[Primary progressive aphasia].

Abstract

Primary progressive aphasia is a rare disease characterised by slow deterioration of language, which remains the leading symptom whereas other cognitive functions such as memory, orientation, judgement, and visual-spatial skills are relatively spared and mostly not affected until 5 to 7 years after disease onset. Structural imaging displays atrophy of the left temporal region which can also affect the frontal region or both hemispheres. Reduced neuronal activity in the left temporal lobe can be seen using functional imaging even before structural anomalies are detected. Neuropathological examination reveals neuronal cell loss and gliosis in the regions of atrophy which are sometimes accompanied by spongiform changes. T-positive neurons or plaques and neurofibrillary tangles are present in rare cases. A specific therapy is not known. Logopaedic therapy focussed on the main symptoms seems to stabilise linguistic abilities.