Clinical Progression in Four Cases of Primary Progressive Apraxia of Speech Purpose This case series details the clinical progression of patients with primary progressive apraxia of speech (PPAOS) to illustrate, using several methods and supplemental material examples, the changes that occur in speech and language functioning in this patient population. Method Four patients who presented with PPAOS were followed ... Clinical Focus
Clinical Focus  |   November 21, 2018
Clinical Progression in Four Cases of Primary Progressive Apraxia of Speech
 
Author Affiliations & Notes
  • Rene L. Utianski
    Department of Neurology, Mayo Clinic, Rochester, MN
  • Joseph R. Duffy
    Department of Neurology, Mayo Clinic, Rochester, MN
  • Heather M. Clark
    Department of Neurology, Mayo Clinic, Rochester, MN
  • Edythe A. Strand
    Department of Neurology, Mayo Clinic, Rochester, MN
  • Sarah M. Boland
    Department of Neurology, Mayo Clinic, Rochester, MN
  • Mary M. Machulda
    Department of Psychiatry & Psychology Services, Mayo Clinic, Rochester, MN
  • Jennifer L. Whitwell
    Department of Radiology, Mayo Clinic, Rochester, MN
  • Keith A. Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN
  • Disclosure: The authors have declared that no competing interests existed at the time of publication.
    Disclosure: The authors have declared that no competing interests existed at the time of publication. ×
  • Correspondence to Rene L. Utianski: utianski.rene@mayo.edu
  • Editor-in-Chief: Julie Barkmeier-Kraemer
    Editor-in-Chief: Julie Barkmeier-Kraemer×
  • Editor: Nancy Solomon
    Editor: Nancy Solomon×
Article Information
Speech, Voice & Prosodic Disorders / Apraxia of Speech & Childhood Apraxia of Speech / Dysarthria / Swallowing, Dysphagia & Feeding Disorders / Special Populations / Older Adults & Aging / Language Disorders / Aphasia / Attention, Memory & Executive Functions / Speech, Voice & Prosody / Clinical Focus
Clinical Focus   |   November 21, 2018
Clinical Progression in Four Cases of Primary Progressive Apraxia of Speech
American Journal of Speech-Language Pathology, November 2018, Vol. 27, 1303-1318. doi:10.1044/2018_AJSLP-17-0227
History: Received December 27, 2017 , Revised March 1, 2018 , Accepted May 14, 2018
 
American Journal of Speech-Language Pathology, November 2018, Vol. 27, 1303-1318. doi:10.1044/2018_AJSLP-17-0227
History: Received December 27, 2017; Revised March 1, 2018; Accepted May 14, 2018

Purpose This case series details the clinical progression of patients with primary progressive apraxia of speech (PPAOS) to illustrate, using several methods and supplemental material examples, the changes that occur in speech and language functioning in this patient population.

Method Four patients who presented with PPAOS were followed between 5 and 6 years. Two patients had predominant articulatory abnormalities (termed phonetic PPAOS), 1 had predominant prosodic abnormalities (prosodic PPAOS), and 1 had relatively equal articulatory and prosodic abnormalities (mixed PPAOS). Detailed speech (including acoustics), language, neurologic, and neuropsychological data were collected.

Results At initial exam, the patients ranged from 60 to 77 years old, with presenting disease duration of 1.5–10 years. Although all patients presented with an isolated apraxia of speech, all developed varying degrees of aphasia and dysarthria. Patients with phonetic PPAOS developed relatively more severe aphasia than the other 2 patients. All patients eventually had severe functional communication limitations and required alternative or augmentative means of communication, although at varying times postonset of their initial speech problem. Two patients developed dysphagia, 3 showed mild–moderate Parkinsonism, and 2 developed depression. For all patients, simple temporal acoustic measurements documented slowed speech rate over time.

Conclusions This case series demonstrates that patients who initially present with PPAOS may develop aphasia and dysarthria, cognitive and behavioral changes, and other neurologic signs. Whether these changes can be predicted by the perceptual characteristics of the apraxia of speech is yet to be determined. The detailed longitudinal profiles provide valuable clinical insight into the progression of disease in people with PPAOS.

Supplemental Material https://doi.org/10.23641/asha.7051616

Acknowledgments
This work was supported by National Institute on Deafness and Communication Disorders Grants R01-DC010367 and R01-DC014942, awarded to Keith A. Josephs, and Grant R01-DC012519, awarded to Jennifer L. Whitwell.
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