Progressive nonfluent aphasia

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

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

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) included patients with both progressive non-fluent aphasia (PNFA) and semantic dementia (SD). The Neary criteria for PNFA and SD are therefore different from the Mesulam criteria for PPA. This is further confused by some doctors using the term primary progressive aphasia to mean patients with progressive non-fluent aphasia only.^[1]

Imaging

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.^[2] 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

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

See also

• Frontotemporal lobar degeneration
• Frontotemporal dementia
• Semantic dementia
• Pick's disease
• Alzheimer's disease
• Corticobasal degeneration
• Primary progressive aphasia

Notes

1. ^ Knibb JA, Hodges JR (2005). "Semantic dementia and primary progressive aphasia: a problem of categorization?". Alzheimer Dis Assoc Disord 19 (Suppl 1): S7–14. doi:10.1097/01.wad.0000183085.22562.13. PMID 16317259. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0893-0341&volume=19&issue=&spage=S7. 
2. ^ 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. 

References

• Neary D, Snowden JS, Gustafson L, et al. (December 1998). "Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria". Neurology 51 (6): 1546–54. PMID 9855500. 
• Gorno-Tempini ML, Dronkers NF, Rankin KP, et al. (March 2004). "Cognition and anatomy in three variants of primary progressive aphasia". Ann. Neurol. 55 (3): 335–46. doi:10.1002/ana.10825. PMC 2362399. PMID 14991811. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2362399. 
• Mesulam MM (October 2003). "Primary progressive aphasia—a language-based dementia". N. Engl. J. Med. 349 (16): 1535–42. doi:10.1056/NEJMra022435. PMID 14561797. http://www.nejm.org/doi/abs/10.1056/NEJMra022435?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmed. 

External links

• FTD information and videos from the UCSF Memory and Aging Center
• FTD Info from the Mayo Clinic
• The Association for Frontotemporal Dementias