Objective: A subgroup of patients with progressive dementia has been reported with a marked predominance of symptoms attributed to the dysfunction of the posterior parieto-occipital cortex. These cases have been referred to as posterior cortical atrophy. The objective of this study was to determine whether posterior cortical atrophy is associated with distinct, uniform neuropathologic findings.
Design: Three individuals with progressive dementia that began with higher visual dysfunction (posterior cortical atrophy) were followed up to definitive neuropathologic diagnosis.
Results: Three separate neuropathologic entities were discovered: subcortical gliosis, Alzheimer's disease, and Creutzfeldt-Jakob disease.
Conclusion: Posterior cortical atrophy is a clinically homogeneous but pathologically heterogeneous syndrome.