An Empirical Comparison of Commonly Used Universal Rating Scales for Dystonia.

Background: There are several widely used clinical rating scales for documenting the severity and distribution of various types of dystonia. Objectives: The goal of this study was to evaluate the performance of the most commonly used scales in a large group of adults with the most common types of isolated dystonia. Methods: Global Dystonia Rating Scale (GDRS) and the Burke-Fahn-Marsden Dystonia Rating Scale (BFM) scores were obtained for 3067 participants. Most had focal or segmental dystonia, with smaller numbers of multifocal or generalized dystonia. These scales were also compared for 209 adults with cervical dystonia that had Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores and 210 adults with blepharospasm that had Blepharospasm Severity Scale (BSRS) scores. Results: There were strong correlations between the GDRS and BFM total scores (r = 0.79) and moderate correlations for their sub scores (r > 0.5). Scores for both scales showed positive skew, with an overabundance of low scores. BFM sub-scores were not normally distributed, due to artifacts caused by the provoking factor. Relevant sub-scores of the GDRS and BFM also showed moderate correlations with the TWSTRS (r > 0.5) for cervical dystonia and the BSRS (r > 0.5) for blepharospasm. Conclusions: The BFM is more widely used than the GDRS, but these results suggest the GDRS may be preferable for focal and segmental dystonias. The overabundance of very low scores for both scales highlights challenges associated with discriminating very mild dystonia from other abnormal movements or variants of normal behavior.

Examining the associations between physical activity, self-esteem, perceived stress, and internalizing symptoms among older adolescents.

INTRODUCTION: In older adolescence, stress has been found to be prevalent. It has been seen that higher physical activity (PA) relates to lower stress levels, which, in turn, relates to fewer anxiety and depressive symptoms (internalizing symptoms). However, how these associations function is not fully understood. PA is strongly associated with greater self-esteem in adolescents. As greater self-esteem is thought to aid better coping with stress and has been seen as beneficial for mental health in adolescents, PA may be associated with lower stress and better mental health through self-esteem and more adaptive stress appraisals. Therefore, the aim of the study was to examine the relationships between PA, self-esteem, stress, and mental health. METHODS: A cross-sectional design was employed, and path analysis was implemented. PA, self-esteem, stress appraisals, distress tolerance, perceived stress, anxiety, and depression were assessed using online questionnaires from 244 adolescent participants from the United Kingdom (aged 15-19, M = 16.75 [SD = 0.82], 145 female). RESULTS: Path analysis revealed that PA was associated with lower perceived stress through increased self-esteem, adaptive appraisals, and higher distress tolerance (total standardized indirect effect; p = .007 (-0.25 to -0.11). Moreover, lower perceived stress was associated with lower anxiety (standardized direct effect; p < .001 [2.65-4.0] and depressive symptoms (standardized direct effect; p < .001 [0.33-0.63]). CONCLUSIONS: Findings suggest that higher PA could be effective in improving mental health among older adolescents, due to its association with perceived stress through higher self-esteem and more adaptive appraisals of stress.

The Dystonia Coalition: A Multicenter Network for Clinical and Translational Studies.

Dystonia is a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal postures, repetitive movements, or both. Research in dystonia has been challenged by several factors. First, dystonia is uncommon. Dystonia is not a single disorder but a family of heterogenous disorders with varied clinical manifestations and different causes. The different subtypes may be seen by providers in different clinical specialties including neurology, ophthalmology, otolaryngology, and others. These issues have made it difficult for any single center to recruit large numbers of subjects with specific types of dystonia for research studies in a timely manner. The Dystonia Coalition is a consortium of investigators that was established to address these challenges. Since 2009, the Dystonia Coalition has encouraged collaboration by engaging 56 sites across North America, Europe, Asia, and Australia. Its emphasis on collaboration has facilitated establishment of international consensus for the definition and classification of all dystonias, diagnostic criteria for specific subtypes of dystonia, standardized evaluation strategies, development of clinimetrically sound measurement tools, and large multicenter studies that document the phenotypic heterogeneity and evolution of specific types of dystonia.

Physical Activity Protects Against the Negative Impact of Coronavirus Fear on Adolescent Mental Health and Well-Being During the COVID-19 Pandemic.

Background: The severity of the Coronavirus pandemic has led to lockdowns in different countries to reduce the spread of the infection. These lockdown restrictions are likely to be detrimental to mental health and well-being in adolescents. Physical activity can be beneficial for mental health and well-being; however, research has yet to examine associations between adolescent physical activity and mental health and well-being during lockdown. Purpose: Examine the effects of adolescent perceived Coronavirus prevalence and fear on mental health and well-being and investigate the extent to which physical activity can be a protective factor against these concerns. Methods: During United Kingdom lockdown restrictions, 165 participants (100 female, aged 13-19) completed an online questionnaire assessing perceived Coronavirus prevalence and fear, physical activity, and indicators of mental health and well-being (stress, anxiety, depression, fatigue, vitality, and perceived health). Separate hierarchical multiple linear regression analyses (with age, gender, perceived Coronavirus prevalence, and fear entered in step 1, and physical activity in step 2) were run to predict each well-being outcome. Results: Regression analyses indicated that in general, while Coronavirus fear was a negative predictor, physical activity was a positive and stronger predictor of enhanced mental health and well-being outcomes. Conclusion: Findings suggest that physical activity during the Coronavirus pandemic can counteract the negative effects of Coronavirus fear on adolescent mental health and well-being. Therefore, physical activity should be promoted during lockdown to support good mental health and well-being.

Clinical and Demographic Characteristics of Upper Limb Dystonia.

BACKGROUND: Knowledge of characteristics in upper limb dystonia remains limited, derived primarily from small, single-site studies. OBJECTIVE: The objective of this study was to characterize demographic and clinical characteristics of upper limb dystonia from the Dystonia Coalition data set, a large, international, multicenter resource. METHODS: We evaluated clinical and demographic characteristics of 367 participants with upper limb dystonia from onset, comparing across subcategories of focal (with and without dystonia spread) versus nonfocal onset. RESULTS: Focal onset occurred in 80%; 67% remained focal without spread. Task specificity was most frequent in this subgroup, most often writer's cramp and affecting the dominant limb (83%). Focal onset with spread was more frequent in young onset (<21 years). Focal onset occurred equally in women and men; nonfocal onset affected women disproportionately. CONCLUSIONS: Upper limb dystonia distribution, focality, and task specificity relate to onset age and likelihood of regional spread. Observations may inform clinical counseling and design, execution, and interpretation of future studies. © 2020 International Parkinson and Movement Disorder Society.

Spectral Shearing of Quantum Light Pulses by Electro-Optic Phase Modulation.

Frequency conversion of nonclassical light enables robust encoding of quantum information based upon spectral multiplexing that is particularly well-suited to integrated-optics platforms. Here we present an intrinsically deterministic linear-optics approach to spectral shearing of quantum light pulses and show it preserves the wave-packet coherence and quantum nature of light. The technique is based upon an electro-optic Doppler shift to implement frequency shear of heralded single-photon wave packets by ±200 GHz, which can be scaled to an arbitrary shift. These results demonstrate a reconfigurable method to controlling the spectral-temporal mode structure of quantum light that could achieve unitary operation.

Clinical and demographic characteristics related to onset site and spread of cervical dystonia.

BACKGROUND: Clinical characteristics of isolated idiopathic cervical dystonia such as onset site and spread to and from additional body regions have been addressed in single-site studies with limited data and incomplete or variable dissociation of focal and segmental subtypes. The objectives of this study were to characterize the clinical characteristics and demographics of isolated idiopathic cervical dystonia in the largest standardized multicenter cohort. METHODS: The Dystonia Coalition, through a consortium of 37 recruiting sites in North America, Europe, and Australia, recruited 1477 participants with focal (60.7%) or segmental (39.3%) cervical dystonia on examination. Clinical and demographic characteristics were evaluated in terms of the body region of dystonia onset and spread. RESULTS: Site of dystonia onset was: (1) focal neck only (78.5%), (2) focal onset elsewhere with later segmental spread to neck (13.3%), and (3) segmental onset with initial neck involvement (8.2%). Frequency of spread from focal cervical to segmental dystonia (22.8%) was consistent with prior reports, but frequency of segmental onset with initial neck involvement was substantially higher than the 3% previously reported. Cervical dystonia with focal neck onset, more than other subtypes, was associated with spread and tremor of any type. Sensory tricks were less frequent in cervical dystonia with segmental components, and segmental cervical onset occurred at an older age. CONCLUSIONS: Subgroups had modest but significant differences in the clinical characteristics that may represent different clinical entities or pathophysiologic subtypes. These findings are critical for design and implementation of studies to describe, treat, or modify disease progression in idiopathic isolated cervical dystonia. © 2016 International Parkinson and Movement Disorder Society.

Clinical and genetic features of cervical dystonia in a large multicenter cohort.

OBJECTIVE: To characterize the clinical and genetic features of cervical dystonia (CD). METHODS: Participants enrolled in the Dystonia Coalition biorepository (NCT01373424) with initial manifestation as CD were included in this study (n = 1,000). Data intake included demographics, family history, and the Global Dystonia Rating Scale. Participants were screened for sequence variants (SVs) in GNAL, THAP1, and Exon 5 of TOR1A. RESULTS: The majority of participants were Caucasian (95%) and female (75%). The mean age at onset and disease duration were 45.5 ± 13.6 and 14.6 ± 11.8 years, respectively. At the time of assessment, 68.5% had involvement limited to the neck, shoulder(s), and proximal arm(s), whereas 47.4% had dystonia limited to the neck. The remaining 31.5% of the individuals exhibited more extensive anatomical spread. A head tremor was noted in 62% of the patients. Head tremor and laryngeal dystonia were more common in females. Psychiatric comorbidities, mainly depression and anxiety, were reported by 32% of the participants and were more common in females. Family histories of dystonia, parkinsonian disorder, and tremor were present in 14%, 11%, and 29% of the patients, respectively. Pathogenic or likely pathogenic SVs in THAP1, TOR1A, and GNAL were identified in 8 participants (0.8%). Two individuals harbored novel missense SVs in Exon 5 of TOR1A. Synonymous and noncoding SVs in THAP1 and GNAL were identified in 4% of the cohort. CONCLUSIONS: Head tremor, laryngeal dystonia, and psychiatric comorbidities are more common in female participants with CD. Coding and noncoding variants in GNAL, THAP1, and TOR1A make small contributions to the pathogenesis of CD.

Development of the Comprehensive Cervical Dystonia Rating Scale: Methodology.

We present the methodology utilized for development and clinimetric testing of the Comprehensive Cervical Dystonia (CD) Rating scale, or CCDRS. The CCDRS includes a revision of the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS-2), a newly developed psychiatric screening tool (TWSTRS-PSYCH), and the previously validated Cervical Dystonia Impact Profile (CDIP-58). For the revision of the TWSTRS, the original TWSTRS was examined by a committee of dystonia experts at a dystonia rating scales workshop organized by the Dystonia Medical Research Foundation. During this workshop, deficiencies in the standard TWSTRS were identified and recommendations for revision of the severity and pain subscales were incorporated into the TWSTRS-2. Given that no scale currently evaluates the psychiatric features of cervical dystonia (CD), we used a modified Delphi methodology and a reiterative process of item selection to develop the TWSTRS-PSYCH. We also included the CDIP-58 to capture the impact of CD on quality of life. The three scales (TWSTRS2, TWSTRS-PSYCH, and CDIP-58) were combined to construct the CCDRS. Clinimetric testing of reliability and validity of the CCDRS are described. The CCDRS was designed to be used in a modular fashion that can measure the full spectrum of CD. This scale will provide rigorous assessment for studies of natural history as well as novel symptom-based or disease-modifying therapies.

Experimental generation of multi-photon Fock states.

We experimentally demonstrate the generation of multi-photon Fock states with up to three photons in well-defined spatial-temporal modes synchronized with a classical clock. The states are characterized using quantum optical homodyne tomography to ensure mode selectivity. The three-photon Fock states are probabilistically generated by pulsed spontaneous parametric down conversion at a rate of one per second, enabling complete characterization in 12 hours.