Interrater reliability of motor severity scales for hemifacial spasm.

To compare the inter-rater reliability (IRR) of five clinical rating scales for video-based assessment of hemifacial spasm (HFS) motor severity. We evaluated the video recordings of 45 HFS participants recruited through the Dystonia Coalition. In Round 1, six clinicians with expertise in HFS assessed the participants' motor severity with five scales used to measure motor severity of HFS: the Jankovic rating scale (JRS), Hemifacial Spasm Grading Scale (HSGS), Samsung Medical Center (SMC) grading system for severity of HFS spasms (Lee's scale), clinical grading of spasm intensity (Chen's scale), and a modified version of the Abnormal Involuntary Movement Scale (Tunc's scale). In Round 2, clinicians rated the same cohort with simplified scale wording after consensus training. For each round, we evaluated the IRR using the intraclass correlation coefficient [ICC (2,1) single-rater, absolute-agreement, 2-way random model]. The scales exhibited IRR that ranged from "poor" to "moderate"; the mean ICCs were 0.41, 0.43, 0.47, 0.43, and 0.65 for the JRS, HSGS, Lee's, Chen's, and Tunc's scales, respectively, for Round 1. In Round 2, the corresponding IRRs increased to 0.63, 0.60, 0.59, 0.53, and 0.71. In both rounds, Tunc's scale exhibited the highest IRR. For clinical assessments of HFS motor severity based on video observations, we recommend using Tunc's scale because of its comparative reliability and because clinicians interpret the scale easily without modifications or the need for consensus training.

Hold that pose: capturing cervical dystonia's head deviation severity from video.

OBJECTIVE: Deviated head posture is a defining characteristic of cervical dystonia (CD). Head posture severity is typically quantified with clinical rating scales such as the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Because clinical rating scales are inherently subjective, they are susceptible to variability that reduces their sensitivity as outcome measures. The variability could be circumvented with methods to measure CD head posture objectively. However, previously used objective methods require specialized equipment and have been limited to studies with a small number of cases. The objective of this study was to evaluate a novel software system-the Computational Motor Objective Rater (CMOR)-to quantify multi-axis directionality and severity of head posture in CD using only conventional video camera recordings. METHODS: CMOR is based on computer vision and machine learning technology that captures 3D head angle from video. We used CMOR to quantify the axial patterns and severity of predominant head posture in a retrospective, cross-sectional study of 185 patients with isolated CD recruited from 10 sites in the Dystonia Coalition. RESULTS: The predominant head posture involved more than one axis in 80.5% of patients and all three axes in 44.4%. CMOR's metrics for head posture severity correlated with severity ratings from movement disorders neurologists using both the TWSTRS-2 and an adapted version of the Global Dystonia Rating Scale (rho = 0.59-0.68, all p <0.001). CONCLUSIONS: CMOR's convergent validity with clinical rating scales and reliance upon only conventional video recordings supports its future potential for large scale multisite clinical trials.

Head tremor in cervical dystonia: Quantifying severity with computer vision.

BACKGROUND: Head tremor (HT) is a common feature of cervical dystonia (CD), usually quantified by subjective observation. Technological developments offer alternatives for measuring HT severity that are objective and amenable to automation. OBJECTIVES: Our objectives were to develop CMOR (Computational Motor Objective Rater; a computer vision-based software system) to quantify oscillatory and directional aspects of HT from video recordings during a clinical examination and to test its convergent validity with clinical rating scales. METHODS: For 93 participants with isolated CD and HT enrolled by the Dystonia Coalition, we analyzed video recordings from an examination segment in which participants were instructed to let their head drift to its most comfortable dystonic position. We evaluated peak power, frequency, and directional dominance, and used Spearman's correlation to measure the agreement between CMOR and clinical ratings. RESULTS: Power averaged 0.90 (SD 1.80) deg2/Hz, and peak frequency 1.95 (SD 0.94) Hz. The dominant HT axis was pitch (antero/retrocollis) for 50%, roll (laterocollis) for 6%, and yaw (torticollis) for 44% of participants. One-sided t-tests showed substantial contributions from the secondary (t = 18.17, p < 0.0001) and tertiary (t = 12.89, p < 0.0001) HT axes. CMOR's HT severity measure positively correlated with the HT item on the Toronto Western Spasmodic Torticollis Rating Scale-2 (Spearman's rho = 0.54, p < 0.001). CONCLUSIONS: We demonstrate a new objective method to measure HT severity that requires only conventional video recordings, quantifies the complexities of HT in CD, and exhibits convergent validity with clinical severity ratings.

From null to midline: changes in head posture do not predictably change head tremor in cervical dystonia.

Introduction: A common view is that head tremor (HT) in cervical dystonia (CD) decreases when the head assumes an unopposed dystonic posture and increases when the head is held at midline. However, this has not been examined with objective measures in a large, multicenter cohort. Methods: For 80 participants with CD and HT, we analyzed videos from examination segments in which participants were instructed to 1) let their head drift to its most comfortable position (null point) and then 2) hold their head straight at midline. We used our previously developed Computational Motor Objective Rater (CMOR) to quantify changes in severity, amplitude, and frequency between the two postures. Results: Although up to 9% of participants had exacerbated HT in midline, across the whole cohort, paired t-tests reveal no significant changes in overall severity (t = -0.23, p = 0.81), amplitude (t = -0.80, p = 0.43), and frequency (t = 1.48, p = 0.14) between the two postures. Conclusions: When instructed to first let their head drift to its null point and then to hold their head straight at midline, most patient's changes in HT were below the thresholds one would expect from the sensitivity of clinical rating scales. Counter to common clinical impression, CMOR objectively showed that HT does not consistently increase at midline posture in comparison to the null posture.

Does Raising the Arms Modify Head Tremor Severity in Cervical Dystonia?

Background: A defining characteristic of dystonia is its position-dependence. In cervical dystonia (CD), sensory tricks ameliorate head tremor (HT). But it remains unknown whether raising the arms alone has the same impact. Methods: We analyzed data collected from patients enrolled by the Dystonia Coalition. For 120 patients with HT, we assessed how raising their arms without touching their head changed their HT severity. Results: Forty-eight out of 120 patients exhibited changes in HT severity when raising their arms. These patients were more likely to exhibit decreases in HT severity (N = 35) than increases (N = 13, χ2 (1, N = 48) = 10.1, p = 0.002). Demographic factors and sensory trick efficacy were not significant predictors of whether HT severity changed when raising their arms. Discussion: Raising the arms without touching the head is a posture that can reduce HT severity in some CD patients. Our results extend the concept of position-dependent motor symptoms in CD to include the position of the arms. Highlights: Head tremor (HT) is a prevalent symptom of cervical dystonia (CD) that can often be disabling. This study demonstrates that raising the arms without touching the head is a posture that can reduce HT severity in some CD patients. Our findings also identify a novel form of position-dependence in CD.

Head tremor and pain in cervical dystonia.

BACKGROUND: Although head tremor (HT) and pain are prevalent in cervical dystonia (CD), their joint relationship to phenotypic features of focal dystonia remains unclear. OBJECTIVES: We examined how severity of HT and pain are associated with age of CD onset and duration, and whether HT subtypes ("jerky" or "regular") exhibit distinct relationships between severity of HT and pain. METHODS: The severity of HT and pain were assessed with the Toronto Western Spasmodic Torticollis Rating Scale in retrospective review of 188 CD patients recruited through the Dystonia Coalition. RESULTS: HT severity was associated with longer CD duration (p < 0.0005), whereas pain severity was associated with younger age at onset (p = 0.043). HT severity and pain severity were not correlated for jerky HT (p = 0.996), but positively correlated for regular HT (p = 0.01). CONCLUSIONS: The distinct associations of HT and pain with age at onset, disease duration, and HT subtype further characterize the heterogeneity of CD's clinical presentation and suggest similarly heterogeneous underlying mechanisms.

It's tricky: Rating alleviating maneuvers in cervical dystonia.

OBJECTIVES: To investigate hypothesized sources of error when quantifying the effect of the sensory trick in cervical dystonia (CD) with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS-2), test strategies to mitigate them, and provide guidance for future research on the sensory trick. METHODS: Previous analyses suggested the sensory trick (or "alleviating maneuver", AM) item be removed from the TWSTRS-2 because of its poor clinimetric properties. We hypothesized three sources of clinimetric weakness for rating the AM: 1) whether patients were given sufficient time to demonstrate their AM; 2) whether patients' CD was sufficiently severe for detecting AM efficacy; and 3) whether raters were inadvertently rating the item in reverse of scale instructions. We tested these hypotheses with video recordings and TWSTRS-2 ratings by one "site rater" and a panel of five "video raters" for each of 185 Dystonia Coalition patients with isolated CD. RESULTS: Of 185 patients, 23 (12%) were not permitted sufficient testing time to exhibit an AM, 23 (12%) had baseline CD too mild to allow confident rating of AM effect, and 1 site- and 1 video-rater each rated the AM item with a reverse scoring convention. When these confounds were eliminated in step-wise fashion, the item's clinimetric properties improved. CONCLUSIONS: The AM's efficacy can contribute to measuring CD motor severity by addressing identified sources of error during its assessment and rating. Given the AM's sensitive diagnostic and potential pathophysiologic significance, we also provide guidance on modifications to how AMs can be assessed in future CD research.

Postural Directionality and Head Tremor in Cervical Dystonia.

Background: Although abnormal head and neck postures are defining features of cervical dystonia (CD), head tremor (HT) is also common. However, little is known about the relationship between abnormal postures and HT in CD. Methods: We analyzed clinical data and video recordings from 185 patients enrolled by the Dystonia Coalition. We calculated the likelihood of their HT and HT type ("regular" vs. "jerky") given directionality of abnormal head postures, disease duration, sex, and age. Results: Patients with retrocollis were more likely to have HT than patients with anterocollis (X2 (1, N = 121) = 7.98, p = 0.005). There was no difference in HT likelihood given left or right turning in laterocollis and rotation. Patients with HT had longer disease duration (t(183) = 2.27, p = 0.024). There was no difference in age between patients with and without HT. In a logistic regression model, anterocollis/retrocollis direction (X2 (1, N = 121) = 6.04, p = 0.014), disease duration (X2 (1, N = 121) = 7.28, p = 0.007), and the interaction term between age and disease duration (X2 (1, N = 121) = 7.77, p = 0.005) collectively contributed to HT likelihood. None of the postural directionality or demographic variables were associated with differential likelihood of having regular versus jerky HT. Discussion: We found that HT is more likely for CD patients with a specific directionality in their predominant posture. Our finding that CD patients with longer disease duration have a higher likelihood of HT also raises the question of whether HT becomes more likely over time in individual patients.

Sensory Tricks Are Associated with Higher Sleep-Related Quality of Life in Cervical Dystonia.

Background: Sensory tricks are compensatory gestures that cervical dystonia (CD) patients use to reduce abnormal neck posture and movements. Although sensory tricks are common in CD, little is known about whether trick efficacy changes over time or has effect on quality of life. Methods: We analyzed clinical data and video recordings from 188 patients with isolated CD. We calculated the duration of CD and assessed the Toronto Western Spasmodic Torticollis Rating Scales and the Cervical Dystonia Impact Profile (CDIP-58). Results: A longer duration of CD corresponded to a less effective sensory trick (r(187) = 0.1901, p = 0.009). Patients who demonstrated more effective sensory tricks reported higher sleep-related quality of life than patients with less effective sensory tricks (r(187) = 0.1680, p = 0.0212). There were no significant relationships between the effectiveness of a sensory trick and the other aspects of quality of life as measured by the CDIP-58. Discussion: Patients who have had CD longer had less effective sensory tricks consistent with patients' verbal reports of previously having a trick that no longer works. Patients should be apprised of a wide variety of sensory tricks because their previous tricks may lose efficacy over time and because more effective tricks are associated with higher sleep-related quality of life.

[Knowledge, attitudes, perceptions and community practices for urban malaria. Tumaco, Colombia]. / Conocimientos, actitudes, percepciones y prácticas comunitarias sobre la malaria urbana. Tumaco, Colombia.

OBJECTIVE: To describe the condition of malaria in the District 5 of San Andres de Tumaco-Nariño-Colombia, with regards to knowledge, attitudes, perceptions, and community practices. METHODS: A cross-sectional study was conducted between January and May 2014. Logistic analysis of the CAP-PP survey of the Pan American Health Organization (PAHO) was carried out. Statistical modeling was adjusted through the logarithm of likelihood test, the Pseudo R2, the Akaike's Information Criterion (AIC) and the Bayesian Information Criterion (BIC). Results. The variables that influence the disease of malaria are: taking medicines, malaria tests and vector control by spraying at home. It is important to keep clean ditches around the house in order for people to be protected against the disease. CONCLUSION: Although the population reported having knowledge, attitudes, community practices, and perceptions about the issue, these do not generate protection against the disease. Variables such as carrying out activities in the family and ignorance about the name of the mosquito that transmits malaria must be taken into account for the formulation of community intervention plans.

OBJETIVO: Describir el padecimiento de la malaria en la Comuna 5 de San Andrés de Tumaco-Nariño, Colombia, en cuanto a conocimientos, actitudes, percepciones, y prácticas comunitarias. MATERIALES Y MÉTODOS: Estudio de corte transversal, realizado entre enero y mayo de 2014. Se realizó análisis logístico de la encuesta CAP-PP de la Organización Panamericana de la Salud (OPS). El modelamiento estadístico se ajustó a través del test del logaritmo de la verosimilitud, el Pseudo R2, el Akaike's Information Criterion (AIC) y el Bayesian Information Criterion (BIC). Resultados Las variables que influyen en el padecimiento de la malaria son: tomar medicamentos, examen para malaria y control de vectores mediante rociamiento en casa. Es importante mantener las zanjas limpias alrededor de la vivienda para protegerse contra la enfermedad. CONCLUSIÓN: A pesar de que la población manifiesta tener conocimientos, actitudes, prácticas comunitarias y percepciones, estas no generan protección contra la enfermedad. Variables como la realización de actividades en la familia y el desconocimiento del nombre del mosquito que trasmite la malaria deben ser tenidas en cuenta para la formulación de planes de intervención comunitaria.

Conocimientos, actitudes, percepciones y prácticas comunitarias sobre la malaria urbana. Tumaco, Colombia / Knowledge, attitudes, perceptions and community practices for urban malaria. Tumaco, Colombia

RESUMEN Objetivo Describir el padecimiento de la malaria en la Comuna 5 de San Andrés de Tumaco-Nariño, Colombia, en cuanto a conocimientos, actitudes, percepciones, y prácticas comunitarias. Materiales y Métodos Estudio de corte transversal, realizado entre enero y mayo de 2014. Se realizó análisis logístico de la encuesta CAP-PP de la Organización Panamericana de la Salud (OPS). El modelamiento estadístico se ajustó a través del test del logaritmo de la verosimilitud, el Pseudo R2, el Akaike's Information Criterion (AIC) y el Bayesian Information Criterion (BIC). Resultados Las variables que influyen en el padecimiento de la malaria son: tomar medicamentos, examen para malaria y control de vectores mediante rociamiento en casa. Es importante mantener las zanjas limpias alrededor de la vivienda para protegerse contra la enfermedad. Conclusión A pesar de que la población manifiesta tener conocimientos, actitudes, prácticas comunitarias y percepciones, estas no generan protección contra la enfermedad. Variables como la realización de actividades en la familia y el desconocimiento del nombre del mosquito que trasmite la malaria deben ser tenidas en cuenta para la formulación de planes de intervención comunitaria.(AU)

ABSTRACT Objective To describe the condition of malaria in the District 5 of San Andres de Tumaco-Nariño-Colombia, with regards to knowledge, attitudes, perceptions, and community practices. Methods A cross-sectional study was conducted between January and May 2014. Logistic analysis of the CAP-PP survey of the Pan American Health Organization (PAHO) was carried out. Statistical modeling was adjusted through the logarithm of likelihood test, the Pseudo R2, the Akaike's Information Criterion (AIC) and the Bayesian Information Criterion (BIC). Results. The variables that influence the disease of malaria are: taking medicines, malaria tests and vector control by spraying at home. It is important to keep clean ditches around the house in order for people to be protected against the disease. Conclusion Although the population reported having knowledge, attitudes, community practices, and perceptions about the issue, these do not generate protection against the disease. Variables such as carrying out activities in the family and ignorance about the name of the mosquito that transmits malaria must be taken into account for the formulation of community intervention plans.(AU)