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Disease pathophysiology:
Primary progressive aphasia (PPA) is a clinical syndrome defined by the insidious onset and progressive dissolution of language skills. Although additional cognitive symptoms may emerge later in the course of illness, deficits must be grossly confined to the area of language for at least 2 years to fulfill the criteria for a diagnosis of PPA.
Disease statistics:
In about 70% of individuals with the logopenic variant of PPA, inspection of the brain at autopsy reveals the accumulation of amyloid plaques and tau neurofibrillary tangles. These are the microscopic characteristics of Alzheimer’s disease. These individuals with the logopenic variant of PPA thus can be thought to have an unusual variant of Alzheimer’s disease that begins by affecting the language regions of the brain.
Disease treatment:
Evaluation by speech therapist to improve and explore alternative communicative strategies may be useful. Non therapeutic measures like learning sign language and use of voice synthesizers or laminated cards to communicate may be beneficial. As many patients continue to be independent and active, it is important to explain the disease and the prognosis to the patient.
Major research on disease:
Differentiating the clinicopathological types is important to evaluate new therapeutic strategies in FTLD or to use disease modifying treatments in AD. As PPA is a focal neurodegeneration with a prolonged longitudinal course it offers an excellent opportunity to develop disease modifying therapies to slow or arrest progression.