Primary Progressive Apraxia of Speech

Description

• Primary Progressive Apraxia of Speech (PPAOS) is a neurodegenerative disease affecting the planning and programming of movements necessary to speech production (Botha, H., & Josephs, K. A., 2019). This disorder is characterized by articulatory errors (additions, distortions) and prosodic impairments (slow and segmented speech). It can also be difficult for the patient to initiate speech.
• The onset of the disease is progressive and initially affects speech predominantly. PPAOS must be suspected for every patient with an evolutive speech disorder.
• Men and women are equally affected. Also, no environmental, socioeconomics or educational factors have been linked to the disease (Duffy, J. R., Utianski, R. L., & Josephs, K. A., 2020).
• A positive family history for neurodegenerative disease is found in 25% of patients diagnosed with PPAOS (Duffy, J. R., Utianski, R. L., & Josephs, K. A., 2020).
• PPAOS is a rare neurodegenerative disease and no study has yet looked into its prevalence. However, it can be estimated at 4.4 per 100,000 (Botha, H., & Josephs, K. A., 2019).

Here is a video clip of a man with Primary Progressive Apraxia of Speech (in French Only):

Other signs and symptoms

• Non-verbal oral apraxia (difficulty to perform nonspeech movements involving the face, mouth, or larynx)
• Dysarthria
• Yes/no reversal
• Executive dysfunction (impairment of the ability to be aware of our environment and to adapt to it. It mainly impairs working memory, inhibition, cognitive flexibility, and planning).
• Signs of Parkinsonism that can progress into progressive supranuclear palsy or in corticobasal syndrome:
  • Asymmetric stiffness (limb is rigid when mobilized)
  • Bradykinesia (slowness of movement)
  • Slowed down walk or parkinsonian gait (small steps, festinating, stooped posture, reduced arm swinging)
  • Postural instability and falls
  • Pseudobulbar palsy (lips and tongue weakness, difficulty swallowing, change in voice, or dysphonia)
  • Limb apraxia (Inability executing movements on command)
  • Alien hand syndrome (involuntary and complex movements with the impression of having no control on the limbs)

In more advanced stages, some signs and symptoms may appear:

• Dysphagia
• Urinary incontinence
• Aphasia
• Agrammatism
• Behavioral changes: anxiety, irritability, loss of inhibition

Some cognitive functions remain mostly well-preserved throughout the evolution of PPAOS:

• Memory
• Visuospatial functions
• Comprehension
• Gnosis

What to do when you notice symptoms in a relative?

Consult your family doctor, tell him about the "Research Chair on Primary Progressive Aphasia – Fondation de la famille Lemaire".

Your family doctor can also refer you to different resources: neurologist, speech-language pathologist.

Primary progressive APRAXIA of speech is a neurodegenerative disease for which there is currently no curative pharmacological treatment. However, a speech-language pathologist can help to plan/introduce an augmentative and alternative communication method (ex: use of an electronic tablet, pictograms/symbols, written communication, etc.). To allow optimal care, it is essential to consult as early as possible in the course of the disease.

References

1. Botha, H., & Josephs, K. A. (2019). Primary Progressive Aphasias and Apraxia of Speech. Continuum (Minneapolis, Minn.), 25(1), 101–127. – https://doi-org.acces.bibl.ulaval.ca/10.1212/CON.0000000000000699.
2. Duffy, J. R., Utianski, R. L., & Josephs, K. A. (2020). Primary progressive apraxia of speech: from recognition to diagnosis and care. Aphasiology, 1-32. – https://doi.org/10.1080/02687038.2020.1787732.
3. Josephs, K. A., Duffy, J. R., Strand, E. A., Machulda, M. M., Senjem, M. L., Gunter, J. L., Schwarz, C. G., Reid, R. I., Spychalla, A. J., Lowe, V. J., Jack, C. R., Jr, & Whitwell, J. L. (2014). The evolution of primary progressive apraxia of speech. Brain : a journal of neurology, 137(Pt 10), 2783–2795. – https://doi-org.acces.bibl.ulaval.ca/10.1093/brain/awu223.
4. Clinical Progression in Four Cases of Primary Progressive Apraxia of Speech. American journal of speech-language pathology, 27(4), 1303–1318. – https://doi-org.acces.bibl.ulaval.ca/10.1044/2018_AJSLP-17-0227.
5. Utianski, R. L., Duffy, J. R., Clark, H. M., Strand, E. A., Botha, H., Schwarz, C. G., Machulda, M. M., Senjem, M. L., Spychalla, A. J., Jack, C. R., Jr, Petersen, R. C., Lowe, V. J., Whitwell, J. L., & Josephs, K. A. (2018). Prosodic and phonetic subtypes of primary progressive apraxia of speech. Brain and language, 184, 54–65. – https://doi-org.acces.bibl.ulaval.ca/10.1016/j.bandl.2018.06.004.