Abstract
Primary progressive aphasias (PPA) are neurodegenerative diseases with isolated language impairment and later progression intodementia. PPA include three clinical subtypes with different clinical manifestation and underlying neuropathology.
The nonfluent/agrammatic variant has the lowest language production with apraxia of speech and mostly belongs to tauopathies; the hallmarkof the semantic variant is a progressive loss of the meanings of words resulting in severe impairment of language comprehension,the underlying cause is mostly a TDP-43 proteinopathy; and the logopenic variant is typically manifesting with reducedsentence repetition and anomia, often due to Alzheimer's disease. Behavioral manifestations in PPA typically include agitation,eating abnormalities, disinhibition and irritability, often associated with apathy.
Early recognition of behavioral disturbances inPPA, adequate supportive and pharmacological treatment and concise caregiver support are prerequisites for preventing bothinstitutionalization of patients and excessive caregiver burden.