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Bernhard J. Steinhoff; Sabine Racker, MD; Gregor Herrendorf, MD; Sigrid Poser, MD; Stefan Grosche; Inga Zerr; Hans Kretzschmar, MD; Thomas Weber, MD

Arch Neurol. 1996;53(2):162-166.

Abstract
Objective
To assess the sensitivity, specificity, and interobserver reliability of periodic sharp wave complexes in the electroencephalograms of patients with Creutzfeldt-Jakob disease.

Design
Sixty-eight electroencephalograms in 29 patients who had been suspected of having Creutzfeldt-Jakob disease were reanalyzed by an investigator who was unaware of the clinical data. The incidence of periodic sharp wave complexes in neuropathologically confirmed Creutzfeldt-Jakob disease vs progressive dementia other than Creutzfeldt-Jakob disease was assessed. Blinded electroencephalogram analysis was performed by a second investigator. The interobserver reliability was assessed by the k value.

Setting
University hospital, base of the German National Creutzfeldt-Jakob Disease Surveillance Study.

Patients
Fifteen patients with neuropathologically confirmed Creutzfeldt-Jakob disease and 14 patients who had been suspected of having Creutzfeldt-Jakob disease because of rapidly progressive dementia but in whom other dementias were diagnosed by unblinded investigators based on clinical and electroencephalographic criteria.

Main Outcome Measure
Sensitivity and specifity of periodic sharp wave complexes assessed by their incidence in Creutzfeldt-Jakob disease vs other dementias. Interobserver reliability of periodic sharp wave complexes was expressed by the k value.

Results
For periodic sharp wave complexes, blinded electroencephalographic analysis resulted in a sensitivity and a specificity of 67% and 86%, respectively. Interobserver reliability was excellent (k=0.95).

Conclusion
This blinded electroencephalographic study in Creutzfeldt-Jakob disease confirms the high diagnostic value of electroencephalography, as previously reported by open studies.

Author Affiliations
From the Departments of Clinical Neurophysiology (Drs Steinhoff and Herrendorf), Neurology (Drs Räcker, Poser, and Weber, Mr Grosche, and Ms Zerr), and Neuropathology (Dr Kretzschmar), Georg-August University, Göttingen, Germany.

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