The aim of the study was to evaluate and compare health-related quality of life (HR-QoL) and depression in essential blepharospasm (BSP) and idiopathic cervical dystonia (CD), to identify the clinical and demographic factors associated with poor HR-QoL in both disorders and to analyse the effect of Botulinum Toxin A (BtxA) therapy. Two hundred-twenty consecutive patients with BSP (N = 89, 62 % women, mean age 64 years, mean disease duration 7 years) and CD (N = 131, 64 % women, mean age 53 years, mean disease duration 8 years) recruited from routine referrals to eight Austrian dystonia clinics were included. HR-QoL was measured by the Short Form 36 (SF-36) and depression by the Beck Depression Inventory (BDI). At baseline, patients with CD and BSP scored significantly worse in all eight SF-36 domains compared with an age-matched community sample. In addition, 47 % of patients with CD and 37 % of those with BSP were depressed. Women with BSP scored significantly lower in all SF-36 domains and were more depressed than male patients. In contrast, there was no significant effect of gender on HR-QoL and depression in CD. Neck pain had a significant impact on all SF-36 domains and represented the main determinant of depression in CD. Although BtxA therapy resulted in a significant improvement of clinical symptoms in BSP and CD, HR-QoL did not improve in BSP and only two of the eight SF-36 domains improved significantly in patients with CD. The present study for the first time demonstrated that BSP has a substantial impact on health status emphasizing the need for psychological support with interventions aimed at treating depression in these patients. Our results provide further evidence for the profound impact of CD on HR-QoL and indicate the importance of an adequate management of neck pain in addition to reducing the severity of dystonia in CD. The mismatch between objective BtxA derived improvement of dystonia and lack of change of HR-QoL as determined by the SF-36 illustrates the need for optimized disease specific quality of life rating scales in patients with craniocervical dystonia.

Objective: To develop and test a questionnaire for measuring quality of life in patients with craniocervical dystonia. Methods: A 29-item pool was developed based on semi-structured interviews of patients with cervical dystonia (CD) and blepharospasm (BSP). This preliminary questionnaire was administered to 203 consecutive patients with CD and BSP from Austrian dystonia and botulinum toxin outpatient clinics. For scale generation, a combination of exploratory factor and cluster analysis was applied. This resulted in the 24-item version of the instrument (CDQ-24) based on five subscales: Stigma, Emotional wellbeing, Pain, Activities of daily living, and Social/family life. The validity and reliability of the CDQ-24 was assessed in 231 consecutive patients with CD and BSP different from those examined with the preliminary questionnaire. This second survey included the CDQ-24, a generic QoL instrument (SF-36) and clinical rating scales. Sensitivity to change was analysed in 51 previously untreated (de novo) patients four weeks and one year following the first botulinum toxin treatment. Results: Internal consistency reliability was satisfactory for all subscales, with values of Cronbach's a ranging from 0.77 to 0.89. The CDQ-24 subscales showed moderate to high correlations with those SF-36 subscales measuring similar aspects (Pearson's correlation r = 0.50-0.73; p,0.001, each). Sensitivity to change was confirmed by highly significant improvements of all CDQ-24 subscores in the de novo patients from baseline to four week follow up. One year follow up data revealed a stable improvement. Conclusion: The CDQ-24 is the first fully validated and disease specific questionnaire to evaluate quality of life of patients with cervical dystonia and blepharospasm and we propose its use in clinical trials as well as in daily clinical practice. INTRODUCTIONPrimary dystonia is one of the most prevalent movement disorders with a minimum prevalence of 98 per 100 000 according to a recent population based survey.1 Cervical dystonia (CD) and blepharospasm (BSP) are the most common forms and account for about 75% of cases with primary focal dystonia.2 Patients with craniocervical dystonia face a lifetime of chronic visible disability 3 and previous studies have demonstrated impaired health related quality of life (HR-QoL) in patients with CD 4-9 and BSP.8 9 The generic HR-QoL measures used in these studies consistently demonstrated lower physical and mental QoL scores in craniocervical dystonia compared with healthy controls. While generic QoL instruments compare outcomes across different populations they do not measure specific problems of diagnostic groups.10 A recently published study revealed that QoL in patients with CD is mainly predicted by factors such as self esteem, self deprecation, social participation, social support, stigma, anxiety, and depression in addition to disease severity and response to botulinum toxin 11 -all of which are not addressed by a generic QoL instrument.The purpose of this study was to develop a diseas...

Responsive caregiving is the dimension of parenting most consistently related to later child functioning in both developing and developed countries. There is a growing need for efficient, psychometrically sound and culturally appropriate measurement of this construct. This study describes the cross-cultural validation in Brazil of the Responsive Interactions for Learning (RIFL-P) measure, requiring only eight minutes for assessment and coding. The cross-cultural adaptation used a recognized seven-step procedure. The adapted version was applied to a stratified sample of 153 Brazilian mother–child (18 months) dyads. Videos of mother–child interaction were coded using the RIFL-P and a longer gold standard parenting assessment. Mothers completed a survey on child stimulation (18 months) and child outcomes were measured at 24 months. Internal consistency (α = 0.94), inter-rater reliability (r = 0.83), and intra-rater reliability (r = 0.94) were all satisfactory to high. RIFL-P scores were significantly correlated with another measurement of parenting (r’s ranged from 0.32 to 0.47, p < 0.001), stimulation markers (r = 0.34, p < 0.01), and children’s cognition (r = 0.29, p < 0.001), language (r = 0.28, p < 0.001), and positive behavior (r = 0.17, p < 0.05). The Brazilian Portuguese version is a valid and reliable instrument for a brief assessment of responsive caregiving.

Background Home-visiting programs are a common and effective public health approach to promoting parent and child well-being, including in low- and middle-income countries. The World Health Organization and UNICEF have identified responsive caregiving as one key component of the nurturing care children need to survive and thrive. Nonetheless, the importance of responsive caregiving and how to coach it is often overlooked in trainings for staff in home-visiting programs. Methods To determine whether it is possible to enhance home-visitors’ understanding of responsive caregiving and how to coach it, we conducted a cluster randomized controlled trial with 181 staff working in Brazil’s national home-visiting program. We used a computerized random number generator to randomly assign half of participants to take an online professional development course about responsive caregiving immediately and the other half to a waitlist. Individuals assessing outcome data were blind to group assignment. Results Compared to those in the control group (N = 90, both randomized and analyzed), participants assigned to take the course (N = 91, both randomized and analyzed) were more knowledgeable about responsivity (Cohen’s d = 0.64, 95% Confidence Interval (CI) = 0.34, 0.94) and its importance for children’s socioemotional (odds ratio (OR) = 1.88, 95% CI = 1.00, 3.50) and cognitive (OR = 2.57, 95% CI = 1.15, 5.71) development, better able to identify responsive parental behaviors in videotaped interactions ( d = 1.86, 95% CI = 1.51, 2.21), and suggested more effective strategies for coaching parents on responsivity ( d = 0.51, 95% CI = 0.21, 0.80) and tracking goal implementation (OR = 3.20, 95% CI = 1.28, 7.99). There were no significant changes in participants’ tendency to encourage goal setting and reflection, or their perspective-taking skills. Participants were very satisfied with the course content and mode of delivery and there was no drop-out from the program. Conclusions A short, online professional development program created moderate to large improvements in home-visitors’ knowledge and intended coaching practices. This suggests that such programs are feasible, even in low-income and rural areas, and provide a low-cost, scalable option for possibly maximizing the impact of home-visiting programs – particularly with regard to parental responsivity, and in turn, child outcomes.

Efforts to monitor and improve responsive caregiving for young children, because of its importance for child development, are part of the United Nations Sustainable Development Goals. Two brief observational measures of responsive caregiving have been developed and validated (Responsive Interactions for Learning—parent [RIFL-P] and educator [RIFL-Ed] versions), with the RIFL-P available in English, Portuguese, and Spanish. The aim of the current study was to present and evaluate two online training programs for the RIFL measures. These distance learning courses were designed as open-source and asynchronous to enable their use in low- and middle-income countries and remote areas. The following course components are used: readings, lectures, observation of interactions on video, coding practice with automated feedback on item coding, and quizzes. Of the 76 trainees who registered for one of the online courses, 58 (76%) completed all theoretical module components. Student performance was generally high. Marks on quizzes ranged between 83%–100%. Ninety percent of those who took the reliability tests passed (40/44). Student satisfaction during and after the course was high. The effective online training programs are available free of charge and the RIFL suite of measures is efficient to implement. Implications for research and practice are discussed.