Humour processing in frontotemporal lobar degeneration: A behavioural and neuroanatomical analysis.

Humour is a complex cognitive and emotional construct that is vulnerable in neurodegenerative diseases, notably the frontotemporal lobar degenerations. However, humour processing in these diseases has been little studied. Here we assessed humour processing in patients with behavioural variant frontotemporal dementia (n = 22, mean age 67 years, four female) and semantic dementia (n = 11, mean age 67 years, five female) relative to healthy individuals (n = 21, mean age 66 years, 11 female), using a joint cognitive and neuroanatomical approach. We created a novel neuropsychological test requiring a decision about the humorous intent of nonverbal cartoons, in which we manipulated orthogonally humour content and familiarity of depicted scenarios. Structural neuroanatomical correlates of humour detection were assessed using voxel-based morphometry. Assessing performance in a signal detection framework and after adjusting for standard measures of cognitive function, both patient groups showed impaired accuracy of humour detection in familiar and novel scenarios relative to healthy older controls (p < .001). Patient groups showed similar overall performance profiles; however the behavioural variant frontotemporal dementia group alone showed a significant advantage for detection of humour in familiar relative to novel scenarios (p = .045), suggesting that the behavioural variant syndrome may lead to particular difficulty decoding novel situations for humour, while semantic dementia produces a more general deficit of humour detection that extends to stock comedic situations. Humour detection accuracy was associated with grey matter volume in a distributed network including temporo-parietal junctional and anterior superior temporal cortices, with predominantly left-sided correlates of processing humour in familiar scenarios and right-sided correlates of processing novel humour. The findings quantify deficits of core cognitive operations underpinning humour processing in frontotemporal lobar degenerations and suggest a candidate brain substrate in cortical hub regions processing incongruity and semantic associations. Humour is a promising candidate tool with which to assess complex social signal processing in neurodegenerative disease

Bladder perivascular epithelioid cell tumor: A novel rare neoplasm

Perivascular epithelioid cell tumors (PEComas) have recently been defined as a group of neoplasms that have in common the co-expression of melanocytic and smooth muscle markers. We report a novel case of a rare bladder PEComa and we review the relevant literature. Copyright © 2011 S. Karger AG

Renal autotransplantation: A final option to preserve the kidney after an iatrogenic ureteral injury

Background: Ureteral injuries are not very common and can occur after many surgical procedures. Kidney salvage is desirable. Renal autotransplantation is a final option for some cases. In this case, we report an autotransplantation of the kidney after an iatrogenic injury of the ureter with totally extraperitoneal approach. Case report: A 41 years old female underwent left endoscopic ureterolithotomy with holmium laser for ureteral calculi. An iatrogenic ureteral injury, probably ureteral avulsion, occurred. After multiple interventions, she referred to us with a nephrostomy tube. Imaging was performed and left renal autotransplantation was chosen as surgical management. The approach was totally extraperitoneal. No alteration of renal function or of urine outflow was observed during the follow up. Conclusions: The report supports the safety and efficacy of renal autotransplantation. © 2019 Edizioni Scripta Manent s.n.c.. All rights reserved

Executive dysfunction predicts social cognition impairment in amyotropic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder of the motor system with recognised extra-motor and cognitive involvement. This cross-sectional study examined ALS patients' performance on measures requiring social inference, and determined the relationship between such changes and variations in mood, behaviour, personality, empathy and executive function. Fifty-five ALS patients and 49 healthy controls were compared on tasks measuring social cognition and executive function. ALS patients also completed measures examining mood, behaviour and personality. Regression analyses explored the contribution of executive function, mood, behaviour and personality to social cognition scores within the ALS sample. A between-group MANOVA revealed that, the ALS group was impaired relative to controls on two composite scores for social cognition and executive function. Patients also performed worse on individual tests of executive function measuring cognitive flexibility, response inhibition and concept formation, and on individual aspects of social cognition assessing the attribution of emotional and mental states. Regression analyses indicated that ALS-related executive dysfunction was the main predictor of social cognition performance, above and beyond demographic variables, behaviour, mood and personality. On at least some aspects of social cognition, impaired performance in ALS appears to be secondary to executive dysfunction. The profile of cognitive impairment in ALS supports a cognitive continuum between ALS and frontotemporal dementia