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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]

Classification[edit | edit source]

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. And these are the three classifications of primary progressive aphasia.[5][6][7]

Diagnosis Criteria[edit | edit source]

The following diagnosis criteria were defined by Mesulam [8]

  • Gradual impairment of word-processing, 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[edit | edit source]

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.

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]

Treatment[edit | edit source]

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 | edit source]

References[edit | edit source]

  1. 1.0 1.1 Mesulam MM (April 2001). Primary progressive aphasia. Ann. Neurol. 49 (4): 425–32.
  2. Mesulam M (1982). Slowly progressive aphasia without generalized dementia. Ann Neurol. 11 (6): 592–8.
  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.
  4. Gorno-Tempini ML, Dronkers NF, Rankin KP, et al. (Mar 2004). Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol. 55 (3): 335–46.
  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.
  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.
  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.
  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.
  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.

Further reading[edit | edit source]

  • Amici S, Ogar J, Brambati SM, et al. (Dec 2007). Performance in specific language tasks correlates with regional volume changes in progressive aphasia. Cognitive & Behavioral Neurology 20 (4): 203–11.
  • Gliebus G (March 2010). Primary progressive aphasia: clinical, imaging, and neuropathological findings. Am J Alzheimers Dis Other Demen 25 (2): 125–7.
  • Henry ML, Gorno-Tempini ML (December 2010). The logopenic variant of primary progressive aphasia. Curr. Opin. Neurol. 23 (6): 633–7.
  • Reilly J, Rodriguez AD, Lamy M, Neils-Strunjas J (2010). Cognition, language, and clinical pathological features of non-Alzheimer's dementias: an overview. J Commun Disord 43 (5): 438–52.
  • Rohrer JD, Knight WD, Warren JE, Fox NC, Rossor MN, Warren JD (January 2008). Word-finding difficulty: a clinical analysis of the progressive aphasias. Brain 131 (Pt 1): 8–38.

External links[edit | edit source]

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