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Rapidly Progressive Dementia in the Outpatient Clinic

More Than Prions

Day, Gregory S., MD*,†; Musiek, Erik S., MD, PhD*,†; Morris, John C., MD*,†

Alzheimer Disease & Associated Disorders: October-December 2018 - Volume 32 - Issue 4 - p 291–297
doi: 10.1097/WAD.0000000000000276
Original Articles

Background: Published approaches to the evaluation and management of patients with rapidly progressive dementia (RPD) have been largely informed by experience at academic hospitals and national centers specializing in the diagnosis of Creutzfeldt-Jakob disease. Whether these approaches can be applied to patients assessed within lower-acuity outpatient settings is unknown.

Methods: A total of 96 patients with suspected RPD were assessed within the Washington University School of Medicine (Saint Louis, MO) outpatient memory clinic from February 2006 to February 2016. Consensus etiologic diagnoses were established following independent review of clinical data by 2 dementia specialists.

Results: In total, 67/90 (70%) patients manifested with faster-than-expected cognitive decline leading to dementia within 2 years of symptom onset. Female sex (42/67, 63%), median patient age (68.3 y; range, 45.4 to 89.6), and years of education (12 y; range, 6 to 14) were consistent with clinic demographics. Atypical presentations of common neurodegenerative dementing illnesses accounted for 90% (60/67) of RPD cases. Older age predicted a higher odds of amnestic Alzheimer disease dementia (OR, 2.1 per decade; 95% CI, 1.1-3.8; P=0.02). Parkinsonism (OR, 6.9; 95% CI, 1.6-30.5; P=0.01) or cortical visual dysfunction (OR, 10.8; 95% CI, 1.7-69.4; P=0.01) predicted higher odds of another neurodegenerative cause of RPD, including sporadic Creutzfeldt-Jakob disease.

Conclusions and Relevance: The clinical environment influences the prevalence of RPD causes. The clinical evaluation should be adapted to promote detection of common causes of RPD, specific to the practice setting.

*The Charles F and Joanne Knight Alzheimer Disease Research Center

Department of Neurology; Washington University School of Medicine, Saint Louis, MO

G.S.D.: participated in the conception and design of the study; acquisition, statistical analysis and interpretation of data; and drafting, revision and finalization of the manuscript. He had full access to study data and takes responsibility for the integrity of the data and the accuracy of analysis. E.S.M.: participated in the acquisition and interpretation of data and revised the manuscript for critical content. J.C.M.: participated in the conception and design of the study, interpretation of data, and revision and finalization of the manuscript.

G.S.D. is involved in research supported by an in-kind gift of radiopharmaceuticals from Avid Radiopharmaceuticals and is currently participating in clinical trials of antidementia drugs sponsored by Eli Lilly. He holds stocks (>$10,000) in ANI Pharmaceuticals (a generic pharmaceutical company). E.S.M. is currently participating in clinical trials of antidementia drugs sponsored by the A4 (The Anti-Amyloid Treatment in Asymptomatic Alzheimer’s Disease) trial and has consulted for Eisai Pharmaceuticals. He is funded by NIH grants R01AG054517 and P50AG005681. J.C.M. is currently participating in clinical trials of antidementia drugs from Eli Lilly and Company, Biogen, and Janssen. He serves as a consultant for Lilly, USA. He receives research support from Eli Lilly/Avid Radiopharmaceuticals and is funded by NIH grants # P50AG005681; P01AG003991; P01AG026276; and UF01AG032438. Neither J.C.M. nor his family owns stock or has equity interest (outside of mutual funds or other externally directed accounts) in any pharmaceutical or biotechnology company.

Reprints: Gregory S. Day, MD, Charles F and Joanne Knight Alzheimer Disease Research Center, Washington University School of Medicine, 4488 Forest Park Avenue, Suite 160, Saint Louis, MO 63108 (e-mail: gday@wustl.edu).

Received June 21, 2018

Accepted August 14, 2018

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