Progressive nonfluent aphasia

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Clinic

  • PNFA is one of three clinical syndromes associated with frontotemporal lobar degeneration.
  • PNFA has an insidious onset of language deficits over time
  • The specific degeneration of the frontal and temporal lobes in PNFA creates hallmark language deficits in the initial absence of other cognitive and memory deficits.
  • This disorder commonly has a primary effect on the left hemisphere, causing deficit in language production difficulties and sometimes may disrupt receptive abilities in comprehending grammatically complex language.


Sign / Symptoms

  • Progressive difficulties with speech production.
  • Articulatory breakdown,
  • Phonemic breakdown (difficulties with sounds)
  • Most people will present with more than one problem. Features include
    • Hesitant, effortful speech
    • Apraxia of speech
    • Stutter (including return of a childhood stutter)
    • Anomia
    • Phonemic paraphasia (sound errors in speech e.g. 'gat' for 'cat')
    • Agrammatism (using the wrong tense or word order)


As the disease develops, speech quantity decreases and many patients become mute.

Impaired Cognition ???

Cognitive domains other than language are rarely affected early on. However, as the disease progresses, other domains can be affected. Problems with writing, reading, and speech comprehension can occur, as can behavioural features similar to frontotemporal dementia.


Progressive nonfluent aphasia Black- Box
Entities Miasms Remedies
  • Pronunciation difficult
  • Dementia
CJD
  1. Agar, Nux-m
  2. Cupr, Caust, Merc, Plb, Bell, Phos, Stram
  3. Sil, Ars, Sul

Role of CJD in Progressive nonfluent aphasia is suggested in a Brain SPECT research. [1]

  1. Kobylecki C, Thompson JC, Jones M, Mills SJ, Shaunak S, Ironside JW, Snowden JS, Richardson AM. Sporadic Creutzfeldt-Jakob disease presenting as progressive nonfluent aphasia with speech apraxia. Alzheimer Dis Assoc Disord. 2013 Oct-Dec;27(4):384-6. doi: 10.1097/WAD.0b013e318260ab27. PMID: 22732678.