Abstract
Primary progressive aphasia (PPA) results from selective neurodegeneration mainly in the areas of the language-dominant hemisphere, disrupting processes whose proper functioning is ensured by a complex language network in the brain, localized mainly in the cortex but also in subcortical and deeper brain areas. Speech/language deficits are the first and long-term dominant problem in the initial stage of the disease, causing significant impairment in activities of everyday life in PPA patients.
We summarize the most important aspects of PPA with special emphasis on the characteristics of three PPA variants - the nonfluent/agrammatic, semantic and logopenic variants - and primary apraxia of speech are described in terms of linguistic deficits and co-occurring cognitive disorders (neuropsychological aspects) and neuropsychiatric disorders, especially behavioral disorders. We provide information that can help to identify key symptoms more rapidly in clinical practice.
The clinical picture of PPA is complemented by localization of brain atrophy on MRI and also by summarizing specific PPA neuropathology.