The Presenting Symptoms of Posterior Cortical Atrophy or "Benson's Disease": The UCLA Experience

Objective: To describe the variability of presentations described by patients referred for specialty cognitive evaluation that were given the diagnosis of posterior cortical atrophy (PCA). Background: Originally described at UCLA by D. Frank Benson, posterior cortical atrophy is a neurodegenerative condition characterized by early visuospatial/perceptual symptoms resulting from posterior cortical dysfunction. Affected individuals may be misdiagnosed with primary visual disorders or alternate neurologic or psychiatric conditions which lead to unnecessary testing, procedures, and ineffective management strategies. Design/Methods: We reviewed all patients with medical records evaluated in our UCLA programs since Benson’s original description. All patients that were diagnosed with PCA by a behavioral neurologist, with available clinical records consistent with current Consensus Criteria for PCA were included in the analysis. Results: A total of 56 patients (29F) diagnosed with PCA were reviewed, dating from 1997–2019. Mean age at PCA diagnosis was 61.8 (51–79) with a mean duration of symptoms of 3.9 (1–10) years prior to diagnosis. At least 6 (10%) underwent cataract surgery with persisting symptoms. 11 (20%) did not endorse any primary visual concerns despite significant deficits when tested. Visual difficulties were characterized as memory impairment by 26 (46%) of patients. Other described deficits due to visual impairment included alexia, word finding difficulty, misplacing objects, dressing apraxia, agraphia, acalculia, impaired driving, impaired use of eating utensils and difficulty cooking, trouble using computers and phones, impaired color discrimination, and loss of visualization in dreams. Conclusions: PCA often presents with non-visual concerns which prove to be due to unrecognized visuospatial/perceptual disturbances with a thorough visual system evaluation. New difficulty with driving, locating household objects, reading or writing, using eating utensils, or getting dressed with relative preservation of primary vision should raise suspicion for PCA. Disclosure: Dr. Yerstein has nothing to disclose. Dr. Parand has nothing to disclose. Dr. Mendez has nothing to disclose.