Progressive nonfluent aphasia
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Progressive nonfluent aphasia is one of three clinical syndromes associated with frontotemporal lobar degeneration.
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 (hesitant, effortful speech or speech 'apraxia'), agrammatism (difficulties with syntax e.g. use of the wrong tense or word order), anomia or phonemic breakdown (difficulties with sounds). However, it is rare for patients to have just one of these problems and most people will present with more than one problem.
Features therefore include:
- Hesitant, effortful speech
- Stutter (including return of a childhood stutter)
- Anomia
- Phonemic paraphasias (sound errors in speech e.g. 'gat' for 'cat')
- Agrammatism
- 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.
Nosology
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 to 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. (See Knibb and Hodges recent paper for a good discussion of this ongoing problem - "Semantic dementia and primary progressive aphasia: a problem of categorization?" Alzheimer Dis Assoc Disord. 2005 Oct-Dec;19 Suppl 1:S7-14.)
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.
Management
There is no curative treatment for this condition. Supportive management is helpful.
References
- Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert PH, Albert M, Boone K, Miller BL, Cummings J, Benson DF. "Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria." Neurology (1998) 51(6):1546-54. Available: [2]
- Gorno-Tempini ML, Dronkers NF, Rankin KP, Ogar JM, Phengrasamy L, Rosen HJ, Johnson JK, Weiner MW, Miller BL. "Cognition and anatomy in three variants of primary progressive aphasia." Ann Neurol. (2004) 55(3):335-46.
- Mesulam MM. "Primary progressive aphasia: A language-based dementia." New England Journal of Medicine. 349:1535-1542.
== See also==