Objective: Previous studies have documented manic and hypomanic symptoms in behavioral variant frontotemporal dementia (bvFTD), suggesting a relationship between bipolar disorder and bvFTD.

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

No abstract

Brain injury can result in an increase in positive emotions. We describe a 63-year-old man who presented with a prominent personality change after a gunshot wound to the head. He became “content,” light-hearted, and prone to joking and punning. Prior to his brain injury, he suffered from frequent depression and suicidal ideation, which subsequently resolved. Examination showed a large right calvarial defect and right facial weakness, along with memory impairment and variable executive functions. Further testing was notable for excellent performance on joke comprehension, good facial emotional recognition, adequate Theory of Mind, and elevated happiness. Neuroimaging revealed loss of much of the right frontal and right anterior lobes and left orbitofrontal injury. This patient, and the literature, suggests that frontal predominant injury can facilitate the emergence of mirth along with a sense of increased happiness possibly from disinhibited activation of the subcortical reward/pleasure centers of the ventral striatal limbic area.