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 characterized by progressive language and speech disorders.[1] It was first described as a distinct syndrome by Mesulam in 1982.[2]


The classification of Primary Progressive Aphasias, has a clinical and pathological overlap of Frontotemporal Lobar Degeneration (FTLD) spectrum of disorders and Alzheimer pathology. 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][3] The third variant of primary progressive aphasia Logopenic Progressive Aphasia (LPA)[4] is an atypical form of Alzheimer's disease. And these are the three classifications of primary progressive aphasia.[5][6][7]

Diagnosis 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


There are no known environmental risk factors for the progressive aphasias. However, one observational study has recently suggested that vasectomy could be a risk factor for PPA in men.[9] These results have yet to be replicated elsewhere. This is a retrospective study, so that prospective study will be needed.

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 perispinal etanercept off-label, an anti-TNF treatment strategy also used for Alzheimer's, was recently 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. ^ 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. 
  4. ^ 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. 
  5. ^ 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. 
  6. ^ 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. 
  7. ^ 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. 
  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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