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

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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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Acknowledgements

Funding was provided by Foundation for the National Institutes of Health (Grant Nos. R01-DC12519, R01-DC010367, R01-DC14942, R01-NS89757).

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Authors and Affiliations

  1. Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA

    Johnny Dang, Jonathan Graff-Radford, Keith A. Josephs & Hugo Botha

  2. Mayo Clinic Alix School of Medicine, Mayo Clinic, Rochester, MN, USA

    Johnny Dang

  3. Department Neurology Division of Speech Pathology, Mayo Clinic, Rochester, MN, USA

    Joseph R. Duffy, Rene L. Utianski, Heather M. Clark & Julie A. Stierwalt

  4. Department of Radiology, Mayo Clinic, Rochester, MN, USA

    Jennifer L. Whitwell

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Correspondence to Hugo Botha.

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Dang, J., Graff-Radford, J., Duffy, J.R. et al. Progressive apraxia of speech: delays to diagnosis and rates of alternative diagnoses. J Neurol 268, 4752–4758 (2021). https://doi.org/10.1007/s00415-021-10585-8

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  • DOI: https://doi.org/10.1007/s00415-021-10585-8

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