Eco-anxiety: An adaptive behavior or a mental disorder? Results of a psychometric study.

OBJECTIVE: Eco-anxiety is a complex construct that has been created to grasp the psychological impact of the consequences of global warming. The concept needs a reliably valid questionnaire to better evaluate its impact on the risk of anxiety and depressive disorders. The Eco-Anxiety Questionnaire (EAQ-22) evaluates two dimensions: 'habitual ecological anxiety' and 'distress related to eco-anxiety'. However, a version in French, one of the world's widely spoken languages, was until now lacking. We aimed to translate and validate the French EAQ-22 and to evaluate the prevalence of the level of the two dimensions of eco-anxiety and the relationship with anxiety and depressive symptoms in a representative adult sample of the French general population. METHODS: This study was performed under the auspices of the Institut national du sommeil et de la vigilance (INSV). Participants (18-65 years) were recruited by an institute specialized in conducting online surveys of representative population samples (quota sampling). Two native French speakers and two native English speakers performed a forward-backward translation of the questionnaire. The Hospital Anxiety and Depression scale (HAD) was administered to assess anxiety (HAD-A) and depressive (HAD-D) symptoms and for external validity. Internal structural validity and external validity were analysed. RESULTS: Evaluation was performed on 1004 participants: mean age 43.47 years (SD=13.41, range: [19-66]); 54.1% (n=543) women. Using the HAD, 312 (31.1%) patients had current clinically significant anxiety symptoms (HAD-A>10) and 150 (14.9%) had current clinically significant depressive symptoms (HAD-D>10). Cronbach's alpha coefficient was 0.934, indicating very good internal consistency. Correlation between EAQ-22 and HAD scores was low (r[1004]=0.209, P<0.001), 'habitual ecological anxiety' was correlated less with HAD-A and HAD-D than 'distress related to eco-anxiety', indicating good external validity. CONCLUSION: This study validates the French EAQ-22 and paves the way for using the EAQ-22 as a global tool for assessing eco-anxiety. Further prospective studies are now required to better evaluate the impact of eco-anxiety on the occurrence of anxiety and depressive disorder.

Low level myelomeningoceles: do they need prenatal surgery?

BACKGROUND: Postnatal closure of a myelomeningocele remains the standard of care in many countries. The prenatal closure has given hope for decreasing the damage to the neural placode and has challenged classic management. However, this technique presents potential sources of complications. Patients with MMC with an anatomical level of L4 and below have a better functional prognosis than higher level malformations. Are they still candidates for prenatal surgery? OBJECTIVE: To evaluate outcome of MMC with an anatomical level of L4 and below and discuss, with support of the literature, the indications to perform prenatal closure in this particular group of patients. MATERIALS AND METHODS: Twenty-nine children were included in this observational study. The level of the vertebral malformation was sacral in 12 cases (41.4%) or lumbar (level ≤ L4) in 17 cases (58.6%). All the patients was operated postnatally for closure of the MMC with microsurgical technique as soon as possible after clinical evaluation (range 0-97 days). RESULTS: Only 11 out of 29 patients (37.9%) needed of a CSF diversion. A Chiari II malformation was present before MMC closure in 17 patients (58.6%) and only in 5 (17%) after. Twenty-six patients (89.7%) were able to walk. Seven (23%) and 16 (55%) of our patients have a normal bladder and bowel control, respectively. All school-aged children attend school. CONCLUSIONS: The functional outcome for low-level MMC is good when managed with modern microneurosurgical techniques with a low risk for the patient and the mother. Therefore, we do not suggest prenatal surgery for subgroup of infant with MM.

Corticosteroids in Duchenne muscular dystrophy: impact on the motor function measure sensitivity to change and implications for clinical trials.

AIM: To monitor the evolution of the motor function of ambulatory patients with Duchenne muscular dystrophy (DMD) treated by corticosteroids for 2 years in comparison with untreated patients. METHOD: This observational, multicentre cohort study explores the evolution of the motor function measure (MFM) over a 24-month period for 29 ambulant corticosteroids-treated and 45 ambulant untreated patients with DMD. RESULTS: Significant differences were found between mean MFM scores in corticosteroids-treated and untreated groups for domain 1 of the MFM (standing position and transfers; D1), domain 2 of the MFM (axial and proximal motor function; D2), and domain 3 of the MFM (distal motor function; D3). Subscores were between 0 months and 6 months, and 0 months and 24 months. For the D1 subscore specifically, there was a significant increase in the corticosteroids-treated group (mean±standard deviation [SD] slope of change=12.6±15.5%/y), while a decrease was observed in the untreated group (-17.8±17.7%/y) between 0 months and 6 months (p<0.001). Sensitivity to change as assessed by standardized response means was high between 12 months and 24 months for D1 of both corticosteroids-treated and untreated groups (1.0 and 1.2 respectively), and low for D2 and D3 of both treated and untreated groups. INTERPRETATION: Patients with DMD treated by corticosteroids present a different course of the disease as assessed by MFM, confirming the sensitivity to change of the MFM in this population. WHAT THIS PAPER ADDS: Corticosteroids have a quantitative impact on muscle strength 6 to 24 months after starting treatment. Motor function measure is a valid outcome measure in Duchenne muscular dystrophy patients under corticosteroid treatment.

Rasch analysis of the motor function measure in patients with congenital muscle dystrophy and congenital myopathy.

OBJECTIVES: To monitor treatment effects in patients with congenital myopathies and congenital muscular dystrophies, valid outcome measures are necessary. The Motor Function Measure (MFM) was examined for robustness, and changes are proposed for better adequacy. DESIGN: Observational study based on data previously collected from several cohorts. SETTING: Nineteen departments of physical medicine or neuromuscular consultation in France, Belgium, and the United States. PARTICIPANTS: Patients (N=289) aged 5 to 77 years. INTERVENTIONS: None. MAIN OUTCOME MEASURES: A Rasch analysis examined the robustness of the MFM across the disease spectrum. The 3 domains of the scale (standing position and transfers, axial and proximal motor function, and distal motor function) were independently examined with a partial credit model. RESULTS: The original 32-item MFM did not sufficiently fit the Rasch model expectations in either of its domains. Switching from a 4- to a 3-category response scale in 18 items restored response order in 16. Various additional checks suggested the removal of 7 items. The resulting Rasch-scaled Motor Function Measure with 25 items for congenital disorders of the muscle (Rs-MFM25(CDM)) demonstrated a good fit to the Rasch model. Domain 1 was well targeted to the whole severity spectrum-close mean locations for items and persons (0 vs 0.316)-whereas domains 2 and 3 were better targeted to severe cases. The reliability coefficients of the Rs-MFM25(CDM) suggested sufficient ability for each summed score to distinguish between patient groups (0.9, 0.8, and 0.7 for domains 1, 2, and 3, respectively). A sufficient agreement was found between results of the Rasch analysis and physical therapists' opinions. CONCLUSIONS: The Rs-MFM25(CDM) can be considered a clinically relevant linear scale in each of its 3 domains and may be soon reliably used for assessment in congenital disorders of the muscle.

English cross-cultural translation and validation of the neuromuscular score: a system for motor function classification in patients with neuromuscular diseases.

OBJECTIVE: To develop and validate an English version of the Neuromuscular (NM)-Score, a classification for patients with NM diseases in each of the 3 motor function domains: D1, standing and transfers; D2, axial and proximal motor function; and D3, distal motor function. DESIGN: Validation survey. SETTING: Patients seen at a medical research center between June and September 2013. PARTICIPANTS: Consecutive patients (N=42) aged 5 to 19 years with a confirmed or suspected diagnosis of congenital muscular dystrophy. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: An English version of the NM-Score was developed by a 9-person expert panel that assessed its content validity and semantic equivalence. Its concurrent validity was tested against criterion standards (Brooke Scale, Motor Function Measure [MFM], activity limitations for patients with upper and/or lower limb impairments [ACTIVLIM], Jebsen Test, and myometry measurements). Informant agreement between patient/caregiver (P/C)-reported and medical doctor (MD)-reported NM scores was measured by weighted kappa. RESULTS: Significant correlation coefficients were found between NM scores and criterion standards. The highest correlations were found between NM-score D1 and MFM score D1 (ρ=-.944, P<.0001), ACTIVLIM (ρ=-.895, P<.0001), and hip abduction strength by myometry (ρ=-.811, P<.0001). Informant agreement between P/C-reported and MD-reported NM scores was high for D1 (κ=.801; 95% confidence interval [CI], .701-.914) but moderate for D2 (κ=.592; 95% CI, .412-.773) and D3 (κ=.485; 95% CI, .290-.680). Correlation coefficients between the NM scores and the criterion standards did not significantly differ between P/C-reported and MD-reported NM scores. CONCLUSIONS: Patients and physicians completed the English NM-Score easily and accurately. The English version is a reliable and valid instrument that can be used in clinical practice and research to describe the functional abilities of patients with NM diseases.

Motor function measure: validation of a short form for young children with neuromuscular diseases.

OBJECTIVE: To validate a useful version of the Motor Function Measure (MFM) in children with neuromuscular diseases aged <7 years old. DESIGN: Two prospective cohort studies that documented the MFM completion of children aged between 2 and 7 years old. SETTING: French-speaking rehabilitation departments from France, Belgium, and Switzerland. PARTICIPANTS: Healthy children (n=194) and children with a neuromuscular disease (n=88). INTERVENTIONS: Patients were rated by the MFM either once or twice by trained medical professionals, with a delay between the 2 MFMs ranging between 8 and 30 days. MAIN OUTCOME MEASURE: Intra- and interrater reliability of the MFM. RESULTS: The subtests making up the MFM-32, a scale monitoring severity and progression of motor function in patients with a neuromuscular disease in 3 functional domains, were carried out in healthy children aged 2 to 7 years. Twenty items of the MFM-32 were successfully completed by these children and were used to constitute the MFM-20. Principal component analysis of the MFM-20 confirmed the 3 functional domains. Inter- and intrarater reliability of the 3 subscores and total score were high (intraclass correlation coefficient >.90), and discriminant validity was good. CONCLUSIONS: The MFM-20 can be used as an outcome measure for assessment of motor function in young children with neuromuscular disease.

Better characterizing sleep beliefs for personalized sleep health promotion: the French sleep beliefs scale validation study.

Background: The Sleep Beliefs Scale (SBS) is a well-known tool to design and monitor personalized sleep health promotion at an individual and population level. The lack of an established French version limits the development of effective interventions targeting these populations. Thus, the aim of this study was to validate the French version of the SBS in a representative sample of the general population. Methods: Quota sampling was used to recruit 1,004 participants (18-65 years, mean age: 43 years, 54% of female) who underwent an online survey to complete the SBS, and to assess sleep schedules, sleep quality and disorders, and mental health. Cronbach's α coefficient, confirmatory factor analysis, item-internal consistency (IIC), and item discriminant validity (IDV) of the SBS were computed to assess internal validity while bivariate associations with sleep schedules, sleep quality and disorders, and mental health were used to assess external convergent and discriminant validity. Results: The mean score on the SBS was 12.3 ± 4.9. Item 19 ("Quiet & Dark") showed the highest rate of correct answers (n = 801, 79.8%), while item 20 ("Recovering sleep") showed the lowest rate of correct answers (n = 246, 24.5%). Overall, the SBS showed satisfactory internal consistency (α = 0.87) and confirmed the three-factor structure proposed by the original study. All items were found consistent (IIC > 0.4) and discriminant (IIC > IDV) except for item 20 ("recovering lost sleep by sleeping for a long time"). Females, older participants, and subjects with short time-in-bed, poor sleep quality, insomnia, and circadian rhythm disorder had higher SBS scores while participants with depressive symptoms had lower SBS scores. Conclusion: We successfully translated and validated the French version of the SBS in a representative sample, making it a reliable instrument for researchers and clinicians to assess and target sleep beliefs. Correct answers vary from 25 to 80% which underlines the importance of continuing sleep health promotion campaigns by targeting poorly understood behaviors. Our findings also shed light on the fickleness of beliefs that are prone to vary within individuals across time, in step with societal changes. Several associated factors were identified, thus contributing to our understanding of sleep beliefs and offering insights for personalized approaches to enhance sleep health and overall well-being.

Description of a novel patient with the TRPM3 recurrent p.Val837Met variant.

De novo heterozygous missense mutations in TRPM3 have been shown to cause developmental and epileptic encephalopathies (DEE). It is a very rare condition, as only 9 patients have been described to date. We report here a novel patient carrying the recurrent p.Val837Met variant and presenting new clinical features, such as trigonocephaly, expanding the phenotypical spectrum of the disease.

International Expert Opinions and Recommendations on the Use of Melatonin in the Treatment of Insomnia and Circadian Sleep Disturbances in Adult Neuropsychiatric Disorders.

Introduction: Insomnia and circadian rhythm disorders, such as the delayed sleep phase syndrome, are frequent in psychiatric disorders and their evaluation and management in early stages should be a priority. The aim of this paper was to express recommendations on the use of exogenous melatonin, which exhibits both chronobiotic and sleep-promoting actions, for the treatment of these sleep disturbances in psychiatric disorders. Methods: To this aim, we conducted a systematic review according to PRISMA on the use of melatonin for the treatment of insomnia and circadian sleep disorders in neuropsychiatry. We expressed recommendations for the use of melatonin in psychiatric clinical practice for each disorder using the RAND/UCLA appropriateness method. Results: We selected 41 studies, which included mood disorders, schizophrenia, substance use disorders, attention deficit hyperactivity disorders, autism spectrum disorders, neurocognitive disorders, and delirium; no studies were found for both anxiety and eating disorders. Conclusion: The administration of prolonged release melatonin at 2-10 mg, 1-2 h before bedtime, might be used in the treatment of insomnia symptoms or comorbid insomnia in mood disorders, schizophrenia, in adults with autism spectrum disorders, neurocognitive disorders and during sedative-hypnotics discontinuation. Immediate release melatonin at <1 mg might be useful in the treatment of circadian sleep disturbances of neuropsychiatric disorders.

Patterns of hip migration in non-ambulant children with cerebral palsy: A prospective cohort study.

BACKGROUND: In children with cerebral palsy (CP), we have little information on when hip migration (HM) starts, what causes hip displacement, how HM changes over time, and how to halt this migration to avoid surgery. OBJECTIVES: We aimed to estimate the prevalence of HM percentage (HMP)>40% in a homogeneous population of non-ambulant children with CP and model the changes in HMP over a 2.6-year mean follow-up. METHODS: From September 2009 to September 2015, this observational, prospective, multicenter cohort study recruited 235 children from 51 centers who were 3 to 10 years old and had levels IV and V of the Gross Motor Function Classification System for CP. The outcomes were yearly HMP measurements by the Reimers index. Only children with at least one hip with HMP≤40% at baseline were included in trajectory modeling. Comparisons of chidren's characteristics between trajectory groups were adjusted by the false discovery rate method. RESULTS: The prevalence of children with at least one hip with HMP>40% was estimated at 24.3% (95% confidence interval 18.6-30.0). Pelvic obliquity was observed in 51.4% and 24.4% of children with asymmetric and symmetric HMP (P=0.002). The trajectory modelling identified 3 types of MP changes over time. Many children (67.4% and 79.3% for the right and left hip) could be assigned to the "stable" trajectory group. CONCLUSIONS: In non-ambulant children with CP, the prevalence of HM requiring surgery is low and most hips remain practically stable over time.

[Sleep disorders and their treatment in post-traumatic stress disorder]. / Les troubles du sommeil et leur traitement dans le trouble stress post-traumatique.

Subjects suffering from post-traumatic stress disorder present sleeping disorders like a chronic insomnia, traumatic nightmares, but also less expected, sleep breathing disorders. Sleep problems are a factor of development and maintenance of PTSD, but also a factor of resistance to treatment. After a therapy focused on PTSD, they represent frequent residual symptoms. It is necessary to couple, with the usual management of PTSD, targeted approaches for sleep problems. These targeted approaches allow an improvement of the nocturnal properties but also diurnal specific symptoms of PTSD. Stakes around primary, secondary and tertiary prevention of PTSD emerge around these sleep disorders.

Comparison of desipramine and citalopram treatments for depression in Parkinson's disease: a double-blind, randomized, placebo-controlled study.

Depression is one of the most common psychiatric disturbances in Parkinson's disease (PD). Recent reviews have highlighted the lack of controlled trials and the ensuing difficulty in formulating recommendations for antidepressant use in PD. We sought to establish whether antidepressants provide real benefits and whether tricyclic and selective serotonin reuptake inhibitor (SSRI) antidepressants differ in their short-term efficacy, because the time to onset of therapeutic benefit remains an important criterion in depression. The short-term efficacy (after 14 and 30 days) of two antidepressants (desipramine, a predominantly noradrenergic reuptake inhibitor tricyclic and citalopram, a SSRI) was assessed in a double-blind, randomized, placebo- controlled study of 48 nondemented PD patients suffering from major depression. After 14 days, desipramine prompted an improvement in the Montgomery Asberg Depression Rating Scale (MADRS) score, compared with citalopram and placebo. Both antidepressants produced significant improvements in the MADRS score after 30 days. Mild adverse events were twice as frequent in the desipramine group as in the other groups. A predominantly noradrenergic tricyclic antidepressant induced a more intense short-term effect on parkinsonian depression than did an SSRI. However, desipramine's lower tolerability may outweigh its slight short-term clinical advantage.

Postnatal Management of Myelomeningocele: Outcome with a Multidisciplinary Team Experience.

INTRODUCTION: Myelomeningocele (MMC) is a complex neural tube defect. Few studies report the results of modern postnatal management. The goal of this study was to report the long-term outcome of a multidisciplinary approach of patients with MMC. METHODS: Forty-six MMCs were included. Clinical status was evaluated prospectively. RESULTS: Mean follow-up was 8.1 years. The level of the malformation was sacral or lower lumbar (≤L4) in 27 cases, higher lumbar (between L1 and L3) in 5 cases, and thoracic in 14 cases. A Chiari II malformation was present at birth in 78.3% of the cases. Seventy-six percent of the patients were operated on within the first 24 hours of life. Sixty-one percent needed a cerebrospinal fluid diversion procedure. Seven patients underwent a second surgery for a retethering of the spine. Eighty percent presented with orthopedic problems. Sixty-five percent of our patients were able to walk. Only 13% of patients had a normal urinary elimination. Two groups of patients were identified: one group with a malformation at the level of L4 or below (group 1) and another group with a malformation strictly above L4 (group 2). Group 1 had significantly better outcome. CONCLUSIONS: This series proves that modern multidisciplinary postnatal management of MMC is effective. In the light of these results and of the results of prenatal management of MMC, prenatal surgery seems to be a highly valuable tool to improve the outcome of patients with high lesions (level ≥L3).

Use and tolerability of a side pole static ankle foot orthosis in children with neurological disorders.

BACKGROUND: Transverse-plane foot deformities are a frequently encountered issue in children with neurological disorders. They are the source of many symptoms, such as pain and walking difficulties, making their prevention very important. OBJECTIVES: We aim to describe the use and tolerability of a side pole static ankle foot orthosis used to prevent transverse-plane foot deformities in children with neurologic disorders. STUDY DESIGN: Monocentric, retrospective, observational study. METHODS: Medical data were collected from 103 children with transverse-plane foot deformities in one or both feet caused by a neurological impairment. All children were braced between 2001 and 2010. RESULTS: Unilateral orthosis was prescribed for 32 children and bilateral orthosis for 71. Transverse-plane foot deformities were varus in 66% of the cases and an equinus was associated in 59.2% of the cases. Mean age for the first prescription was 8.6 years. For the 23 patients present at the 4-year visit, 84.8% still wore the orthosis daily, and 64.7% wore the orthosis more than 6 h per day. The rate of permanent discontinuation of wearing the orthosis was 14.7%. CONCLUSION: The side pole static ankle foot orthosis is well tolerated with very few side effects, which promotes regular wearing and observance. Clinical relevance Side pole static ankle foot orthoses are well tolerated and can be safely used for children with foot abnormalities in the frontal plane that have a neurological pathology origin.

Influence of a two-year steroid treatment on body composition as measured by dual X-ray absorptiometry in boys with Duchenne muscular dystrophy.

Steroids are nowadays routinely used as a long-term treatment in Duchenne muscular dystrophy (DMD). Their effects on body composition were assessed using dual X-ray absorptiometry. The study followed over 2 years 29 genetically confirmed DMD patients: 21 in the steroid-treated group and 8 in the steroid-naïve group. After 2 years of steroid treatment, the lean tissue mass values increased significantly (p<0.0001), the percentage of body fat mass remained practically constant (p=0.94) in comparison with the initial visit. In the steroid-naïve patients, there were no significant increases in the lean tissue mass but deterioration in body composition confirmed by a significant increase in the percentage of body fat mass. Besides, significant negative correlations were found between the percentage of body fat mass and the MFM total score (R=-0.79, n=76, p<0.0001). A 2-year steroid treatment improves significantly body composition of boys with DMD through a significant increase in lean tissue mass. We suggest that a thorough check of body composition should be carried out before steroid treatment discontinuation in case of overweight gain.