Posterior cortical atrophy.

Abstract

BACKGROUND: The term posterior cortical atrophy (PCA) was introduced in 1988 to describe five patients with fairly homogeneous, but otherwise unclassified, symptoms. These patients showed signs of a slowly progressive dementia bearing behavioral and physiologic similarities to Alzheimer's disease, but with notable distinctions. Specifically, PCA is characterized by an early onset of visual agnosia, followed by some or all components of Balint's syndrome, Gerstmann's syndrome, and transcortical sensory aphasia. REVIEW SUMMARY: In this review, the history, epidemiology, pathophysiology, neurobehavioral aspects, assessment (including neurologic and neuropsychologic), differential diagnosis, and treatment recommendations for this disorder are reviewed. CONCLUSIONS: As originally defined, PCA appears to be a clinically homogeneous syndrome. The cluster of symptoms that are common to virtually all examined cases evidences this. Although the behavioral and cognitive properties of the disorder are well established, many aspects of PCA remain unclear. Specifically, available research and understanding of PCA epidemiology and treatment are highly inadequate. In fact, the majority of such information regarding PCA is derived from studies of Alzheimer's disease. To a lesser extent, Pick's disease and Creutzfeldt-Jakob disease research have also provided insight into the underpinnings of PCA. Until PCA is categorically defined as a variant or subgroup of these other neurodegenerative disorders, however, such derivations are merely speculations.