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Published in: Journal of Neurology 12/2021

01-12-2021 | Frontotemporal Dementia | Original Communication

Progressive apraxia of speech: delays to diagnosis and rates of alternative diagnoses

Authors: Johnny Dang, Jonathan Graff-Radford, Joseph R. Duffy, Rene L. Utianski, Heather M. Clark, Julie A. Stierwalt, Jennifer L. Whitwell, Keith A. Josephs, Hugo Botha

Published in: Journal of Neurology | Issue 12/2021

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Abstract

Background

Progressive apraxia of speech (PAOS) is a neurodegenerative disorder of speech programming distinct from aphasia and dysarthria, most commonly associated with a 4-repeat tauopathy. Our objective was to better understand the reasons for possible delays or diagnostic errors for patients with PAOS.

Methods

Seventy-seven consecutive PAOS research participants from the Neurodegenerative Research Group were included in this study. The medical records for these patients were reviewed in detail. For each speech-related visit, data such as the chief complaint, clinical findings, and neuroimaging findings were recorded.

Results

Apraxia of speech was the initial diagnosis in 20.1% of participants at first evaluation noted in the historical record. Other common diagnoses included primary progressive aphasia (PPA) (20.1%), dysarthria (18.18%), MCI/Dementia (6.5%), and motor neuron disease (3.9%). It took a median of 2.02 (range: 0.16–8.18) years from symptoms onset for participants to receive an initial diagnosis and 3.00 (range: 0.49–9.42) years to receive a correct diagnosis. Those who were seen by a speech-language pathologist (SLP) during their first documented encounter were more likely to be correctly diagnosed with PAOS (37/48) after SLP consultation than those who were not seen by an SLP on initial encounter (5/29) (p < 0.001).

Conclusion

Approximately 80% of patients with PAOS were imprecisely diagnosed at their first visit, with it taking a median of 3 years from symptom onset to receiving a diagnosis of PAOS. Being seen by a speech-language pathologist during the initial evaluation increased the likelihood of a correct apraxia of speech diagnosis.
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Metadata
Title
Progressive apraxia of speech: delays to diagnosis and rates of alternative diagnoses
Authors
Johnny Dang
Jonathan Graff-Radford
Joseph R. Duffy
Rene L. Utianski
Heather M. Clark
Julie A. Stierwalt
Jennifer L. Whitwell
Keith A. Josephs
Hugo Botha
Publication date
01-12-2021
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 12/2021
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-021-10585-8

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