Primary progressive aphasia

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

Primary progressive aphasia (PPA) is a group of disorders characterized by progressive language and speech difficulties.[1] 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 2 variants: a non-fluent type Progressive Nonfluent Aphasia (PNFA) and a fluent type Semantic Dementia (SD).[1][6] A third variant of primary progressive aphasia, Logopenic Progressive Aphasia (LPA)[7] is an atypical form of Alzheimer's disease.

Diagnostic criteria[edit]

The following diagnosis criteria were defined by Mesulam [8]

  • Gradual impairment of object naming, syntax and word-processing
  • Premorbid language function is usually intact
  • Acalculia: inability to perform simple mathematical calculations
  • Ideomotor Apraxia: loss of the ability to execute or carry out learned purposeful movements

Risk Factors[edit]

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.[9] 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.[10]


There is no approved treatment. 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.[11] A video depicting the patient's improvement was published in conjunction with the print article. These findings have not been independently replicated and remain controversial.

See also[edit]


  1. ^ a b Mesulam MM (April 2001). "Primary progressive aphasia". Annals of Neurology 49 (4): 425–32. doi:10.1002/ana.91. PMID 11310619. 
  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 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. ^ 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. 
  7. ^ 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 2362399. PMID 14991811. 
  8. ^ Mesulam MM: Primary progressive aphasia—a language-based dementia. N Engl J Med 2003, 349:1535–1542
  9. ^ 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.
  10. ^ 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. 
  11. ^ 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 2491668. PMID 18679537. 

Further reading[edit]

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