Progressive nonfluent aphasia

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Progressive nonfluent aphasia is one of three clinical syndromes associated with frontotemporal lobar degeneration. The major symptom is progressive difficulties with the production of speech.

Clinical features[edit]

The main clinical features are progressive difficulties with speech production. There can be problems in different parts of the speech production system, hence patients can present with articulatory breakdown, phonemic breakdown (difficulties with sounds) and other problems. However, it is rare for patients to have just one of these problems and most people will present with more than one problem. Features include:

  • Hesitant, effortful speech
  • Speech 'apraxia'
  • 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 will become mute.

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.

Classification[edit]

There is some confusion in the terminology used by different neurologists. Mesulam's original description in 1982 of progressive language problems caused by neurodegenerative disease (which he called Primary Progressive Aphasia (PPA) [1][2] included patients with progressive non-fluent (PNFA), Semantic Dementia(SD), and Logopenic progressive aphasia (LPA).[3][4][5]

Imaging[edit]

Imaging studies have shown differing results which probably represents the heterogeneity of language problems than can occur in PNFA. However, classically atrophy of left perisylvian areas is seen. Comprehensive meta-analyses on MRI and FDG-PET studies identified alterations in the whole left frontotemporal network for phonological and syntactical processing as the most consistent finding.[6] Based on these imaging methods, progressive nonfluent aphasia can be regionally dissociated from the other subtypes of frontotemporal lobar degeneration, frontotemporal dementia and semantic dementia.

Management[edit]

There is no curative treatment for this condition. Supportive management is helpful.

See also[edit]

References[edit]

  1. ^ Mesulam M (1982). "Slowly progressive aphasia without generalized dementia". Ann Neurol. 11 (6): 592–8. doi:10.1002/ana.410110607. PMID 7114808. 
  2. ^ Mesulam MM (April 2001). "Primary progressive aphasia". Ann. Neurol. 49 (4): 425–32. doi:10.1002/ana.91. PMID 11310619. 
  3. ^ Gorno-Tempini ML, Hillis AE, Weintraub S, et al. (March 2011). "Classification of primary progressive aphasia and its variants". Neurology 76 (11): 1006–14. doi:10.1212/WNL.0b013e31821103e6. PMC 3059138. PMID 21325651. 
  4. ^ Bonner MF, Ash S, Grossman M (November 2010). "The new classification of primary progressive aphasia into semantic, logopenic, or nonfluent/agrammatic variants". Curr Neurol Neurosci Rep 10 (6): 484–90. doi:10.1007/s11910-010-0140-4. PMC 2963791. PMID 20809401. 
  5. ^ Harciarek M, Kertesz A (September 2011). "Primary progressive aphasias and their contribution to the contemporary knowledge about the brain-language relationship". Neuropsychol Rev 21 (3): 271–87. doi:10.1007/s11065-011-9175-9. PMC 3158975. PMID 21809067. 
  6. ^ Schroeter ML, Raczka KK, Neumann J, von Cramon DY (2007). "Towards a nosology for frontotemporal lobar degenerations – A meta-analysis involving 267 subjects.". NeuroImage 36 (3): 497–510. doi:10.1016/j.neuroimage.2007.03.024. PMID 17478101. 

Further reading[edit]

External links[edit]