, end-stage AD and DLB, and possibly other conditions. 25,31 Brain biopsy Brain biopsy is sometimes necessary when the less invasive workup is nondiagnostic. At our center, we found that brain biopsy was diagnostic in 65% of patients with RPD (excluding nonlymphomatous tumors or patients with HIV), and the results led to initiation of therapy in 44%.…”