Primary progressive aphasia

Main article: Aphasia
Primary progressive aphasia
Classification and external resources
OMIM 607485
MeSH D018888

Primary progressive aphasia (PPA) is a type of neurological syndrome in which language capabilities slowly and progressively become impaired. Although it was first described as solely impairment of language capabilities while other mental functions remain intact,[1] it is now recognized that many, if not most of those afflicted suffer impairment of memory, short term memory formation and loss of executive functions. It was first described as a distinct syndrome by M.-Marsel Mesulam in 1982.[2] Primary progressive aphasias have a clinical and pathological overlap with the frontotemporal lobar degeneration (FTLD) spectrum of disorders and Alzheimer's disease.


Three classifications of primary progressive aphasia have been described.[3][4][5] In the classical Mesulam criteria for primary progressive aphasia, there are two variants: a non-fluent type progressive nonfluent aphasia (PNFA) and a fluent type semantic dementia (SD).[6][7] A third variant of primary progressive aphasia, logopenic progressive aphasia (LPA),[8] is an atypical form of Alzheimer's disease. Each of these syndromes is based on a specific profile of language impairment and has a different distribution of atrophy, as well as possible differences in underlying pathology.[9]

Diagnostic criteria

The following diagnosis criteria were defined by Mesulam:[10]

Risk factors

There are no known environmental risk factors for the progressive aphasias. However, one observational, retrospective study suggested that vasectomy could be a risk factor for PPA in men.[11] These results have yet to be replicated or demonstrated by prospective studies.

PPA is not considered a hereditary disease. However, relatives of a person with any form of frontotemporal lobar degeneration, including PPA, are at slightly greater risk of developing PPA or another form of the condition.[12]


There is no approved treatment, but speech therapy can assist an individual with strategies to overcome difficulties. There are three very broad categories of therapy interventions for aphasia: restorative therapy approaches, compensatory therapy approaches, and social therapy approaches.[13] Rapid and sustained improvement in speech and dementia in a patient with primary progressive aphasia utilizing off-label perispinal etanercept, an anti-TNF treatment strategy also used for Alzheimer's, has been reported.[14] A video depicting the patient's improvement was published in conjunction with the print article.[15] These findings have not been independently replicated and remain controversial.


On September 23, 2016, it was reported that Terry Jones of Monty Python had been diagnosed with the condition.[16]

See also


  1. "Primary Progressive Aphasia - National Aphasia Association". National Aphasia Association. Retrieved 2015-11-12.
  2. Mesulam M (1982). "Slowly progressive aphasia without generalized dementia". Annals of Neurology. 11 (6): 592–8. doi:10.1002/ana.410110607. PMID 7114808.
  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 3059138Freely accessible. 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 2963791Freely accessible. 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 3158975Freely accessible. PMID 21809067.
  6. Mesulam MM (April 2001). "Primary progressive aphasia". Annals of Neurology. 49 (4): 425–32. doi:10.1002/ana.91. PMID 11310619.
  7. Adlam AL, Patterson K, Rogers TT, et al. (Nov 2006). "Semantic dementia and fluent primary progressive aphasia: two sides of the same coin?". Brain. 129 (Pt 11): 3066–80. doi:10.1093/brain/awl285. PMID 17071925.
  8. Gorno-Tempini ML, Dronkers NF, Rankin KP, et al. (Mar 2004). "Cognition and anatomy in three variants of primary progressive aphasia". Annals of Neurology. 55 (3): 335–46. doi:10.1002/ana.10825. PMC 2362399Freely accessible. PMID 14991811.
  9. Kertesz, Andrew; Harciarek, Michal (2014). "Primary Progressive Aphasia". Scandinavian Journal of Psychology. 55 (3): 191–201 via Wiley Online Library.
  10. Mesulam MM: Primary progressive aphasia—a language-based dementia. N Engl J Med 2003, 349:1535–1542
  11. Weintraub S, Fahey C, Johnson N, et al. (December 2006). "Vasectomy in men with primary progressive aphasia". Cogn Behav Neurol 19 (4): 190–3. doi:10.1097/01.wnn.0000213923.48632.ab. PMID 17159614.
  12. Goldman JS, Farmer JM, Wood EM, et al. (Dec 2005). "Comparison of family histories in FTLD subtypes and related tauopathies". Neurology. 65 (11): 1817–9. doi:10.1212/01.wnl.0000187068.92184.63. PMID 16344531.
  13. Manasco, H. (2014). The Aphasias. In Introduction to Neurogenic Communication Disorders (Vol. 1, p. 91). Burlington, MA: Jones & Bartlett Learning.
  14. Tobinick E (2008). "Perispinal etanercept produces rapid improvement in primary progressive aphasia: identification of a novel, rapidly reversible TNF-mediated pathophysiologic mechanism". Medscape Journal of Medicine. 10 (6): 135. PMC 2491668Freely accessible. PMID 18679537.

Further reading

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