Primary progressive aphasia
|Primary progressive aphasia|
|Classification and external resources|
Primary progressive aphasia (PPA) is a type of neurological syndrome in which language capabilities slowly and progressively become impaired. Although it was first described as solely impairment of language capabilities while other mental functions remain intact, it is now recognized that many, if not most of those afflicted suffer impairment of memory, short term memory formation and loss of executive functions. 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 two 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. Each of these syndromes is based on a specific profile of language impairment and has a different distribution of atrophy, as well as possible differences in underlying pathology.
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, but speech therapy can assist an individual with strategies to overcome difficulties. There are three very broad categories of therapy interventions for aphasia: restorative therapy approaches, compensatory therapy approaches, and social therapy approaches. 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.
- "Primary Progressive Aphasia - National Aphasia Association". National Aphasia Association. Retrieved 2015-11-12.
- 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 . 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 . 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 . PMID 21809067. doi:10.1007/s11065-011-9175-9.
- Mesulam MM (April 2001). "Primary progressive aphasia". Annals of Neurology. 49 (4): 425–32. PMID 11310619. doi:10.1002/ana.91.
- 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 . PMID 14991811. doi:10.1002/ana.10825.
- Kertesz, Andrew; Harciarek, Michal (2014). "Primary Progressive Aphasia". Scandinavian Journal of Psychology. 55 (3): 191–201 – via Wiley Online Library.
- 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.
- Manasco, H. (2014). The Aphasias. In Introduction to Neurogenic Communication Disorders (Vol. 1, p. 91). Burlington, MA: Jones & Bartlett Learning.
- 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 . PMID 18679537.
- Tobinick, Edward (10 June 2008). "Video 1". Medscape J Med. 10 (6): 135. PMC . PMID 18679537.
- "Monty Python's Terry Jones diagnosed with dementia". BBC.com. 23 September 2016. Retrieved 5 July 2017.
The news was confirmed as Bafta Cymru announced the Welsh-born comedian is to be honoured with an outstanding contribution award.
- "Terry Jones, of Monty Python fame, diagnosed with dementia". 23 September 2016.
- 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 . 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 . 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 . 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 . 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
- Primary Progressive Aphasia Support Group (Facebook)