[Eleven years of autopsy on account of Creutzfeldt-Jakob disease in the Netherlands].
OBJECTIVE
To describe our experience with the diagnostics of Creutzfeldt-Jakob disease (CJD) and other prion diseases in the Netherlands over a period of 11 years (1997-2007).


METHODS
Retrospective.


METHODS
In the period 1997-2007 autopsies were carried out on 280 patients with probable or possible CJD at the Dutch Surveillance Center for Prion Diseases in Utrecht. We registered clinical details, results of additional investigations such as EEG, MRI and cerebrospinal fluid tests, and outcomes of neurological investigations. The contribution of the different disorders within this group was estimated retrospectively.


RESULTS
A prion disease was diagnosed in 146 patients (52%) with probable or possible CJD. 133 (91%) of these had the sporadic form. 2 patients were diagnosed with the 'variant CJD' (caused by bovine spongiform encephalopathy). 5 patients were diagnosed as having an iatrogenic form of CJD and 6 patients had a genetic form of the disease. A different disease was diagnosed in 134 patients (48%), such as Alzheimer disease (40%), multi-infarct dementia (13%), neoplasm (10%) and Lewy body dementia (8%). In this group, periodic sharp wave complexes were observed on EEG in 17 patients (13%), most frequently in those with Alzheimer disease. The 14-3-3 protein test on cerebrospinal fluid was positive in 28 of these patients (21%), most frequently in patients with vascular dementia and Alzheimer disease.


CONCLUSIONS
In all cases of an unclear clinical picture suggestive of neurodegenerative disease, prion disease must be considered. Periodic sharp wave complexes on EEG and a positive 14-3-3 protein test on cerebrospinal fluid alone are not diagnostic of CJD.
Universitair Medisch Centrum Utrecht, Nederlands Surveillance Centrum voor Prionziekten, afd. Pathologie, Utrecht, The Netherlands. c.jansen@umcutrecht.nl
Casper
C
Jansen
Maaike
M
Schuur
Wim G M
WG
Spliet
Willem A
WA
van Gool
Cornelia M
CM
van Duijn
Annemieke J M
AJ
Rozemuller
D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid*; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000544:Alzheimer Disease / Q000134:cerebrospinal fluid / Q000175:diagnosis; D001344:Autopsy; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid / Q000175:diagnosis*; D015140:Dementia, Vascular / Q000134:cerebrospinal fluid / Q000175:diagnosis; D003937:Diagnosis, Differential; D004569:Electroencephalography; D005260:Female; D006801:Humans; D020961:Lewy Body Disease / Q000134:cerebrospinal fluid / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D009426:Netherlands; D012189:Retrospective Studies; D018571:Sentinel Surveillance; D055815:Young Adult
D048948:14-3-3 Proteins