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{{ClinPsy}}
 
{{ClinPsy}}
'''Primary progressive aphasia (PPA)''' is a gradually worsening disorder of speech with an insidious onset. It was first described by Marsel Mesulam in 1982.
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{{Main|Aphasia}}
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{{Infobox disease
There is considerable controversy over the [[nosology]] of this disorder. Clinical and pathological overlap have led it to being considered as part of the frontotemporal lobar degeneration (FTLD) spectrum of disorders. In the classical Mesulam criteria for PPA there are 2 variants: a non-fluent type ([[progressive non-fluent aphasia]] or PNFA) and a fluent aphasia. However, recent work has suggested that the underlying cognitive impairment in patients with progressive fluent aphasias is loss of semantic knowledge ([[semantic dementia]] or SD). [http://brain.oxfordjournals.org/cgi/content/full/129/11/3066] In the consensus criteria for FTLD described by Neary et al in 1989, PNFA and SD are the two classifications used.
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| Name = Primary progressive aphasia
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| Image =
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| Caption =
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| DiseasesDB =
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| ICD10 =
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| ICD9 =
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| ICDO =
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| OMIM = 607485
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| MedlinePlus =
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| eMedicineSubj =
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| eMedicineTopic =
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| MeshID = D018888
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}}
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'''Primary progressive aphasia (PPA)''' is characterized by [[Progressive_illness|progressive]] language and speech disorders.<ref name="Mesulam, 2001">{{cite journal |author=Mesulam MM |title=Primary progressive aphasia |journal=Ann. Neurol. |volume=49 |issue=4 |pages=425–32 |year=2001 |month=April |pmid=11310619 |doi=10.1002/ana.91 |url=}}</ref> It was first described as a distinct syndrome by Mesulam in 1982.<ref>{{cite journal |author=Mesulam M |title=Slowly progressive aphasia without generalized dementia |journal=Ann Neurol. |volume=11 |issue=6 |pages=592–8 |year=1982 |pmid=7114808 |doi=10.1002/ana.410110607}}</ref>
   
According to [http://www.sciencedaily.com/releases/2007/02/070212183237.htm/ Science Daily], in 2007, [[Northwestern University]] researchers discovered a link between higher rates of PPA dementia in men who had had vasectomies. Researchers theorize that this correlational observation may be due to increased antibody production related to the surgery. During the surgery, the protective barrier preventing sperm from mixing with the bloodstream is broken, causing the body to produce anti-sperm antibodies in 60 to 70 percent of men. However, researchers admit that more studies are needed and do not want to recommend that men abstain from vasectomy surgery because of the statistical correlation.
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==Classification==
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The classification of Primary Progressive Aphasias, has a clinical and pathological overlap of [[frontotemporal lobar degeneration|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|Progressive Nonfluent Aphasia (PNFA)]] and a fluent type [[semantic dementia|Semantic Dementia (SD)]].<ref name="Mesulam, 2001"/><ref>{{cite journal |author=Adlam AL, Patterson K, Rogers TT, ''et al.'' |title=Semantic dementia and fluent primary progressive aphasia: two sides of the same coin? |journal=Brain |volume=129 |issue=Pt 11 |pages=3066–80 |year=2006 |month=Nov |pmid=17071925 |doi=10.1093/brain/awl285 |url=http://brain.oxfordjournals.org/cgi/content/full/129/11/3066}}</ref> The third variant of primary progressive aphasia [[logopenic progressive aphasia|Logopenic Progressive Aphasia (LPA)]]<ref>{{cite journal |author=Gorno-Tempini ML, Dronkers NF, Rankin KP, ''et al.'' |title=Cognition and anatomy in three variants of primary progressive aphasia |journal=Ann Neurol. |volume=55 |issue=3 |pages=335–46 |year=2004 |month=Mar |pmid=14991811 |pmc=2362399 |doi=10.1002/ana.10825 }}</ref> is an atypical form of Alzheimer's. And these are the three classifications of primary progressive aphasia.<ref>{{cite journal |author=Gorno-Tempini ML, Hillis AE, Weintraub S, ''et al.'' |title=Classification of primary progressive aphasia and its variants |journal=Neurology |volume=76 |issue=11 |pages=1006–14 |year=2011 |month=March |pmid=21325651 |pmc=3059138 |doi=10.1212/WNL.0b013e31821103e6 |url=}}</ref><ref>{{cite journal |author=Bonner MF, Ash S, Grossman M |title=The new classification of primary progressive aphasia into semantic, logopenic, or nonfluent/agrammatic variants |journal=Curr Neurol Neurosci Rep |volume=10 |issue=6 |pages=484–90 |year=2010 |month=November |pmid=20809401 |pmc=2963791 |doi=10.1007/s11910-010-0140-4 |url=}}</ref><ref>{{cite journal |author=Harciarek M, Kertesz A |title=Primary progressive aphasias and their contribution to the contemporary knowledge about the brain-language relationship |journal=Neuropsychol Rev |volume=21 |issue=3 |pages=271–87 |year=2011 |month=September |pmid=21809067 |pmc=3158975 |doi=10.1007/s11065-011-9175-9 |url=}}</ref>
   
The San Francisco group have recently suggested that there is a third variant of PPA (so-called logopaenic PPA - Gorno-Tempini et al, 2004). However, there are limited descriptions of this disorder in the medical literature and early reports suggest that it is an atypical form of [[Alzheimer's disease]] rather than a disease falling into the frontotemporal lobar degeneration spectrum.
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==Diagnosis Criteria==
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The following diagnosis criteria were defined by Mesulam <ref>Mesulam MM: Primary progressive aphasia—a language-based dementia. N Engl J Med 2003, 349:1535–1542</ref>
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*Gradual impairment of word-processing, object naming, syntax and word-processing
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*[[Premorbid]] language function is usually intact
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*[[Acalculia]] - inability to perform simple mathematical calculations
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*[[Apraxia|Ideomotor Apraxia]] - loss of the ability to execute or carry out learned purposeful movements
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==Risk==
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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.<ref>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.</ref> These results have yet to be replicated elsewhere.
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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.<ref>{{cite journal |author=Goldman JS, Farmer JM, Wood EM, ''et al.'' |title=Comparison of family histories in FTLD subtypes and related tauopathies |journal=Neurology |volume=65 |issue=11 |pages=1817–9 |year=2005 |month=Dec |pmid=16344531 |doi=10.1212/01.wnl.0000187068.92184.63 }}</ref>
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==Treatment==
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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-[[Tumor necrosis factor|TNF]] treatment strategy also used for [[Alzheimer's]], was recently reported.<ref>{{cite journal |author=Tobinick E |title=Perispinal etanercept produces rapid improvement in primary progressive aphasia: identification of a novel, rapidly reversible TNF-mediated pathophysiologic mechanism |journal=Medscape Journal of Medicine |volume=10 |issue=6 |pages=135 |year=2008 |pmid=18679537 |pmc=2491668 }}</ref> A [http://nrimed.com/PPA.WMV.htm video depicting the patient's improvement] was published in conjunction with the print article. These findings have not been independently replicated, and remain controversial.
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==See also==
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{{col-begin}}
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{{col-2}}
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* [[Anomic aphasia]]
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* [[Aphasiology]]
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* [[Apraxia of speech]]
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* [[Speech and Language Pathology]]
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* [[Speech disorder]]
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{{col-2}}
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* [[Transcortical sensory aphasia]]
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{{col-end}}
   
 
== References ==
 
== References ==
* Mesulam, MM (2001) "Primary progressive aphasia" ''Ann Neurol.'' '''49'''(4):425-32.
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{{reflist|2}}
*Gorno-Tempini ML, Dronkers NF, Rankin KP, Ogar JM, Phengrasamy L, Rosen HJ, Johnson JK, Weiner MW, Miller BL. (2004) "Cognition and anatomy in three variants of primary progressive aphasia." ''Ann Neurol.'' '''55'''(3):335-46.
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== Further reading ==
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* {{cite journal |author=Amici S, Ogar J, Brambati SM, ''et al.'' |title=Performance in specific language tasks correlates with regional volume changes in progressive aphasia |journal=Cognitive & Behavioral Neurology |volume=20 |issue=4 |pages=203–11 |year=2007 |month=Dec |pmid=18091068 |doi=10.1097/WNN.0b013e31815e6265 }}
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* {{cite journal |author=Gliebus G |title=Primary progressive aphasia: clinical, imaging, and neuropathological findings |journal=Am J Alzheimers Dis Other Demen |volume=25 |issue=2 |pages=125–7 |year=2010 |month=March |pmid=20124255 |doi=10.1177/1533317509356691 |url=}}
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* {{cite journal |author=Henry ML, Gorno-Tempini ML |title=The logopenic variant of primary progressive aphasia |journal=Curr. Opin. Neurol. |volume=23 |issue=6 |pages=633–7 |year=2010 |month=December |pmid=20852419 |pmc=3201824 |doi=10.1097/WCO.0b013e32833fb93e |url=}}
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* {{cite journal |author=Reilly J, Rodriguez AD, Lamy M, Neils-Strunjas J |title=Cognition, language, and clinical pathological features of non-Alzheimer's dementias: an overview |journal=J Commun Disord |volume=43 |issue=5 |pages=438–52 |year=2010 |pmid=20493496 |pmc=2922444 |doi=10.1016/j.jcomdis.2010.04.011 |url=}}
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* {{cite journal |author=Rohrer JD, Knight WD, Warren JE, Fox NC, Rossor MN, Warren JD |title=Word-finding difficulty: a clinical analysis of the progressive aphasias |journal=Brain |volume=131 |issue=Pt 1 |pages=8–38 |year=2008 |month=January |pmid=17947337 |pmc=2373641 |doi=10.1093/brain/awm251 |url=}}
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==External links==
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* [http://www.ppaconnection.org/index/faq FAQ] on PPA from IMPPACT, the International PPA Connection
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* [http://memory.ucsf.edu/ftd/overview/biology/language/multiple/aphasia PPA information] from the [[UCSF]] Memory and Aging Center
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* [http://www.brain.northwestern.edu/ppa/index.html Northwestern Cognitive Neurology and Alzheimer's Disease Center]
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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]

ClassificationEdit

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 CriteriaEdit

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

RiskEdit

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]

TreatmentEdit

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 alsoEdit

References Edit

  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

  • 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 linksEdit

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