Neurológia pre prax 6/2018
Behaviorální a řečové poruchy u primární progresivní afázie
Primary progressive aphasias (PPA) are neurodegenerative diseases with isolated language impairment and later progression into dementia. 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 hallmark of 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 reduced sentence 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 in PPA, adequate supportive and pharmacological treatment and concise caregiver support are prerequisites for preventing both institutionalization of patients and excessive caregiver burden.
Keywords: primary progressive aphasia, speech therapy, disinhibiton, irritability, focal atrophy, tauopathy, TDP-43 proteinopathy, Alzheimer’s disease
