TY - JOUR
T1 - Rapidly progressive dementia in the outpatient clinic
T2 - More than prions
AU - Day, Gregory S.
AU - Musiek, Erik S.
AU - Morris, John C.
N1 - Funding Information:
G.S.D. is involved in research supported by an in-kind gift of radio-pharmaceuticals 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 con-sulted 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.
Funding Information:
The authors thank Mr. Gregory Alcazeren for assistance with data extraction and analysis during his Knight Alzheimer Disease Research Center summer studentship, and Dr. Rohini Samudralwar for providing useful comments on the manuscript. This project was supported by a career development grant (GS Day: Weston Brain Institute and American Brain Foundation), and by an endowed post-doctoral fellowship (GS Day: Neiss-Gain Fellowship) through the Knight Alzheimer Disease Research Center.
Publisher Copyright:
Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2018
Y1 - 2018
N2 - 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.
AB - 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.
KW - Creutzfeldt-Jakob disease
KW - memory clinic
KW - neurodegenerative disease
KW - outpatient
KW - rapidly progressive dementia
UR - http://www.scopus.com/inward/record.url?scp=85053817260&partnerID=8YFLogxK
U2 - 10.1097/WAD.0000000000000276
DO - 10.1097/WAD.0000000000000276
M3 - Article
C2 - 30222606
AN - SCOPUS:85053817260
SN - 0893-0341
VL - 32
SP - 291
EP - 297
JO - Alzheimer disease and associated disorders
JF - Alzheimer disease and associated disorders
IS - 4
ER -