Primary progressive aphasia
|Primary progressive aphasia|
|Classification and external resources|
|NCI||Primary progressive aphasia|
|Patient UK||Primary progressive aphasia|
Primary progressive aphasia (PPA) is a group of disorders characterized by progressive language and speech difficulties. It was first described as a distinct syndrome by M.-Marsel Mesulam in 1982. 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. 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). A third variant of primary progressive aphasia, Logopenic Progressive Aphasia (LPA) is an atypical form of Alzheimer's disease.
The following diagnosis criteria were defined by Mesulam 
- 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, retrospective study suggested that vasectomy could be a risk factor for PPA in men. 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.
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. 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.
- Mesulam MM (April 2001). "Primary progressive aphasia". Annals of Neurology 49 (4): 425–32. PMID 11310619. doi:10.1002/ana.91.
- Mesulam M (1982). "Slowly progressive aphasia without generalized dementia". Annals of Neurology 11 (6): 592–8. PMID 7114808. doi:10.1002/ana.410110607.
- Gorno-Tempini ML, Hillis AE, Weintraub S et al. (March 2011). "Classification of primary progressive aphasia and its variants". Neurology 76 (11): 1006–14. PMC 3059138. PMID 21325651. doi:10.1212/WNL.0b013e31821103e6.
- 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. PMC 2963791. PMID 20809401. doi:10.1007/s11910-010-0140-4.
- 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. PMC 3158975. PMID 21809067. doi:10.1007/s11065-011-9175-9.
- 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. PMID 17071925. doi:10.1093/brain/awl285.
- 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. PMC 2362399. PMID 14991811. doi:10.1002/ana.10825.
- Mesulam MM: Primary progressive aphasia—a language-based dementia. N Engl J Med 2003, 349:1535–1542
- 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.
- 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. PMID 16344531. doi:10.1212/01.wnl.0000187068.92184.63.
- 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.
- 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. PMID 18091068. doi:10.1097/WNN.0b013e31815e6265.
- Gliebus G (March 2010). "Primary progressive aphasia: clinical, imaging, and neuropathological findings". Am J Alzheimers Dis Other Demen 25 (2): 125–7. PMID 20124255. doi:10.1177/1533317509356691.
- Henry ML, Beeson PM, Alexander GE, Rapcsak SZ (February 2012). "Written language impairments in primary progressive aphasia: a reflection of damage to central semantic and phonological processes". J Cogn Neurosci 24 (2): 261–75. PMC 3307525. PMID 22004048. doi:10.1162/jocn_a_00153.
- Henry ML, Gorno-Tempini ML (December 2010). "The logopenic variant of primary progressive aphasia". Current Opinion in Neurology 23 (6): 633–7. PMC 3201824. PMID 20852419. doi:10.1097/WCO.0b013e32833fb93e.
- 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. PMC 2922444. PMID 20493496. doi:10.1016/j.jcomdis.2010.04.011.
- 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. PMC 2373641. PMID 17947337. doi:10.1093/brain/awm251.
- FAQ on PPA from IMPPACT, the International PPA Connection
- PPA information from the UCSF Memory and Aging Center
- Northwestern Cognitive Neurology and Alzheimer's Disease Center