Semantic dementia (SD) is a recently described clinical syndrome characteri
sed by an acquired progressive inability to name or comprehend common conce
pts, with little or no distortion of the phonological and syntactic aspects
of language, and relative sparing of other aspects of cognition, such as e
pisodic memory, nonverbal problem-solving, and perceptual and visuo-spatial
skills. The cognitive locus of this syndrome appears to lie in the permane
nt store of long-term memory representing general world knowledge-semantic
memory. The anatomical distribution of atrophy is less well-defined, and th
e contribution of various imaging modalities is discussed in the context of
a body of 45 published and unpublished cases. We conclude that involvement
of the left infero-lateral temporal cortex is the critical area in the gen
esis of SD. SD probably always represents a non-Alzheimer neurodegenerative
process; a variety of pathological lesions may be present, and possible ca
uses, together with debates about their correct classification, are discuss
ed.