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a Department of
Neurology, Georg-August-Universität Göttingen,
Robert-Koch-Stra
e 40, D-37075 Göttingen, Germany Germany, b Department of Neuropathology, c Epilepsiezentrum Kork,
Germany
Correspondence to: Dr H J Tschampa htschamp{at}uni-bonn.de
Received 3 April 2000 and in revised form 29 November 2000;
Accepted 12 February
2001
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.
METHODSBrains 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.
RESULTSDemented
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%).
CONCLUSIONSIn
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 differential diagnosis if
parkinsonism or fluctuations are present. Findings on EEG or CSF
typical of CJD do not exclude AD or DLB.
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