Sexual Harassment, Abuse, and Discrimination in Obstetrics and Gynecology: A Systematic Review.

Importance: Unlike other surgical specialties, obstetrics and gynecology (OB-GYN) has been predominantly female for the last decade. The association of this with gender bias and sexual harassment is not known. Objective: To systematically review the prevalence of sexual harassment, bullying, abuse, and discrimination among OB-GYN clinicians and trainees and interventions aimed at reducing harassment in OB-GYN and other surgical specialties. Evidence Review: A systematic search of PubMed, Embase, and ClinicalTrials.gov was conducted to identify studies published from inception through June 13, 2023.: For the prevalence of harassment, OB-GYN clinicians and trainees on OB-GYN rotations in all subspecialties in the US or Canada were included. Personal experiences of harassment (sexual harassment, bullying, abuse, and discrimination) by other health care personnel, event reporting, burnout and exit from medicine, fear of retaliation, and related outcomes were included. Interventions across all surgical specialties in any country to decrease incidence of harassment were also evaluated. Abstracts and potentially relevant full-text articles were double screened.: Eligible studies were extracted into standard forms. Risk of bias and certainty of evidence of included research were assessed. A meta-analysis was not performed owing to heterogeneity of outcomes. Findings: A total of 10 eligible studies among 5852 participants addressed prevalence and 12 eligible studies among 2906 participants addressed interventions. The prevalence of sexual harassment (range, 250 of 907 physicians [27.6%] to 181 of 255 female gynecologic oncologists [70.9%]), workplace discrimination (range, 142 of 249 gynecologic oncologists [57.0%] to 354 of 527 gynecologic oncologists [67.2%] among women; 138 of 358 gynecologic oncologists among males [38.5%]), and bullying (131 of 248 female gynecologic oncologists [52.8%]) was frequent among OB-GYN respondents. OB-GYN trainees commonly experienced sexual harassment (253 of 366 respondents [69.1%]), which included gender harassment, unwanted sexual attention, and sexual coercion. The proportion of OB-GYN clinicians who reported their sexual harassment to anyone ranged from 21 of 250 AAGL (formerly, the American Association of Gynecologic Laparoscopists) members (8.4%) to 32 of 256 gynecologic oncologists (12.5%) compared with 32.6% of OB-GYN trainees. Mistreatment during their OB-GYN rotation was indicated by 168 of 668 medical students surveyed (25.1%). Perpetrators of harassment included physicians (30.1%), other trainees (13.1%), and operating room staff (7.7%). Various interventions were used and studied, which were associated with improved recognition of bias and reporting (eg, implementation of a video- and discussion-based mistreatment program during a surgery clerkship was associated with a decrease in medical student mistreatment reports from 14 reports in previous year to 9 reports in the first year and 4 in the second year after implementation). However, no significant decrease in the frequency of sexual harassment was found with any intervention. Conclusions and Relevance: This study found high rates of harassment behaviors within OB-GYN. Interventions to limit these behaviors were not adequately studied, were limited mostly to medical students, and typically did not specifically address sexual or other forms of harassment.

Repair of Vesicovaginal Fistulae: A Systematic Review.

OBJECTIVE: To assess and compile the current level of evidence regarding successful surgical treatment of vesicovaginal fistulae and how these perioperative interventions affect anatomic, patient-centered, and adverse outcomes. DATA SOURCES: PubMed and EMBASE were searched from inception through September 9, 2022. METHODS OF STUDY SELECTION: This review included comparative studies (of any sample size) and single-group studies (1,000 or more participants) of primary or recurrent vesicovaginal fistula (ie, vesicovaginal fistula, urethrovaginal fistula, and bladder neck-vaginal fistula). We evaluated preintervention assessment or management, various techniques for intraoperative management, and postoperative management. Outcomes of interest included anatomic and objective outcomes (such as successful repair, fistula closure, urinary incontinence, recurrent fistula, perioperative complications) and subjective outcomes (such as voiding symptoms and quality of life). Abstracts and full-text articles were screened in duplicate, and study descriptions and findings were extracted into standardized extraction forms. Risk of bias was assessed independently by two investigators and adjudicated by a third. Study quality was summarized with standardized tools. We conducted random-effects model and restricted maximum-likelihood meta-analyses of relative risks when at least three studies compared similar interventions and reported similar outcome measures. TABULATION, INTEGRATION, AND RESULTS: Forty-six studies met the inclusion criteria. Studies were categorized into 11 domains: 1) preoperative assessment, 2) preoperative and postoperative physical therapy, 3) route of surgery, 4) incorporation of a flap, 5) trimming, 6) layered closure, 7) intraoperative antibiotics, 8) fibrin glue, 9) fascial sling, 10) postoperative Foley catheter duration, and 11) quality of life. Although the strength of the data is insufficient, preoperative phenazopyridine, physical therapy, layered closure, and intraoperative antibiotics seemed to improve the rate of successful fistula repair. Route of surgery (vaginal vs abdominal laparotomy) was determined primarily by surgeon preference and showed no difference in successful fistula repair. In addition, use of interpositional flaps, trimming fistula edges, fibrin glue, and fascial sling did not show significant improvement in rates of fistula cure. Overall, quality-of-life scores improved postoperatively regardless of route of repair and use of interpositional flaps. CONCLUSION: Our findings highlight the limited information available to guide evidence-based treatment of vesicovaginal fistula repair. Overall, high-quality evidence is lacking to provide guidelines; therefore, expert opinion remains the primary influence for fistula repair recommendations. SYSTEMATIC REVIEW REGISTRATION: PROSPERO, CRD42021214948.

Concomitant progressive supranuclear palsy and Lewy body pathology presenting with circumscribed visual memory loss: A clinicopathological case.

A 70-year-old man presented to the clinic with impairment of visual memory and marked predominantly right sided mesial temporal lobe atrophy on imaging. He died 6 years following symptom onset and neuropathological examination showed concomitant progressive supranuclear palsy and Lewy body pathology. Although he did not fulfil clinical criteria for either condition at presentation, we propose that interactions between the two pathologies in mesial temporal regions could result in this atypical clinical phenotype.

Prescription Opioid Use for Acute Pain and Persistent Opioid Use After Gynecologic Surgery: A Systematic Review.

OBJECTIVE: To assess the amount of opioid medication used by patients and the prevalence of persistent opioid use after discharge for gynecologic surgery for benign indications. DATA SOURCES: We systematically searched MEDLINE, EMBASE, and ClinicalTrials.gov from inception to October 2020. METHODS OF STUDY SELECTION: Studies with data on gynecologic surgical procedures for benign indications and the amount of outpatient opioids consumed, or the incidence of either persistent opioid use or opioid-use disorder postsurgery were included. Two reviewers independently screened citations and extracted data from eligible studies. TABULATION, INTEGRATION, AND RESULTS: Thirty-six studies (37 articles) met inclusion criteria. Data were extracted from 35 studies; 23 studies included data on opioids consumed after hospital discharge, and 12 studies included data on persistent opioid use after gynecologic surgery. Average morphine milligram equivalents (MME) used in the 14 days after discharge were 54.0 (95% CI 39.9-68.0, seven tablets of 5-mg oxycodone) across all gynecologic surgery types, 35.0 (95% CI 0-75.12, 4.5 tablets of 5-mg oxycodone) after a vaginal hysterectomy, 59.5 (95% CI 44.4-74.6, eight tablets of 5-mg oxycodone) after laparoscopic hysterectomy, and 108.1 (95% CI 80.5-135.8, 14.5 tablets of 5-mg oxycodone) after abdominal hysterectomy. Patients used 22.4 MME (95% CI 12.4-32.3, three tablets of 5-mg oxycodone) within 24 hours of discharge after laparoscopic procedures without hysterectomy and 79.8 MME (95% CI 37.1-122.6, 10.5 tablets of 5-mg oxycodone) from discharge to 7 or 14 days postdischarge after surgery for prolapse. Persistent opioid use occurred in about 4.4% of patients after gynecologic surgery, but this outcome had high heterogeneity due to variation in populations and definitions of the outcome. CONCLUSION: On average, patients use the equivalent of 15 or fewer 5-mg oxycodone tablets (or equivalent) in the 2 weeks after discharge after major gynecologic surgery for benign indications. Persistent opioid use occurred in 4.4% of patients who underwent gynecologic surgery for benign indications. Our findings could help surgeons minimize overprescribing and reduce medication diversion or misuse. SYSTEMATIC REVIEW REGISTRATION: PROSPERO, CRD42020146120.

Structural and metabolic correlates of neuropsychological profiles in multiple system atrophy and Parkinson's disease.

BACKGROUND: Despite increased recognition of cognitive impairment in Multiple System Atrophy (MSA), its neuroanatomical correlates are not well defined. We aimed to explore cognitive profiles in MSA with predominant parkinsonism (MSA-P) and Parkinson's disease (PD) and their relationship to frontostriatal structural and metabolic changes. METHODS: Detailed clinical and neuropsychological evaluation was performed together with diffusion tensor imaging (DTI) and [18F]-fluoro-deoxyglucose positron emission tomography ([18F]-FDG-PET) in patients with MSA-P (n = 11) and PD (n = 11). We compared clinical and neuropsychological data to healthy controls (n = 9) and correlated neuropsychological data with imaging findings in MSA-P and PD. RESULTS: Patients with MSA-P showed deficits in executive function (Trail Making Test B-A) and scored higher in measures of depression and anxiety compared to those with PD and healthy controls. Widespread frontostriatal white matter tract reduction in fractional anisotropy was seen in MSA-P and PD compared to an imaging control group. Stroop Test interference performance correlated with [18F]-FDG uptake in the bilateral dorsolateral prefrontal cortex (DLPFC) and with white matter integrity between the striatum and left inferior frontal gyrus (IFG) in PD. Trail Making Test performance correlated with corticostriatal white matter integrity along tracts from the bilateral IFG in MSA-P and from the right DLPFC in both groups. CONCLUSION: Executive dysfunction was more prominent in patients with MSA-P compared to PD. DLPFC metabolism and frontostriatal white matter integrity seem to be a driver of executive function in PD, whereas alterations in corticostriatal white matter integrity may contribute more to executive dysfunction in MSA-P.

Distinct performance profiles on the Brixton test in frontotemporal dementia.

The Brixton Spatial Anticipation Test is a well-established test of executive function that evaluates the capacity to abstract, follow, and switch rules. There has been remarkably little systematic analysis of Brixton test performance in the prototypical neurodegenerative disorder of the frontal lobes: behavioural variant frontotemporal dementia (bvFTD) or evaluation of the test's ability to distinguish frontal from temporal lobe degenerative disease. We carried out a quantitative and qualitative analysis of Brixton performance in 76 patients with bvFTD and 34 with semantic dementia (SD) associated with temporal lobe degeneration. The groups were matched for demographic variables and illness duration. The bvFTD group performed significantly more poorly (U = 348, p < .0001, r = .58), 53% of patients scoring in the poor-impaired range compared with 6% of SD patients. Whereas bvFTD patients showed problems in rule acquisition and switching, SD patients did not, despite their impaired conceptual knowledge. Error analysis revealed more frequent perseverative errors in bvFTD, particularly responses unconnected to the stimulus, as well as random responses. Stimulus-bound errors were rare. Within the bvFTD group, there was variation in performance profile, which could not be explained by demographic, neurological, or genetic factors. The findings demonstrate sensitivity and specificity of the Brixton test in identifying frontal lobe degenerative disease and highlight the clinical value of qualitative analysis of test performance. From a theoretical perspective, the findings provide evidence that semantic knowledge and the capacity to acquire rules are dissociable. Moreover, they exemplify the separable functional contributions to executive performance.

Cognition and behaviour in frontotemporal dementia with and without amyotrophic lateral sclerosis.

OBJECTIVE: The precise relationship between frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) is incompletely understood. The association has been described as a continuum, yet data suggest that this may be an oversimplification. Direct comparisons between patients who have behavioural variant FTD (bvFTD) with and without ALS are rare. This prospective comparative study aimed to determine whether there are phenotypic differences in cognition and behaviour between patients with FTD-ALS and bvFTD alone. METHODS: Patients with bvFTD or FTD-ALS and healthy controls underwent neuropsychological testing, focusing on language, executive functions and social cognition. Behavioural change was measured through caregiver interview. Blood samples were screened for known FTD genes. RESULTS: 23 bvFTD, 20 FTD-ALS and 30 controls participated. On cognitive tests, highly significant differences were elicited between patients and controls, confirming the tests' sensitivities to FTD. bvFTD and FTD-ALS groups performed similarly, although with slightly greater difficulty in patients with ALS-FTD on category fluency and a sentence-ordering task that assesses grammar production. Patients with bvFTD demonstrated more widespread behavioural change, with more frequent disinhibition, impulsivity, loss of empathy and repetitive behaviours. Behaviour in FTD-ALS was dominated by apathy. The C9ORF72 repeat expansion was associated with poorer performance on language-related tasks. CONCLUSIONS: Differences were elicited in cognition and behaviour between bvFTD and FTD-ALS, and patients carrying the C9ORF72 repeat expansion. The findings, which raise the possibility of phenotypic variation between bvFTD and FTD-ALS, have clinical implications for early detection of FTD-ALS and theoretical implications for the nature of the relationship between FTD and ALS.

The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) in frontotemporal dementia.

OBJECTIVES: To examine the usefulness of the Edinburgh Cognitive and Behavioral Amyotrophic Lateral Sclerosis (ALS) Screen (ECAS) as a cognitive screening tool for the detection of behavioral variant frontotemporal dementia (bvFTD). A secondary aim was to determine whether people with FTD combined with ALS (ALS-FTD) exhibit a similar ECAS profile to that of people with bvFTD alone. Methods: Patients with ALS-FTD and bvFTD and healthy controls were recruited. Participants were administered the ECAS, which comprises tests of language, verbal fluency, executive functions, memory, and visual-spatial functions. They also carried out analogous, full-length cognitive tests that examine naming, spelling, sentence completion, and social cognition skills. Results: The study cohort comprised 20 ALS-FTD patients, 23 with bvFTD, and 30 controls. Highly significant group differences were elicited for all cognitive domains, reflecting poorer performance in patients compared to controls. No significant differences in overall test scores were found between ALS-FTD and bvFTD patients, although ALS-FTD patients showed a higher frequency of impairment on verbal fluency. Correlative analyses revealed inter-relationships in patients (but not controls) between scores in different domains, most marked in bvFTD. There were strong correlations between performance on ECAS subtests and analogous cognitive tasks. Conclusion: The ECAS is a sensitive and valuable tool for the assessment of FTD. Executive, language and behavioral breakdown may, however, compromise performance in other cognitive domains, reducing the specificity of the 'frontotemporal' cognitive profile. Subtle differences observed between ALS-FTD and bvFTD raise questions regarding the precise relationship between bvFTD with and without ALS.

Naming and conceptual understanding in frontotemporal dementia.

Behavioural variant frontotemporal dementia (bvFTD) is characterised by behaviour change and impaired executive skills. There is growing evidence that naming difficulties may also be present but the basis for these is unclear. A primary semantic deficit has been proposed, although executive contributions to naming breakdown are also possible. The study aimed to improve understanding of the naming disorder in bvFTD through direct comparison with semantic dementia (SD), and examination of neural correlates. It aimed also to address current controversies about the role of the anterior temporal lobes in semantic memory. We studied 71 bvFTD and 32 SD patients. Naming data were elicited by two picture naming tests (one challenging and one less demanding) and word comprehension by word-picture matching. Structural magnetic resonance images were rated blind using a standardised visual rating scale. Around half of bvFTD patients showed impaired naming and 17% impaired word-picture matching. Deficits in bvFTD were less severe than in SD, but showed a similar pattern. There were strong inverse correlations between naming scores and atrophy in temporal structures, particularly temporal pole and fusiform gyrus. Word comprehension scores correlated more strongly with posterior than anterior temporal lobe atrophy in SD. Error analysis highlighted a significant relationship in both groups between associative-type responses and temporal pole atrophy. By contrast, 'don't know' responses, suggesting a loss of conceptual knowledge, correlated with more posterior temporal regions. There was some correlation in bvFTD between naming and executive test performance but not with frontal lobe atrophy. The findings support the view that naming problems can arise in bvFTD independently of patients' 'frontal' executive impairment and highlight clinical overlap between bvFTD and SD. We discuss the findings in relation to the hub and spoke model of semantic memory and argue against the notion of an anterior temporal lobe semantic hub.

Outpatient visits versus telephone interviews for postoperative care: a randomized controlled trial.

INTRODUCTION AND HYPOTHESIS: Our aim was to determine whether postoperative telephone follow-up was noninferior to in-person clinic visits based on patient satisfaction. Secondary outcomes were safety and clinical outcomes. METHODS: Women scheduled for pelvic surgery were recruited from a single academic institution and randomized to clinic or telephone follow-up. The clinic group returned for visits 2, 6, and 12 weeks postoperatively and the telephone group received a call from a nurse at the same time intervals. Women completed the Consumer Assessment of Healthcare Providers and Systems Surgical Care Survey (S-CAHPS) questionnaire, Pelvic Floor Distress Inventory (PFDI)-20, and pain scales prior to and 3 months postoperatively. Randomized patients who completed the S-CAHPS at 3 months were included for analysis. Sample size calculations, based on a 15% noninferiority limit in the S-CAHPS global assessment surgeon rating, required 100 participants, with power = 80% and alpha = 0.025. RESULTS: From October 2016 to November 2017, 100 participants were consented, underwent surgery, were randomized, and included in the final analysis (clinic group n = 50, telephone group n = 50). Mean age was 58.5 ± 12.2 years. Demographic data and surgery type, dichotomized into outpatient and inpatient, did not differ between groups. The S-CAHPS global assessment surgeon rating from patients in the telephone group was noninferior to the clinic group (92 vs 88%, respectively, rated their surgeons 9 and10, with a noninferiority limit of 36.1; p = 0.006). Adverse events did not differ between groups (n = 26; 57% fclinic vs 43% telephone; p = 0.36). Patients in the telephone group did not require additional emergency room or primary care visits. Clinical outcome measures improved in both groups, with no differences (all p > 0.05). CONCLUSIONS: Telephone follow-up after pelvic floor surgery results in noninferior patient satisfaction, without differences in clinical outcomes or adverse events. Telephone follow-up may improve healthcare quality and decrease patient and provider burden for postoperative care. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov , www.clinicaltrials.gov , NCT02891187.

Neuropsychological differentiation of progressive aphasic disorders.

The differentiation of subtypes of primary progressive aphasia (PPA) remains challenging. We aimed to identify optimum neuropsychological measures for characterizing PPA, to examine the relationship between behavioural change and subtypes of PPA and to determine whether characteristic profiles of language, working memory, and behavioural changes occur in PPA. Forty-seven patients with PPA and multi-domain Alzheimer's disease (AD) together with 19 age-matched controls underwent a large battery of working memory and language tests. We found that simple tasks of sentence ordering, narrative production, and buccofacial praxis were particularly useful in differentiating non-fluent/agrammatic variant PPA (nfvPPA) from other PPA subtypes, whereas a test of single word comprehension was useful in detecting semantic dementia (SD). No individual tests were discriminating for logopenic variant PPA (lvPPA) relative to nfvPPA. LvPPA and multidomain AD exhibited similar language profiles. A principal components analysis revealed that characteristic PPA profiles extended beyond the realms of language, in particular, the presence of apraxia in nfvPPA, behavioural changes in SD, and working memory deficits in lvPPA. These findings suggest that not all tests are equally discriminatory for PPA and highlight the importance of a test profile in differentiating PPA. These results also support the view that lvPPA is a focal form of AD and emphasize the difficulties classifying lvPPA.

Which computer-use behaviours are most indicative of cognitive decline? Insights from an expert reference group.

Computer use is becoming ubiquitous among older adults. As computer use depends on complex cognitive functions, measuring individuals' computer-use behaviours over time may provide a way to detect changes in their cognitive functioning. However, it is uncertain which computer-use behaviour changes are most likely to be associated with declines of particular cognitive functions. To address this, we convened six experts from clinical and cognitive neurosciences to take part in two workshops and a follow-up survey to gain consensus on which computer-use behaviours would likely be the strongest indicators of cognitive decline. This resulted in a list of 21 computer-use behaviours that the majority of experts agreed would offer a 'strong indication' of decline in a specific cognitive function, across Memory, Executive function, Language and Perception and Action domains. This list enables a hypothesis-driven approach to analysing computer-use behaviours predicted to be markers of cognitive decline.

Trends in patient procurement of postoperative opioids and route of hysterectomy in the United States from 2004 through 2014.

BACKGROUND: The opioid epidemic in the United States is a public health emergency. Minimally invasive surgical technology has decreased length of hospital stay, improved postoperative recovery, and decreased postoperative pain. Hysterectomy is one of the most commonly performed surgeries in the United States. Increasing trends in minimally invasive gynecologic surgery are expected to reduce patients' postoperative pain. It is unclear whether this assumption has resulted in decreasing postoperative opioid prescriptions or patient need for these prescriptions, as prescribing patterns may be contributing to the current opioid public health emergency. OBJECTIVE: We sought to describe opioid prescribing and patient procurement practices for postoperative pain at time of discharge for benign hysterectomy from 2004 through 2014 using the Truven Health Analytics MarketScan Research Database. The trends of the route of hysterectomy over this time period were concomitantly described to reflect the movement toward more minimally invasive approaches. STUDY DESIGN: The Truven Health Analytics MarketScan Research Database including the Commercial Claims and Encounters Database, and the Medicare Supplemental and Coordination of Benefits Database were utilized. Current Procedural Terminology, 4th Edition, and International Classification of Diseases, Ninth Revision, codes identified all patients who underwent a hysterectomy for benign indications from 2004 through 2014. Hysterectomy routes were categorized into abdominal, laparoscopic, and vaginal. The MarketScan database captures prescriptions filled at a retail or mail-order pharmacy and does not capture prescriptions filled within the inpatient, hospital facility. The days of opioids procured by patients at the time of discharge were identified for each encounter. Descriptive statistics were used to summarize data within the entire study period. Although this article is purely descriptive, further analyses were conducted for exploratory purposes only. analysis of variance and χ2 analyses were used for continuous and categorical variables, respectively. Multiple linear regression models were used to describe associations between variables of interest and postoperative opioid prescriptions. RESULTS: We identified 793,016 patients who underwent a hysterectomy for benign indications from 2004 through 2014. Of these, 96% were identified from the Commercial Claims and Encounters Database. During the study period, the overall route of hysterectomy was categorized into 40.5% abdominal, 42.0% laparoscopic, and 17.5% vaginal hysterectomy. The route of hysterectomy changed from 60.2-25.6% (a decrease of Δ = 34.58; 95% confidence interval, 33.96-35.20) for abdominal, 17.0-61.9% (an increase of Δ = 44.83; 95% confidence interval, 44.21-45.44) for laparoscopic, and 22.8-12.6% (a decrease of Δ = 10.25; 95% confidence interval, 9.77-10.73) for vaginal. At discharge, the percentage of patients who were prescribed opioids and filled them increased from 25.6-82.1% (an increase of Δ = 56.50; 95% confidence interval, 55.88-57.13 with P < .001) from 2004 through 2014 for all hysterectomy routes. Additionally, the quantity of opioids prescribed also increased. CONCLUSION: Opioid prescriptions filled for postoperative pain after hysterectomy substantially increased from 2004 through 2014. Opioid prescription procurement has increased despite a concomitant increase in minimally invasive hysterectomy routes. In light of the current opioid epidemic, physicians must recognize that postoperative prescribing practices may contribute to chronic opioid use. Heightened awareness of opioid prescribing practices following surgery is critically important to decrease risk of development of chronic opioid dependence.

Semantic dementia and the left and right temporal lobes.

Semantic dementia, a circumscribed disorder of semantic knowledge, provides a unique model for understanding the neural basis for semantic representation. The study addressed areas of contention: the relative roles of the left and right temporal lobe, the contribution of anterior versus posterior temporal cortex and the status of the anterior temporal lobes as amodal hub. Naming and word comprehension was examined in 41 semantic dementia patients, 31 with left-predominant and 10 right-predominant atrophy. In keeping with expectation, naming and comprehension were significantly poorer in left-predominant patients. Structural magnetic resonance image analysis, using a visual rating scale, showed strong inverse correlations between naming scores and severity of both left anterior and posterior temporal lobe atrophy. By contrast, comprehension performance was more strongly correlated with left posterior temporal atrophy. Analysis of naming errors revealed a correlation between anterior temporal atrophy and associative/functional descriptive responses, implying availability of semantic information. By contrast, 'don't know' responses, indicative of loss of semantic knowledge, were linked to left posterior temporal lobe atrophy. Semantic errors, the hallmark of semantic dementia, were linked to right hemisphere atrophy, especially the right posterior temporal lobe. Matched visual-verbal tasks (famous face and name identification, Pyramids and Palm trees pictures and words, animal knowledge from 3-D models and animal names) administered to nine patients elicited variable correspondence between performance on nonverbal and verbal versions of the task. Marked performance dissociations were demonstrated in some patients: poorer understanding of names/words in left-predominant patients and of faces/pictures/models in right-predominant cases. The findings are compatible with the notion of the anterior temporal lobes as areas of convergence, but are less easily accommodated within the framework of amodal conceptual representation. The data, which reconcile some apparent contradictions in the literature, are discussed in the light of the nature and distribution of degenerative change in semantic dementia.

Metabolic regional and network changes in Alzheimer's disease subtypes.

Clinical variants of Alzheimer's disease (AD) include the common amnestic subtype as well as subtypes characterised by leading visual processing impairments or by multimodal neurocognitive deficits. We investigated regional metabolic patterns and networks between AD subtypes. The study comprised 9 age-matched controls and 25 patients with mild to moderate AD. Methods included clinical and neuropsychological assessment, high-resolution FDG PET and T1-weighted 3D MR imaging with PET-MR coregistration, grey matter segmentation, atlas-based regions-of-interest, linear mixed effects and regional correlation analysis. Regional metabolic patterns differed significantly between groups, but significant hypometabolism in the posterior cingulate cortex (PCC) was common to all subtypes. The most distinctive regional abnormality was occipital hypometabolism in the visual subtype. In controls, two large clusters of positive regional metabolic correlations were observed. The most pronounced breakdown of the normal correlation pattern was found in amnestic patients who, in contrast, showed the least regional focal metabolic deficits. The normal positive correlation between PCC and hippocampus was lost in all subtypes. In conclusion, PCC hypometabolism and metabolic correlation breakdown between PCC and hippocampus are the common functional core of all AD subtypes. Network alterations exceed focal regional impairment and are most prominent in the amnestic subtype.

Functional neuroanatomical associations of working memory in early-onset Alzheimer's disease.

OBJECTIVE: To characterize metabolic correlates of working memory impairment in clinically defined subtypes of early-onset Alzheimer's disease. BACKGROUND: Established models of working memory suggest a key role for frontal lobe function, yet the association in Alzheimer's disease between working memory impairment and visuospatial and language symptoms suggests that temporoparietal neocortical dysfunction may be responsible. METHODS: Twenty-four patients with predominantly early-onset Alzheimer's disease were clinically classified into groups with predominantly amnestic, multidomain or visual deficits. Patients underwent neuropsychological evaluation focused on the domains of episodic and working memory, T1-weighted magnetic resonance imaging and brain fluorodeoxyglucose positron emission tomography. Fluorodeoxyglucose positron emission tomography data were analysed by using a region-of-interest approach. RESULTS: Patients with multidomain and visual presentations performed more poorly on tests of working memory compared with amnestic Alzheimer's disease. Working memory performance correlated with glucose metabolism in left-sided temporoparietal, but not frontal neocortex. Carriers of the apolipoprotein E4 gene showed poorer episodic memory and better working memory performance compared with noncarriers. CONCLUSIONS: Our findings support the hypothesis that working memory changes in early-onset Alzheimer's disease are related to temporoparietal rather than frontal hypometabolism and show dissociation from episodic memory performance. They further support the concept of subtypes of Alzheimer's disease with distinct cognitive profiles due to prominent neocortical dysfunction early in the disease course. Copyright © 2017 John Wiley & Sons, Ltd.

Cognitive decline in Huntington's disease expansion gene carriers.

BACKGROUND: In Huntington's Disease (HD) cognitive decline can occur before unequivocal motor signs become apparent. As cognitive decline often starts early in the course of the disease and has a progressive nature over time, cognition can be regarded as a key target for symptomatic treatment. The specific progressive profile of cognitive decline over time is unknown. OBJECTIVE: The aim of this study is to quantify the progression of cognitive decline across all HD stages, from pre-motormanifest to advanced HD, and to investigate if CAG length mediates cognitive decline. METHODS: In the European REGISTRY study 2669 HD expansion gene carriers underwent annual cognitive assessment. General linear mixed models were used to model the cognitive decline for each cognitive task across all disease stages. Additionally, a model was developed to evaluate the cognitive decline based on CAG length and age rather than disease stage. RESULTS: There was significant cognitive decline on all administered tasks throughout pre-motormanifest (close to estimated disease onset) participants and the subsequent motormanifest participants from stage 1 to stage 4. Performance on the Stroop Word and Stroop Color tests additionally declined significantly across the two pre-motormanifest groups: far and close to estimated disease onset. The evaluation of cognition performance in relation to CAG length and age revealed a more rapid cognitive decline in participants with longer CAG length than participants with shorter CAG length over time. CONCLUSION: Cognitive performance already shows decline in pre-motormanifest HD gene expansion carriers and gradually worsens to late stage HD. HD gene expansion carriers with certain CAG length have their own cognitive profile, i.e., longer CAG length is associated with more rapid decline.

Examining the language and behavioural profile in FTD and ALS-FTD.

BACKGROUND: A proportion of patients with behavioural variant frontotemporal dementia (bvFTD) develop amyotrophic lateral sclerosis (ALS). It is currently unknown whether the behavioural and cognitive syndrome in bvFTD with ALS (ALS-FTD) is indistinguishable from that of bvFTD alone. METHODS: A retrospective cohort of 241 patients with clinical diagnoses of bvFTD (n=185) or ALS-FTD (n=56) was examined with respect to behavioural, cognitive and neuropsychiatric symptoms. Features were rated as present or absent based on information recorded from clinical interviews and detailed neuropsychological assessment. RESULTS: A number of behavioural and affective changes were reported more frequently in bvFTD than ALS-FTD: social disinhibition (p<0.001), inertia (p<0.001), loss of sympathy and empathy (p=0.008), repetitive behaviours (p<0.001) and dietary changes (p<0.001). Warmth of affect demonstrated in the clinic setting was reported more often in ALS-FTD than bvFTD (p<0.001). Executive impairments occurred equally in both groups. Language impairments were more common in ALS-FTD than bvFTD: agrammatism (p<0.017) and impaired sentence comprehension (p<0.036). Psychotic features were relatively rare and did not distinguish the groups. CONCLUSIONS: Our findings suggest differences between bvFTD and ALS-FTD. In particular, while changes in social behaviour are prominent in bvFTD alone, there may be a comparatively greater degree of language impairment in ALS-FTD. Prospective exploration of the pattern of differences between these groups will be essential. Identification of a distinct neuropsychological phenotype in ALS-FTD may have clinical implications for early diagnosis, disease management and care planning and theoretical implications for our understanding of the relationship between ALS and FTD.