Johnny Dang1,2, Jonathan Graff-Radford1, Joseph R Duffy3, Rene L Utianski3, Heather M Clark3, Julie A Stierwalt3, Jennifer L Whitwell4, Keith A Josephs1, Hugo Botha5. 1. Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA. 2. Mayo Clinic Alix School of Medicine, Mayo Clinic, Rochester, MN, USA. 3. Department Neurology Division of Speech Pathology, Mayo Clinic, Rochester, MN, USA. 4. Department of Radiology, Mayo Clinic, Rochester, MN, USA. 5. Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA. Botha.Hugo@mayo.edu.
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.
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.
Authors: Keith A Josephs; Joseph R Duffy; Edythe A Strand; Mary M Machulda; Matthew L Senjem; Val J Lowe; Clifford R Jack; Jennifer L Whitwell Journal: Neurology Date: 2013-06-26 Impact factor: 9.910
Authors: M L Gorno-Tempini; A E Hillis; S Weintraub; A Kertesz; M Mendez; S F Cappa; J M Ogar; J D Rohrer; S Black; B F Boeve; F Manes; N F Dronkers; R Vandenberghe; K Rascovsky; K Patterson; B L Miller; D S Knopman; J R Hodges; M M Mesulam; M Grossman Journal: Neurology Date: 2011-02-16 Impact factor: 9.910
Authors: Katerina A Tetzloff; Joseph R Duffy; Heather M Clark; Rene L Utianski; Edythe A Strand; Mary M Machulda; Hugo Botha; Peter R Martin; Christopher G Schwarz; Matthew L Senjem; Robert I Reid; Jeffrey L Gunter; Anthony J Spychalla; David S Knopman; Ronald C Petersen; Clifford R Jack; Val J Lowe; Keith A Josephs; Jennifer L Whitwell Journal: Brain Date: 2019-08-01 Impact factor: 13.501
Authors: Adam L Boxer; Jin-Tai Yu; Lawrence I Golbe; Irene Litvan; Anthony E Lang; Günter U Höglinger Journal: Lancet Neurol Date: 2017-06-13 Impact factor: 44.182
Authors: Hugo Botha; Joseph R Duffy; Jennifer L Whitwell; Edythe A Strand; Mary M Machulda; Christopher G Schwarz; Robert I Reid; Anthony J Spychalla; Matthew L Senjem; David T Jones; Val Lowe; Clifford R Jack; Keith A Josephs Journal: Cortex Date: 2015-05-27 Impact factor: 4.027
Authors: Melissa J Armstrong; Irene Litvan; Anthony E Lang; Thomas H Bak; Kailash P Bhatia; Barbara Borroni; Adam L Boxer; Dennis W Dickson; Murray Grossman; Mark Hallett; Keith A Josephs; Andrew Kertesz; Suzee E Lee; Bruce L Miller; Stephen G Reich; David E Riley; Eduardo Tolosa; Alexander I Tröster; Marie Vidailhet; William J Weiner Journal: Neurology Date: 2013-01-29 Impact factor: 9.910
Authors: Günter U Höglinger; Gesine Respondek; Maria Stamelou; Carolin Kurz; Keith A Josephs; Anthony E Lang; Brit Mollenhauer; Ulrich Müller; Christer Nilsson; Jennifer L Whitwell; Thomas Arzberger; Elisabet Englund; Ellen Gelpi; Armin Giese; David J Irwin; Wassilios G Meissner; Alexander Pantelyat; Alex Rajput; John C van Swieten; Claire Troakes; Angelo Antonini; Kailash P Bhatia; Yvette Bordelon; Yaroslau Compta; Jean-Christophe Corvol; Carlo Colosimo; Dennis W Dickson; Richard Dodel; Leslie Ferguson; Murray Grossman; Jan Kassubek; Florian Krismer; Johannes Levin; Stefan Lorenzl; Huw R Morris; Peter Nestor; Wolfgang H Oertel; Werner Poewe; Gil Rabinovici; James B Rowe; Gerard D Schellenberg; Klaus Seppi; Thilo van Eimeren; Gregor K Wenning; Adam L Boxer; Lawrence I Golbe; Irene Litvan Journal: Mov Disord Date: 2017-05-03 Impact factor: 10.338
Authors: Keith A Josephs; Joseph R Duffy; Edythe A Strand; Mary M Machulda; Matthew L Senjem; Ankit V Master; Val J Lowe; Clifford R Jack; Jennifer L Whitwell Journal: Brain Date: 2012-03-01 Impact factor: 13.501