Five patients had progressive dementia heralded by disorders of higher visual function. All eventually developed alexia, agraphia, visual agnosia, and components of Balint's, Gerstmann's, and transcortical sensory aphasia syndromes. Memory, insight, and judgment were relatively preserved until late in the course. Predominant parieto-occipital atrophy was demonstrated on both computed tomography and magnetic resonance imaging in two of the patients; posterior circulation was normal by angiography in the three studied. To date, no pathologic specimen is available for study; speculations on the underlying pathologic condition include an atypical clinical variant of Alzheimer's disease, a lobar atrophy analogous to Pick's disease, or some previously unrecognized entity.