Characterizing sexual behavior in frontotemporal dementia

Background: Frontotemporal dementia (FTD) is characterized by a number of prominent behavioral changes. While FTD has been associated with the presence of aberrant or unusual sexual behaviors in a proportion of patients, few studies have formally investigated changes in sexual function in this disease. Objective: We aimed to systematically quantify changes in sexual behavior, including current symptoms and changes from prior diagnoses, in behavioral-variant (bvFTD) and semantic dementia (SD), compared to Alzheimer’s disease (AD). Methods: Carers of 49 dementia patients (21 bvFTD, 11 SD, 17 AD) were interviewed using the Sexual Behavior and Intimacy Questionnaire (SIQ), a survey designed to assess changes in sexual function across multiple domains including initiating, level of affection, and aberrant or unusual sexual behavior. Results: BvFTD patients show prominent hyposexual behavior including decreased affection, initiation, and response to advances by partners, and decreased frequency of sexual relations, compared to AD and to SD patients. The greatest changes in sexual behavior compared to pre-diagnoses were found in the bvFTD group with a 90–100% decrease in initiation, response, and frequency of sexual relations. Notably, aberrant or unusual sexual behavior was reported in a minority of bvFTD and SD patients and occurred in patients who also showed hyposexual behavior toward their partner. Conclusion: Overall loss of affection, reduced initiation of sexual activity, and responsiveness is an overwhelming feature of bvFTD. In contrast, aberrant or unusual sexual behavior is observed in the minority of bvFTD patients. The underlying pathophysiology of these changes likely reflects structural and functional changes in frontoinsular and limbic regions including the hypothalamus

Aberrant meiotic behavior in Agave tequilana Weber var. azul

BACKGROUND: Agave tequilana Weber var. azul, is the only one variety permitted by federal law in México to be used for tequila production which is the most popular contemporary alcoholic beverage made from agave and recognized worldwide. Despite the economic, genetic, and ornamental value of the plant, it has not been subjected to detailed cytogenetic research, which could lead to a better understanding of its reproduction for future genetic improvement. The objective of this work was to study the meiotic behavior in pollen mother cells and its implications on the pollen viability in Agave tequilana Weber var. azul. RESULTS: The analysis of Pollen Mother Cells in anaphase I (A-I) showed 82.56% of cells with a normal anaphase and, 17.44% with an irregular anaphase. In which 5.28% corresponded to cells with side arm bridges (SAB); 3.68% cells with one bridge and one fragment; 2.58% of irregular anaphase showed cells with one or two lagging chromosomes and 2.95% showed one acentric fragment; cells with two bridges and cells with two bridges and one acentric fragment were observed in frequencies of 1.60% and 1.35% respectively. In anaphase II some cells showed bridges and fragments too. Aberrant A-I cells had many shrunken or empty pollen grains (42.00%) and 58.00 % viable pollen. CONCLUSION: The observed meiotic irregularities suggest that structural chromosome aberrations have occurred, such as heterozygous inversions, sister chromatid exchanges, deletions and duplications which in turn are reflected in a low pollen viability

Targeting the Stress System During Gestation: Is Early Handling a Protective Strategy for the Offspring?

The perinatal window is a critical developmental time when abnormal gestational stimuli may alter the development of the stress system that, in turn, influences behavioral and physiological responses in the newborns. Individual differences in stress reactivity are also determined by variations in maternal care, resulting from environmental manipulations. Despite glucocorticoids are the primary programming factor for the offspring’s stress response, therapeutic corticosteroids are commonly used during late gestation to prevent preterm negative outcomes, exposing the offspring to potentially aberrant stress reactivity later in life. Thus, in this study, we investigated the consequences of one daily s.c. injection of corticosterone (25 mg/kg), from gestational day (GD) 14–16, and its interaction with offspring early handling, consisting in a brief 15-min maternal separation until weaning, on: (i) maternal behavior; and (ii) behavioral reactivity, emotional state and depressive-like behavior in the adolescent offspring. Corticosterone plasma levels, under non-shock- and shock-induced conditions, were also assessed. Our results show that gestational exposure to corticosterone was associated with diminished maternal care, impaired behavioral reactivity, increased emotional state and depressive-like behavior in the offspring, associated with an aberrant corticosterone response. The early handling procedure, which resulted in increased maternal care, was able to counteract the detrimental effects induced by gestational corticosterone exposure both in the behavioral- and neurochemical parameters examined. These findings highlight the potentially detrimental consequences of targeting the stress system during pregnancy as a vulnerability factor for the occurrence of emotional and affective distress in the adolescent offspring. Maternal extra-care proves to be a protective strategy that confers resiliency and restores homeostasis

Caregiver's distress related to the patient's neuropsychiatric symptoms as a function of the care-setting

[Abstract] Prevalence of neuropsychiatric symptoms (NPS) and correlation between its intensity and caregiver distress were evaluated as a function of the care-setting in a cross-sectional study including 72 patient-caregiver dyads. The Neuropsychiatric Inventory and the Caregiver Distress Scale were administered. The most prevalent symptoms were: in the formal care-setting, agitation/aggression and depression/dysphoria (42.4%), and in the informal care-setting, aberrant motor behavior (59.0%). While changes in appetite and eating behaviors, depression/dysphoria and irritability/lability were more prevalent in the formal care-setting (p < .0001, p = .011, p = .021), aberrant motor behavior was more prevalent in the informal care-setting (p = .007). NPS were positively correlated with caregiver distress. High patients' scores in hallucinations, sleep and night-time disturbances, anxiety, and aberrant motor behavior were the best predictors of professional caregiver's distress. Agitation/aggression, delusions, disinhibition, apathy/indifference, depression/dysphoria, and elation/euphoria were the best predictors of informal caregiver's distress. Findings may have important clinical implications

An exploratory investigation of aberrant consumer behaviour in Libya: a sociocultural approach

Studies concerning aberrant consumer behaviour (ACB) are dominated by research conducted in the West. By examining the impact social and cultural factors have on the management and understanding of ACB in Libya, a Muslim country, this paper extends knowledge by exploring this issue in a different setting. Materials were collected by conducting in-depth interviews with 26 sellers in Libya and ACB was explored in three different contexts: grocery stores, computer stores and hotels. The study finds that the sellers use alternative marketplace behaviours to manage ACB to that described in the literature, namely informal, community based approaches which reflect accepted societal and cultural norms. Furthermore, the study finds that not all activities reported to be ACB in the literature are perceived to be misbehaviour by the Libyan sellers

On the origin of trisomy 21 Down syndrome

Background: Down syndrome, characterized by an extra chromosome 21 is the most common genetic cause for congenital malformations and learning disability. It is well known that the extra chromosome 21 most often originates from the mother, the incidence increases with maternal age, there may be aberrant maternal chromosome 21 recombination and there is a higher recurrence in young women. In spite of intensive efforts to understand the underlying reason(s) for these characteristics, the origin still remains unknown. We hypothesize that maternal trisomy 21 ovarian mosaicism might provide the major causative factor. Results: We used fluorescence in situ hybridization (FISH) with two chromosome 21-specific probes to determine the copy number of chromosome 21 in ovarian cells from eight female foetuses at gestational age 14–22 weeks. All eight phenotypically normal female foetuses were found to be mosaics, containing ovarian cells with an extra chromosome 21. Trisomy 21 occurred with about the same frequency in cells that had entered meiosis as in pre-meiotic and ovarian mesenchymal stroma cells. Conclusion: We suggest that most normal female foetuses are trisomy 21 ovarian mosaics and the maternal age effect is caused by differential selection of these cells during foetal and postnatal development until ovulation. The exceptional occurrence of high-grade ovarian mosaicism may explain why some women have a child with Down syndrome already at young age as well as the associated increased incidence at subsequent conceptions. We also propose that our findings may explain the aberrant maternal recombination patterns previously found by family linkage analysis

A Mutant \u3cem\u3edec-1\u3c/em\u3e Transgene Induces Dominant Female Sterility in \u3cem\u3eDrosophila melanogaster\u3c/em\u3e

The Drosophila dec-1 gene produces three proproteins required for female fertility and eggshell assembly. The three proproteins are distinguished by their C termini. Fc106, the most abundant proprotein, is cleaved within the vitelline membrane to three mature derivatives in a developmentally regulated manner. To define sequences within fc106 that are critical for its function, we created wild-type and mutant versions of an fc106 cDNA transgene. The functional consequences of the mutations were assessed in dec-14, a female-sterile splicing mutant that does not produce the fc106 isoform. The fertility of dec-14 females was restored by the introduction of either a wild-type transgene or a transgene bearing a C-terminal deletion that included fc106-specific sequences. Surprisingly, the removal of internal coding sequences created an aberrant DEC-1 proprotein that induced female sterility when introduced into wild-type flies. Dominant female sterility was not associated with larger deletions that included the fc106 N terminus, suggesting that abnormal juxtaposition of N- and C-terminal sequences in the aberrant proprotein interfered with endogenous DEC-1 proteins. Changes in the fractionation behavior of the endogenous fc106 C-terminal derivative, s60, and morphological changes in the endochorion in response to expression of the aberrant proprotein support this interpretation

A pilot evaluation of positive behavioural support workshops for families of adults with intellectual disabilities who present challenging behaviour: 'It should have been offered years ago'

Background: Positive behavioural support (PBS) workshops were designed for families supporting their adult son or daughter with an intellectual disability engaging in challenging behaviours. This was in recognition of the impact of their challenging behaviour, in particular the level of stress carers often experience and positive reports from other studies incorporating multi-family learning. Method and materials: Two sets of pilot workshops were completed, incorporating modules in PBS and physical interventions. Workshops included presentations and self-directed/group learning tasks, recorded in a specifically designed workbook. Results: Quantitative data was analysed using non-parametric tests. Positive results were found for changes in various areas including self-reported stress levels. Positive trends were also found in pre and post application of the Aberrant Behaviour Checklist (Aman and Singh, 1994). Conclusions: This study showed very encouraging results in regard to the impact of this model of training for the carers involved. Further research is required to assess any longer term effects, and to explore the impact on larger numbers of participants