Nonfluent/Agrammatic Primary Progressive Aphasia

Based on Marshall et al., 2018

It can be viewed as somewhat similar to Broca’s aphasia, but evolves progressively over time rather than appearing suddenly after a brain injury such as a stroke or a traumatic brain injury.

In this variant, slow and effortful speech are noted, as if there is difficulty getting words out.

Diagnostic features
At least one feature among the following:
Agrammatism in language production (grammar or syntax errors, leading to telegraphic speech in severe cases) Apraxia of speech (motor speech disorder affecting the planning and sequencing of speech sound movements and leading to a hesitant and effortful speech with speech sound errors)
At least two features among the following:
Impaired comprehension of syntactically complex sentences (example: The dog is followed by the girl; It is the girl that the dog follows) Spared comprehension of single words Spared object knowledge

Diagnostic features based on Gorno-Tempini et al., 2011

Videos courtesy of Dr Marilu Gorno-Tempini's team at
University of San Francisco (California), Memory and Aging Center

Other signs and symptoms often associated with nonfluent/ agrammatic PPA:

• Answering yes when the person means no, or vice versa.
• Orofacial apraxia: difficulty to mimic a yawn, a cough, etc.
• Parkinsonism which can evolve into progressive supranuclear palsy (PSP) or corticobasal syndrome (CBS).
  • Paralysis of upward and downward gaze
  • Postural instability and falls
  • Slower gait
  • Akineto-rigid syndrome
  • Pseudobulbar dysfunction (lip and tongue mobility disorders, swallowing disorders, changes in the tone of voice)
  • Limb apraxia (difficulty executing a series of movements without any other motor or sensory impairment)
  • Dystonia (neurological disorder leading to involuntary and sustained muscle contractions)
  • Alien limb syndrome (involuntary complex movement of a limb, with the feeling of not being able to control it)
• Changes in behaviour such as apathy, impulsivity and depression.

Testimonial

Mrs. Laforest’s experience with the nonfluent/agrammatic variant of PPA

• PPA in a few words
  (00:00 to 00:30)
• The first symptoms
  (00:30 to 03:09)
• And the diagnosis is…
  (03:09 to 06:15)
• The course of the disease
  (06:15 to 07:31)
• Daily challenges
  (07:31 to 14:31)
• An impressive range of motor and behavioral changes
  (14:31 to 18:06)
• The last chapter
  (18:06 to the end)

Resources:

• Association des proches aidants de la Capitale-Nationale: https://www.apacn.org/
• Baluchon Alzheimer: https://baluchonalzheimer.com/
• Monastère des Augustines: https://monastere.ca/en
• Alzheimer’s Society: https://alzheimer.ca/federationquebecoise/en
• AQDR : https://www.aqdr.org/
• The Association for Frontotemporal Degeneration: https://www.theaftd.org

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