Progressive posterior cortical dysfunction - A clinicopathologic series

Background: Atypical presentations of neurodegenerative dementing disorders include the syndrome of progressive posterior cortical dysfunction (PPCD) involving selective higher order visuospatial deficits. The neuropathologic correlates of PPCD remain poorly defined. Methods: This is a retrospective case series of 27 individuals ( 14 men, 13 women) diagnosed clinically with PPCD. Participants were either enrolled in the Alzheimer's Disease Research Center (ADRC) or referred to the memory diagnostic center of an urban academic medical center. Clinical evaluations included physical and neurologic examinations, the Clinical Dementia Rating (CDR), and psychometric measures. Neuropathologic examinations were completed in 21 individuals with PPCD. Psychometric measures from 65 individuals with mild dementia of the Alzheimer type (DAT) enrolled in the ADRC were used for comparison. Results: Neuropathologic etiologies of PPCD were Alzheimer disease (AD) (n = 13), AD plus Parkinson disease ( n = 1), AD-Lewy body variant ( n = 2), dementia with Lewy bodies plus progressive subcortical gliosis of Neumann ( n = 1), corticobasal degeneration ( n = 2), and prion-associated diseases: Creutzfeldt-Jakob disease ( n = 1) and fatal familial insomnia ( n = 1). Confirming the clinical impression, psychometric profiles for individuals with PPCD differed from those of people with DAT alone and revealed disproportionate deficits on measures of visuospatial ability. Conclusions: AD was the most frequent cause of PPCD in this series, although non-Alzheimer's dementing disorders also should be considered.