Accuracy of Clinical Diagnosis of Dementia with Lewy Bodies versus Neuropathology

Article type: Research Article

Authors: Skogseth, Ragnhild^{a; b; 1; *} | Hortobágyi, Tibor^{c; d; 1} | Soennesyn, Hogne^e | Chwiszczuk, Luiza^{b; f} | Ffytche, Dominic^c | Rongve, Arvid^{b; f} | Ballard, Clive^{c; g} | Aarsland, Dag^{c; e}

Affiliations: [a] Haraldsplass Deaconess Hospital, Kavli Research Centre for Geriatrics and Dementia, Bergen, Norway | [b] Department of Clinical Medicine, University of Bergen, Bergen, Norway | [c] Department of Old Age Psychiatry, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, London, UK | [d] MTA-DE Cerebrovascular and Neurodegenerative Research Group, Departments of Neurology & Neuropathology, University of Debrecen, Debrecen, Hungary | [e] Centre for Age-Related Medicine, Stavanger University Hospital, Stavanger, Norway | [f] Department of Research and Innovation, Helse Fonna, Haugesund Hospital, Haugesund, Norway | [g] University of Exeter Medical School, University of Exeter, Exeter, UK

Correspondence: [*] Correspondence to: Dr. Ragnhild Eide Skogseth, Haraldsplass Deaconess Hospital, Kavli Research Centre for Geriatrics and Dementia, Postboks 6165, 5892 Bergen, Norway. E-mails: ragnhild.skogseth@gmail.com; ragnhild.eide.skogseth@haraldsplass.no

Note: [1] These authors contributed equally to this work.

Abstract: Background: The first consensus criteria for dementia with Lewy bodies (DLB) published in 1996 were revised in 2005, partly because the original clinical criteria had suboptimal sensitivity. Few studies have assessed the accuracy of the 2005 criteria applied prospectively in newly diagnosed patients who have been followed longitudinally. Objective:To explore the correlation between clinical and pathological diagnoses in patients with DLB and Parkinson’s disease with dementia (PDD). Methods:From a prospective referral cohort study with enriched recruitment of patients with DLB and PDD, we included the first 56 patients coming to autopsy. Patients had mild dementia at inclusion and were followed annually until death with standardized clinical assessments. Pathological assessment was performed blind to clinical information according to standardized protocols and consensus criteria for DLB. Results:20 patients received a pathological diagnosis of Lewy body disease; the corresponding clinical diagnoses were probable DLB (n = 11), PDD (n = 5), probable (n = 2) or possible (n = 2) Alzheimer’s disease (AD). Of 14 patients with a clinical diagnosis of probable DLB, 11 had DLB/PDD and 3 had AD at pathology. One patient with clinically possible DLB fulfilled criteria for pathological AD. Sensitivity, specificity, positive predictive value, and negative predictive values for probable DLB were 73%, 93%, 79%, and 90%. Conclusion:Our findings suggest that the international clinical consensus criteria for DLB perform reasonably well. However, false positive and false negative diagnoses still occur, indicating that the criteria need to be improved, that biomarkers may be needed, and that neuropathological feedback is vital to improve accuracy.

Keywords: Autopsy, dementia, diagnosis, Lewy body disease, neuropathology, Parkinson’s disease

DOI: 10.3233/JAD-170274

Journal: Journal of Alzheimer's Disease, vol. 59, no. 4, pp. 1139-1152, 2017