Benson’s Disease or Posterior Cortical Atrophy, Revisited

Background: D. Frank Benson and colleagues first described the clinical neuropathological features of posterior cortical atrophy (PCA) from patients in UCLA Neurobehavior Program. Objective: We reviewed Program’s subsequent experience with PCA, its potential for clarifying this relatively rare syndrome comparison to accumulated literature on PCA. Methods: Using original criteria derived clinic, 65 neuroimaging-supported PCA were diagnosed between 1995 2020. Results: On presentation, most had visual localization complaints related visuospatial symptoms, but nearly half memory followed by symptoms depression. Neurobehavioral testing showed predominant difficulty constructions, Gerstmann’s syndrome, Balint’s also impaired naming. retrospective application current Consensus Criteria 59 (91%) met a modification allowing “significantly greater over naming deficits.” There 37 deaths (56.9%) median overall survival 10.3 years (95% CI: 9.6–13.6 years), consistent slow neurodegenerative disorder patients. Conclusion: Together, these findings recommend modifying “relatively spared” memory, language, behavior include secondary depression, increased emphasis presence syndromes.