TY - JOUR
T1 - Progressive apraxia of speech
T2 - delays to diagnosis and rates of alternative diagnoses
AU - Dang, Johnny
AU - Graff-Radford, Jonathan
AU - Duffy, Joseph R.
AU - Utianski, Rene L.
AU - Clark, Heather M.
AU - Stierwalt, Julie A.
AU - Whitwell, Jennifer L.
AU - Josephs, Keith A.
AU - Botha, Hugo
N1 - Funding Information:
Funding was provided by Foundation for the National Institutes of Health (Grant Nos. R01-DC12519, R01-DC010367, R01-DC14942, R01-NS89757).
Publisher Copyright:
© 2021, Springer-Verlag GmbH Germany, part of Springer Nature.
PY - 2021/12
Y1 - 2021/12
N2 - 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.
AB - 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.
KW - Diagnostic delay
KW - Frontotemporal dementia
KW - Primary progressive aphasia
KW - Progressive apraxia of speech
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U2 - 10.1007/s00415-021-10585-8
DO - 10.1007/s00415-021-10585-8
M3 - Article
C2 - 33945003
AN - SCOPUS:85105453590
SN - 0340-5354
VL - 268
SP - 4752
EP - 4758
JO - Journal of Neurology
JF - Journal of Neurology
IS - 12
ER -