Primary progressive 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]
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Classification [edit]
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]
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 [edit]
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]
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]
References [edit]
- ^ a b Mesulam MM (April 2001). "Primary progressive aphasia". Ann. Neurol. 49 (4): 425–32. doi:10.1002/ana.91. PMID 11310619.
- ^ Mesulam M (1982). "Slowly progressive aphasia without generalized dementia". Ann Neurol. 11 (6): 592–8. doi:10.1002/ana.410110607. PMID 7114808.
- ^ 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.
- ^ 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. doi:10.1002/ana.10825. PMC 2362399. PMID 14991811.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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. doi:10.1212/01.wnl.0000187068.92184.63. PMID 16344531.
- ^ 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]
- 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. doi:10.1097/WNN.0b013e31815e6265. PMID 18091068.
- Gliebus G (March 2010). "Primary progressive aphasia: clinical, imaging, and neuropathological findings". Am J Alzheimers Dis Other Demen 25 (2): 125–7. doi:10.1177/1533317509356691. PMID 20124255.
- Henry ML, Gorno-Tempini ML (December 2010). "The logopenic variant of primary progressive aphasia". Curr. Opin. Neurol. 23 (6): 633–7. doi:10.1097/WCO.0b013e32833fb93e. PMC 3201824. PMID 20852419.
- 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. doi:10.1016/j.jcomdis.2010.04.011. PMC 2922444. PMID 20493496.
- 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. doi:10.1093/brain/awm251. PMC 2373641. PMID 17947337.
External links [edit]
- 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
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