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01-08-2019 | Frontotemporal Dementia | Original Communication

Factors that predict diagnostic stability in neurodegenerative dementia

Authors: David C. Perry, Samir Datta, Zachary A. Miller, Katherine P. Rankin, Maria Luisa Gorno-Tempini, Joel H. Kramer, Howard J. Rosen, William W. Seeley, Bruce L. Miller

Published in: Journal of Neurology | Issue 8/2019

Abstract

Objective

To determine the frequency and characteristics of clinical diagnostic change in frontotemporal dementia (FTD)-spectrum syndromes and Alzheimer’s disease (AD)-type dementia.

Methods

We reviewed records and categorized diagnostic changes in patients seen ≥ 2 times with behavioral variant FTD (bvFTD, n = 99), nonfluent and semantic variant primary progressive aphasia (nfvPPA, n = 32; svPPA, n = 59), corticobasal syndrome (CBS, n = 40), progressive supranuclear palsy-Richardson syndrome (PSP-RS, n = 34), and AD-type dementia (n = 49). For bvFTD, we compared patients with and without diagnostic change, and assessed predictors of diagnostic change by logistic regression.

Results

Initial diagnoses changed infrequently at subsequent visits in svPPA (6.8%), PSP-RS (8.8%), and nfvPPA (12.5%), with rare changes largely involving clinicopathological overlap or diagnostic ambiguity. Changes in AD-type dementia (30.6%) and CBS (37.5%) were more common, but reflected greater specificity, predicted co-pathology, or overlapping syndromes. Diagnostic change in bvFTD was also common (32.3%), but more diverse, including motor neuron disease development, alternative neurodegenerative syndromes, and non-neurodegenerative diseases. Diagnostic change occurred more often in those who met possible rather than probable bvFTD criteria (70.6% vs 15.3%, p < 0.001). Patients with stable diagnoses showed greater overall impairment, bvFTD behavioral severity, and atrophy in core right-hemisphere bvFTD regions. Patients with diagnostic change had more severe depression (p < 0.05) and more frequent contributing, secondary diagnoses (p = 0.01), such as cerebrovascular disease. By logistic regression, the accuracy of predicting stable bvFTD diagnoses using first-visit data was 80%.

Conclusion

bvFTD displays more diverse diagnostic change than other neurodegenerative syndromes. First-visit bvFTD diagnoses may waver if based on meeting possible criteria only.

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Title: Factors that predict diagnostic stability in neurodegenerative dementia
Authors: David C. Perry
Samir Datta
Zachary A. Miller
Katherine P. Rankin
Maria Luisa Gorno-Tempini
Joel H. Kramer
Howard J. Rosen
William W. Seeley
Bruce L. Miller
Publication date: 01-08-2019
Publisher: Springer Berlin Heidelberg
Keywords: Frontotemporal Dementia
Frontotemporal Dementia
Alzheimer's Disease
Dementia
Alzheimer's Disease
Dementia
Progressive Supranuclear Palsy
Published in: Journal of Neurology / Issue 8/2019
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-019-09362-5

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Factors that predict diagnostic stability in neurodegenerative dementia

Abstract

Objective

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Methods

Results

Conclusion

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