A caregiver's perspective on clinically relevant symptoms in behavioural variant frontotemporal dementia: tools for disease management and trial design.

BACKGROUND: Adequate detection of symptoms and disease progression in behavioural variant frontotemporal dementia (bvFTD) is complex. Dementia cohorts usually utilize cognitive and functional measures, which fail to detect dominant behavioural and social cognitive deficits in bvFTD. Moreover, since patients typically have a loss of insight, caregivers are important informants. This is the first qualitative study to investigate caregiver relevant symptoms during the disease course of bvFTD, aiming to improve tools for diagnosis, progression, and future clinical trials. METHODS: Informal caregivers of patients in different disease stages of bvFTD (N = 20) were recruited from the neurology outpatient clinic of the Amsterdam UMC and a patient organization for peer support in the Netherlands. Their perspectives on clinical relevance were thoroughly explored during individual semi-structured interviews. Inductive content analysis with open coding was performed by two researchers independently to establish overarching themes and patterns. RESULTS: Caregivers reported a variety of symptoms, in which (i) loss of emotional connection, (ii) preoccupation and restlessness, and (iii) apathy and dependency compose major themes of relevance for diagnosis and treatment. Within heterogeneous disease trajectories, symptom presence differed between stages and among individuals, which is relevant in the context of progression and outcome measures. Significant socio-emotional changes dominated in early stages, while severe cognitive, behavioural, and physical deterioration shifted focus from predominant personality change to quality of life in later stages. CONCLUSIONS: Caregiver perspectives on target symptoms in bvFTD differ according to clinical stage and patient-caregiver characteristics, with significant socio-emotional changes characterizing early stages. These findings call for more appropriate tools and symptomatic treatments, as well as a personalized approach in treatment of bvFTD and a focus on early stage interventions in clinical trial design.

Estrogen receptor ß actions in the female cardiovascular system: A systematic review of animal and human studies.

Five medical databases were searched for studies that assessed the role of ERß in the female cardiovascular system and the influence of age and menopause on ERß functioning. Of 9472 references, 88 studies met our inclusion criteria (71 animal model experimental studies, 15 human model experimental studies and 2 population based studies). ERß signaling was shown to possess vasodilator and antiangiogenic properties by regulating the activity of nitric oxide, altering membrane ionic permeability in vascular smooth muscle cells, inhibiting vascular smooth muscle cell migration and proliferation and by regulating adrenergic control of the arteries. Also, a possible protective effect of ERß signaling against left ventricular hypertrophy and ischemia/reperfusion injury via genomic and non-genomic pathways was suggested in 27 studies. Moreover, 5 studies reported that the vascular effects of ERß may be vessel specific and may differ by age and menopause status. ERß seems to possess multiple functions in the female cardiovascular system. Further studies are needed to evaluate whether isoform-selective ERß-ligands might contribute to cardiovascular disease prevention.

High prevalence of incomplete right bundle branch block in facioscapulohumeral muscular dystrophy without cardiac symptoms.

The exact prevalence and nature of cardiac involvement in facioscapulohumeral muscular dystrophy (FSHD) is unknown. Nevertheless, the current opinion is that symptomatic cardiac disease is rare. We performed a cardiac screening [electrocardiogram (ECG) and echocardiography in the event of ECG abnormalities] in 75 genetically confirmed, ambulant FSHD patients without cardiac symptoms, with an eight-year follow-up of 57 patients, and compared the findings with results of previously performed cardiac screenings in the normal population. Baseline ECG demonstrated incomplete right bundle branch block (RBBB) in 33%, complete RBBB in 4%, and other minor abnormalities in 16%. Echocardiography showed no abnormalities. No significant changes were found after eight years of follow-up. Comparison with ECG abnormalities in the normal population showed a higher prevalence of incomplete RBBB (9.7 times higher) and of complete RBBB (4.8 times higher) in FSHD patients. This study in cardiac asymptomatic FSHD patients shows i) increased prevalence of incomplete RBBB in the absence of cardiomyopathy; ii) no progression of these abnormalities during eight years of follow-up. We conclude that FSHD patients without cardiac complaints do not need specific cardiac screening or surveillance. Furthermore, the increased prevalence of incomplete RBBB in the absence of cardiomyopathy suggests a selective involvement of the His-Purkinje system in FSHD.

Cognition Improvement in Taekwondo Novices Over 40. Results from the SEKWONDO Study.

Age-related cognitive decline is associated with increased risk of disability, dementia, and death. Recent studies suggest improvement in cognitive speed, attention, and executive functioning with physical activity. However, whether such improvements are activity specific is unclear. Therefore, we aimed to study the effect of 1 year age-adapted Taekwondo training on several cognitive functions, including reaction/motor time, information processing speed, and working and executive memory, in 24 healthy volunteers over 40. Reaction and motor time decreased with 41.2 and 18.4 s (p = 0.004, p = 0.015), respectively. Digit symbol coding task improved with a mean of 3.7 digits (p = 0.017). Digit span, letter fluency, and trail making test task-completion-time all improved, but not statistically significant. The questionnaire reported "better" reaction time in 10 and "unchanged" in 9 of the 19 study compliers. In conclusion, our data suggest that age-adapted Taekwondo training improves various aspects of cognitive function in people over 40, which may, therefore, offer a cheap, safe, and enjoyable way to mitigate age-related cognitive decline.