Medicine:Progressive nonfluent aphasia
Progressive nonfluent aphasia (PNFA) is one of three clinical syndromes associated with frontotemporal lobar degeneration. PNFA has an insidious onset of language deficits over time as opposed to other stroke-based aphasias, which occur acutely following trauma to the brain. The specific degeneration of the frontal and temporal lobes in PNFA creates hallmark language deficits differentiating this disorder from other Alzheimer-type disorders by the initial absence of other cognitive and memory deficits. This disorder commonly has a primary effect on the left hemisphere, causing the symptomatic display of expressive language deficits (production difficulties) and sometimes may disrupt receptive abilities in comprehending grammatically complex language.[1]
Presentation
The main clinical features are signature language 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:[2]
- 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.
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.[citation needed]
Diagnosis
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.[3] 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.[citation needed]
Classification
Some confusion exists 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)[4][5] included patients with progressive nonfluent (aphasia, semantic dementia, and logopenic progressive aphasia.[6][7][8]
Management
No cure or treatment for this condition has been found. Supportive management is helpful.[citation needed]
See also
References
- ↑ M. Hunter Manasco (2014). Introduction to Neurogenic Communication Disorders. pp. 86–88. ISBN 9780323290920. https://books.google.com/books?id=wM9sBQAAQBAJ.
- ↑ "Classification of primary progressive aphasia and its variants". Neurology 76 (11): 1006–1014. March 2011. doi:10.1212/WNL.0b013e31821103e6. PMID 21325651.
- ↑ "Towards a nosology for frontotemporal lobar degenerations-a meta-analysis involving 267 subjects". NeuroImage 36 (3): 497–510. July 2007. doi:10.1016/j.neuroimage.2007.03.024. PMID 17478101.
- ↑ "Slowly progressive aphasia without generalized dementia". Annals of Neurology 11 (6): 592–598. June 1982. doi:10.1002/ana.410110607. PMID 7114808.
- ↑ "Primary progressive aphasia". Annals of Neurology 49 (4): 425–432. April 2001. doi:10.1002/ana.91. PMID 11310619.
- ↑ "Classification of primary progressive aphasia and its variants". Neurology 76 (11): 1006–1014. March 2011. doi:10.1212/WNL.0b013e31821103e6. PMID 21325651.
- ↑ "The new classification of primary progressive aphasia into semantic, logopenic, or nonfluent/agrammatic variants". Current Neurology and Neuroscience Reports 10 (6): 484–490. November 2010. doi:10.1007/s11910-010-0140-4. PMID 20809401.
- ↑ "Primary progressive aphasias and their contribution to the contemporary knowledge about the brain-language relationship". Neuropsychology Review 21 (3): 271–287. September 2011. doi:10.1007/s11065-011-9175-9. PMID 21809067.
Further reading
- "Primary progressive aphasia: clinical, imaging, and neuropathological findings". American Journal of Alzheimer's Disease and Other Dementias 25 (2): 125–127. March 2010. doi:10.1177/1533317509356691. PMID 20124255.
- "Cognition and anatomy in three variants of primary progressive aphasia". Annals of Neurology 55 (3): 335–346. March 2004. doi:10.1002/ana.10825. PMID 14991811.
- "The logopenic variant of primary progressive aphasia". Current Opinion in Neurology 23 (6): 633–637. December 2010. doi:10.1097/WCO.0b013e32833fb93e. PMID 20852419.
- "Primary progressive aphasia--a language-based dementia". The New England Journal of Medicine 349 (16): 1535–1542. October 2003. doi:10.1056/NEJMra022435. PMID 14561797.
- "Cognition, language, and clinical pathological features of non-Alzheimer's dementias: an overview". Journal of Communication Disorders 43 (5): 438–452. 2010. doi:10.1016/j.jcomdis.2010.04.011. PMID 20493496.
- "Word-finding difficulty: a clinical analysis of the progressive aphasias". Brain 131 (Pt 1): 8–38. January 2008. doi:10.1093/brain/awm251. PMID 17947337.
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