Primary progressive aphasia

Primary progressive aphasia (PPA) is a type of dementia characterized most prominently by an insidious and progressive disorder of language and speech abilities. It was first described by Marsel Mesulam in 1982. 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 [http://www.ncbi.nlm.nih.gov/pubmed/18679537] . A video depicting the patient's improvement was published in conjunction with the print article [http://nrimed.com/PPA.WMV.htm] . 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, although Mesulam in his reviews has acknowledged that one third of the cases have an underlying Alzheimer pathology. 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.

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.

There are no known risk factors for the progressive aphasias. However, one observational study has recently suggested that vasectomy could be a risk factor for PPA in men. These results have yet to be replicated elsewhere.

References

* Amici S, Ogar J, Brambati SM, Miller BL, Neuhaus J, Dronkers NL, Gorno-Tempini ML. [http://www.ncbi.nlm.nih.gov/pubmed/18091068?ordinalpos=3&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Performance in specific language tasks correlates with regional volume changes in progressive aphasia] . "Cognitive & Behavioral Neurology". 2007;20:203-11.
* Gorno-Tempini ML, Dronkers NF, Rankin KP, Ogar JM, Phengrasamy L, Rosen HJ, Johnson JK, Weiner MW, Miller BL. [http://www.ncbi.nlm.nih.gov/pubmed/14991811?dopt=Abstract Cognition and anatomy in three variants of primary progressive aphasia] . "Ann Neurol." 2004;55(3):335-46.
* Mesulam, MM. Primary progressive aphasia. "Ann Neurol." 2001;49(4):425-32.
* Rosen HJ, Allison SC, Ogar JM, Amici S, Rose K, Dronkers N, Miller BL, Gorno-Tempini ML. [http://www.ncbi.nlm.nih.gov/pubmed/17130406?ordinalpos=12&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Behavioral features in semantic dementia vs other forms of progressive aphasias] . "Neurology". 2006;67:1752-6.
* Seeley WW, Matthews BR, Crawford RK, Gorno-Tempini ML, Foti D, Mackenzie IR, Miller BL. [http://www.ncbi.nlm.nih.gov/pubmed/18057074?ordinalpos=5&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Unravelling Boléro: progressive aphasia, transmodal creativity and the right posterior neocortex] . "Brain". 2008;131:39-49.
* Edward Tobinick [http://www.ncbi.nlm.nih.gov/pubmed/18679537: Perispinal etanercept produces rapid improvement in primary progressive aphasia: identification of a novel, rapidly reversible TNF-mediated pathophysiologic mechanism.] "Medscape Journal of Medicine". 2008 Jun 10;10(6):135.
* Weintraub S, Fahey C, Johnson N, Mesulam MM, Gitelman DR, Weitner BB, Rademaker A. Vasectomy in men with primary progressive aphasia. "Cogn Behav Neurol". 2006;19(4):190-3. [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17159614&query_hl=3&itool=pubmed_docsum]

External links

* [http://memory.ucsf.edu/Education/Disease/ftd.html PPA information] from the UCSF Memory and Aging Center
* [http://nrimed.com/PPA.WMV.htm] is the webpage which contains the published video documenting rapid improvement in PPA following perispinal etanercept.