As with other types of aphasia, the symptoms that accompany PPA depend on what parts of the brain's left hemisphere are significantly damaged.
[3] PPAs have a clinical and pathological overlap with the frontotemporal lobar degeneration spectrum of disorders and Alzheimer's disease.
It has been found that genetic predisposition varies among the different PPA variants, with progressive nonfluent aphasia (PNFA) being more commonly familial in nature than semantic dementia (SD).
Patients must first be diagnosed with PPA, and then divided into variants based on speech production features, repetition, single- word and syntax comprehension, confrontation naming, semantic knowledge, and reading/spelling.
[13][14] A third variant of primary progressive aphasia, LPA was then added,[15] and is an atypical form of Alzheimer's disease.
Naming impairments can be severe, specially for low-frequency objects, and can eventually lead to a more widespread semantic memory deficiency over time.
[10] The logopenic variant involves impairments in word retrieval, sentence repetition, and phonological paraphasias, comparable to conduction aphasia.
[4] Unlike those affected by Alzheimer's, people with PPA are generally able to maintain the ability to care for themselves, remain employed, and pursue interests and hobbies.
[2] The primary approach to treating PPA has been with behavioral treatment, with the hope that these methods can provide new ways for patients to communicate in order to compensate for their deteriorated abilities.
[17] 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.