The clinical differentiation of progressive disorders of language is descri
bed in the context of the evolution of current terminology. The syndromes o
f semantic dementia and progressive non-fluent aphasia can be distinguished
on clinical and neuropsychological grounds; the former is characterized by
a progressive and selective disintegration of the semantic component of lo
ngterm memory. Semantic dementia is also associated with characteristic str
uctural and functional neuroimaging findings, and may represent a form of P
ick's disease (focal lobar atrophy without Alzheimer histology). Selective
impairment of this fundamental component of human cognition has allowed the
empirical investigation of a range of theoretical questions. We discuss id
eas about the organization and representation of knowledge, the interaction
of semantic and episodic memory, and the contribution of semantic memory t
o reading ability. Many of these ideas can be informatively modelled in the
framework of connectionist theory.