Occult breast cancer (OBC) is described as an axillary metastatic carcinoma without detection of a primary breast lesion and is uncommon. Significant advances in breast imaging have occurred since ...its description, decreasing its incidence. However current management is based upon old studies, with variable clinical, radiological and pathological definitions of OBC. We suggest standardised definitions of OBC to facilitate more homogenous data representation in the literature. This review also discusses the conflicting heterogeneous data and its influence in determining the current management guidelines. We discuss whether the current significant surgical recommendations are necessary and postulate whether they could be safely substituted with less invasive management.
•Pathological occult breast cancer is defined as no lesion detectable on MRI or pathological breast lesion post-surgery when examined at 5mm slices•NAC in OBC may result in the de-escalation of axillary surgery.•Current evidence indicates that ALND with ipsilateral breast RT has equivalent outcomes compared to ALND with mastectomy
Fathers' support can influence mothers' breastfeeding decisions and behavior. Potentially supportive behaviors have been reported in previous studies, but no studies have directly examined which, if ...any, of those actions are actually more likely to result in desired breastfeeding outcomes. The two studies reported in this paper address this gap by examining relationships between fathers' reported breastfeeding support and mothers' perceptions of received support and breastfeeding intentions, satisfaction, and duration. The Partner Breastfeeding Influence Scale (PBIS) was used in an online survey with 64 women and 41 men (34 couples) and a telephone survey with 80 mothers and 65 fathers (63 couples). Fathers' and mothers' reports of how often fathers engage in the types of support measured by the PBIS were used to predict breastfeeding intentions, satisfaction, and duration. In Study 1, responsiveness predicted breastfeeding success and satisfaction for men and satisfaction for women. However, mothers' intended breastfeeding duration was shorter when fathers both wanted them to breastfeed for a long time and were more appreciative and savvy about breastfeeding. In Study 2, when fathers reported being more appreciative and directly involved in breastfeeding, mothers reported shorter breastfeeding duration. In both studies, mothers' perceptions of their partners' responsiveness and fathers' reports of their own responsiveness predicted longer breastfeeding intentions and duration. These findings suggest that the most effective breastfeeding support is delivered using a sensitive, coordinated teamwork approach that is responsive to the mother's needs.
Over the past decade, seafood mislabeling has been increasingly documented, raising public concern over the identity, safety, and sustainability of seafood. Negative outcomes from seafood mislabeling ...are suspected to be substantial and pervasive as seafood is the world’s most highly traded food commodity. Here we provide empirical systems-level evidence that enabling conditions exist for seafood mislabeling in the United States (US) to lead to negative impacts on marine populations and support consumption of products from poorly managed fisheries. Using trade, production, and mislabeling data, we determine that substituted products are more likely to be imported than the product listed on the label. We also estimate that about 60% of US mislabeled apparent consumption associated with the established pairs involves products that are exclusively wild caught. We use these wild-caught pairs to explore population and management consequences of mislabeling. We find that, compared to the product on the label, substituted products come from fisheries with less healthy stocks and greater impacts of fishing on other species. Additionally, substituted products are from fisheries with less effective management and with management policies less likely to mitigate impacts of fishing on habitats and ecosystems compared with the label product. While we provide systematic evidence of environmental impacts from food fraud, our results also highlight the current challenges with production, trade, and mislabeling data, which increase the uncertainty surrounding seafood mislabeling consequences. More integrated, holistic, and collaborative approaches are needed to understand mislabeling impacts and design interventions to minimize mislabeling.
The emphasis in early minstrelsy studies has often neglected Black women and the caricature used to represent them, the wench. The wench was the performance of Black women by White men on stage, ...either through cross-dressed, drag performance, or referenced in skits, songs, and advertisements. Although cross-dressing and the homoerotic nature of those drag performances in the minstrel show has been the focus of some scholarship, the construction of Black women in this popular form of entertainment has been neglected. The early labels of Black women in the U.S. historically fell into two main categories: jezebel and mammy. Each of these labels transverse both fictional and nonfictional Black women and were continually reinforced in popular culture through literature, theater, advertisements, and later, television and film.
Background: Chronic glial dysfunction may contribute to the pathogenesis of frontotemporal dementia (FTD). Cerebrospinal fluid (CSF) levels of glia-derived proteins YKL-40 and chitotriosidase are ...increased in Alzheimer’s disease (AD) but have not been explored in detail across the spectrum of FTD. Methods: We investigated whether CSF YKL-40 and chitotriosidase levels differed between FTD patients and controls, across different clinical and genetic subtypes of FTD, and between individuals with a clinical FTD syndrome due to AD versus non-AD (frontotemporal lobar degeneration, FTLD) pathology (based on CSF neurodegenerative biomarkers). Eighteen healthy controls and 64 people with FTD (behavioural variant FTD, n = 20; primary progressive aphasia PPA, n = 44: nfvPPA, n = 16, svPPA, n = 11, lvPPA, n = 14, PPA-NOS, n = 3) were included. 10/64 had familial FTD, with mutations in GRN(n = 3), MAPT(n = 4), or C9orf72 (n = 3). 15/64 had neurodegenerative biomarkers consistent with AD pathology. Levels were measured by immunoassay and compared using multiple linear regressions. We also examined relationships of YKL-40 and chitotriosidase with CSF total tau (T-tau), phosphorylated tau 181 (P-tau) and β-amyloid 1–42 (Aβ42), with each other, and with age and disease duration. Results: CSF YKL-40 and chitotriosidase levels were higher in FTD, particularly lvPPA (both) and nfvPPA (YKL-40), compared with controls. GRN mutation carriers had higher levels of both proteins than controls and C9orf72 expansion carriers, and YKL-40 was higher in MAPT mutation carriers than controls. Individuals with underlying AD pathology had higher YKL-40 and chitotriosidase levels than both controls and those with likely FTLD pathology. CSF YKL-40 and chitotriosidase levels were variably associated with levels of T-tau, P-tau and Aβ42, and with each other, depending on clinical syndrome and underlying pathology. CSF YKL-40 but not chitotriosidase was associated with age, but not disease duration. Conclusion: CSF YKL-40 and chitotriosidase levels are increased in individuals with clinical FTD syndromes, particularly due to AD pathology. In a preliminary analysis of genetic groups, levels of both proteins are found to be highly elevated in FTD due to GRN mutations, while YKL-40 is increased in individuals with MAPT mutations. As glia-derived protein levels generally correlate with T-tau and P-tau levels, they may reflect the glial response to neurodegeneration in FTLD.
Impaired processing of emotional signals is a core feature of frontotemporal dementia syndromes, but the underlying neural mechanisms have proved challenging to characterize and measure. Progress in ...this field may depend on detecting functional changes in the working brain, and disentangling components of emotion processing that include sensory decoding, emotion categorization and emotional contagion. We addressed this using functional MRI of naturalistic, dynamic facial emotion processing with concurrent indices of autonomic arousal, in a cohort of patients representing all major frontotemporal dementia syndromes relative to healthy age-matched individuals. Seventeen patients with behavioural variant frontotemporal dementia four female; mean (standard deviation) age 64.8 (6.8) years, 12 with semantic variant primary progressive aphasia four female; 66.9 (7.0) years, nine with non-fluent variant primary progressive aphasia five female; 67.4 (8.1) years and 22 healthy controls 12 female; 68.6 (6.8) years passively viewed videos of universal facial expressions during functional MRI acquisition, with simultaneous heart rate and pupillometric recordings; emotion identification accuracy was assessed in a post-scan behavioural task. Relative to healthy controls, patient groups showed significant impairments (analysis of variance models, all P < 0.05) of facial emotion identification (all syndromes) and cardiac (all syndromes) and pupillary (non-fluent variant only) reactivity. Group-level functional neuroanatomical changes were assessed using statistical parametric mapping, thresholded at P < 0.05 after correction for multiple comparisons over the whole brain or within pre-specified regions of interest. In response to viewing facial expressions, all participant groups showed comparable activation of primary visual cortex while patient groups showed differential hypo-activation of fusiform and posterior temporo-occipital junctional cortices. Bi-hemispheric, syndrome-specific activations predicting facial emotion identification performance were identified (behavioural variant, anterior insula and caudate; semantic variant, anterior temporal cortex; non-fluent variant, frontal operculum). The semantic and non-fluent variant groups additionally showed complex profiles of central parasympathetic and sympathetic autonomic involvement that overlapped signatures of emotional visual and categorization processing and extended (in the non-fluent group) to brainstem effector pathways. These findings open a window on the functional cerebral mechanisms underpinning complex socio-emotional phenotypes of frontotemporal dementia, with implications for novel physiological biomarker development.
Pelizaeus-Merzbacher disease is a rare X-linked leukodystrophy accompanied by central nervous system hypomyelination with a spectrum of clinical phenotypes. This is the first survey of caregivers of ...individuals with Pelizaeus-Merzbacher disease to investigate the presenting symptoms, path to diagnosis, identity and impact of most bothersome symptoms, and needs that future treatment should address. One hundred participants completed the survey. Results from this survey demonstrate that the majority of Pelizaeus-Merzbacher disease symptoms manifest before 2 years of age and commonly include deficits in gross and fine motor skills, speech, and communication. Caregivers rated difficulty crawling, standing, or walking as the most bothersome symptoms due to Pelizaeus-Merzbacher disease, with constipation and difficulty with sleep, manual dexterity, and speech and communication rated nearly as high. The most important treatment goals for caregivers were improved mobility and communication. The survey findings present a caregiver perspective of the impact of symptoms in Pelizaeus-Merzbacher disease and provide helpful guidance to affected families, physicians, and drug developers on the often-long path to diagnosis and the unmet medical needs of this patient population.
IntroductionThe term frontotemporal dementia (FTD) refers to a heterogeneous group of neurodegenerative disorders affecting the frontal and temporal lobes. Cognitively, impairment of executive ...function and social cognition predominates across the FTD spectrum, although other domains can be affected. Traditionally, cognition is tested through standard ‘pen and paper’ tasks in FTD. However, recent attempts have been made across other neurodegenerative disorders such as Alzheimer’s disease to develop computerised batteries that allow more accurate and sensitive detection of cognitive impairment.Methods and analysisThis paper describes the development of a novel battery of tests for a tablet computer, particularly focused on FTD. It consists of 12 different tasks which aim to tap into information processing speed, various aspects of executive function, social cognition, semantic knowledge, calculation and visuospatial skills. Future studies will focus on validating the battery in a healthy control cohort, comparing it against a standard ‘pen and paper’ psychometric battery, and finally testing it within an FTD cohort, including those with genetic forms of FTD where we will be able to assess its ability to detect very early cognitive deficits prior to the onset of symptoms.Ethics and disseminationNormative data will be produced in the initial validation study (approved by the UCL Ethics Committee, project ID 17691/002) and will be made available online.
Laughter is a fundamental communicative signal in our relations with other people and is used to convey a diverse repertoire of social and emotional information. It is therefore potentially a useful ...probe of impaired socio-emotional signal processing in neurodegenerative diseases. Here we investigated the cognitive and affective processing of laughter in forty-seven patients representing all major syndromes of frontotemporal dementia, a disease spectrum characterised by severe socio-emotional dysfunction (twenty-two with behavioural variant frontotemporal dementia, twelve with semantic variant primary progressive aphasia, thirteen with nonfluent-agrammatic variant primary progressive aphasia), in relation to fifteen patients with typical amnestic Alzheimer's disease and twenty healthy age-matched individuals. We assessed cognitive labelling (identification) and valence rating (affective evaluation) of samples of spontaneous (mirthful and hostile) and volitional (posed) laughter versus two auditory control conditions (a synthetic laughter-like stimulus and spoken numbers). Neuroanatomical associations of laughter processing were assessed using voxel-based morphometry of patients' brain MR images. While all dementia syndromes were associated with impaired identification of laughter subtypes relative to healthy controls, this was significantly more severe overall in frontotemporal dementia than in Alzheimer's disease and particularly in the behavioural and semantic variants, which also showed abnormal affective evaluation of laughter. Over the patient cohort, laughter identification accuracy was correlated with measures of daily-life socio-emotional functioning. Certain striking syndromic signatures emerged, including enhanced liking for hostile laughter in behavioural variant frontotemporal dementia, impaired processing of synthetic laughter in the nonfluent-agrammatic variant (consistent with a generic complex auditory perceptual deficit) and enhanced liking for numbers (‘numerophilia’) in the semantic variant. Across the patient cohort, overall laughter identification accuracy correlated with regional grey matter in a core network encompassing inferior frontal and cingulo-insular cortices; and more specific correlates of laughter identification accuracy were delineated in cortical regions mediating affective disambiguation (identification of hostile and posed laughter in orbitofrontal cortex) and authenticity (social intent) decoding (identification of mirthful and posed laughter in anteromedial prefrontal cortex) (all p < .05 after correction for multiple voxel-wise comparisons over the whole brain). These findings reveal a rich diversity of cognitive and affective laughter phenotypes in canonical dementia syndromes and suggest that laughter is an informative probe of neural mechanisms underpinning socio-emotional dysfunction in neurodegenerative disease.
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