Specialty neurology MeSH D018888 | OMIM 607485 | |
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.
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Classification
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.
Diagnostic criteria
The following diagnosis criteria were defined by Mesulam:
Risk factors
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.
Treatment
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.
Examples
On September 23, 2016, it was reported that Terry Jones of Monty Python had been diagnosed with the condition.