Clinical and Demographic Characteristics of Upper Limb Dystonia

Norris, Scott A.; Jinnah, Hyder A.; Klein, Christine; Jankovic, Joseph; Berman, Brian D.; Roze, Emmanuel; Mahajan, Abhimanyu; Espay, Alberto J.; Murthy, Avinash V.; Fung, Victor S.C.; LeDoux, Mark S.; Chang, Florence; Vidailhet, Marie; Testa, Claudia; Barbano, Richard L.; Malaty, Irene A.; Bäumer, Tobias; Loens, Sebastian; Wright, Laura; Perlmutter, Joel S.

doi:10.1002/mds.28223

Cited by 10 publications

(10 citation statements)

References 34 publications

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“…Whereas the design of this earlier prospective study minimizes recall bias concerning onset by restricting disease duration to 5 years or less, it also carries a higher risk of misclassifying as "non‐spreading" those individuals who might develop spread later in the disease course. We also observed a slightly higher prevalence of spread compared to the previous literature in patients with upper limb onset [38,39] (52%) and laryngeal onset [40] (41%), which, as mentioned above, might be attributable to small differences in recall bias across studies. As expected, spread was more prevalent for all onset region subgroups when tremor was included as a standalone manifestation of disease.…”

Section: Discussionsupporting

confidence: 62%

Predictive modeling of spread in adult‐onset isolated dystonia: Key properties and effect of tremor inclusion

Wang

Sajobi

Morgante

et al. 2021

Euro J of Neurology

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Background and purpose Several clinical and demographic factors relate to anatomic spread of adult‐onset isolated dystonia, but a predictive model is still lacking. The aims of this study were: (i) to develop and validate a predictive model of anatomic spread of adult‐onset isolated dystonia; and (ii) to evaluate whether presence of tremor associated with dystonia influences model predictions of spread. Methods Adult‐onset isolated dystonia participants with focal onset from the Dystonia Coalition Natural History Project database were included. We developed two prediction models, one with dystonia as sole disease manifestation (“dystonia‐only”) and one accepting dystonia OR tremor in any body part as disease manifestations (“dystonia OR tremor”). Demographic and clinical predictors were selected based on previous evidence, clinical plausibility of association with spread, or both. We used logistic regressions and evaluated model discrimination and calibration. Internal validation was carried out based on bootstrapping. Results Both predictive models showed an area under the curve of 0.65 (95% confidence intervals 0.62–0.70 and 0.62–0.69, respectively) and good calibration after internal validation. In both models, onset of dystonia in body regions other than the neck, older age, depression and history of neck trauma were predictors of spread. Conclusions This predictive modeling of spread in adult‐onset isolated dystonia based on accessible predictors (demographic and clinical) can be easily implemented to inform individuals’ risk of spread. Because tremor did not influence prediction of spread, our results support the argument that tremor is a part of the dystonia syndrome, and not an independent or coincidental disorder.

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Section: Discussionsupporting

confidence: 62%

Predictive modeling of spread in adult‐onset isolated dystonia: Key properties and effect of tremor inclusion

Wang

Sajobi

Morgante

et al. 2021

Euro J of Neurology

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“…In the clinical evaluation of OMD [24], SD [25], and upper limb dystonia [26,27], it is very important to exclude non-neurological conditions that can mimic dystonic postures (pseudodystonias) [19].…”

Section: Oromandibular Laryngeal and Upper Limb Dystoniasmentioning

confidence: 99%

Diagnostic and therapeutic recommendations in adult dystonia: a joint document by the Italian Society of Neurology, the Italian Academy for the Study of Parkinson’s Disease and Movement Disorders, and the Italian Network on Botulinum Toxin

et al. 2022

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The diagnostic framework and the therapeutic management of patients with adult dystonia can represent a challenge for clinical neurologists. The objective of the present paper is to delineate diagnostic and therapeutic recommendations for dystonia provided by a panel of Italian experts afferent to the Italian Society of Neurology, the Italian Academy for the Study of Parkinson's Disease and Movement Disorders, and the Italian Network on Botulinum Toxin. We first discuss the clinical approach and the instrumental assessment useful for diagnostic purpose. Then, we analyze the pharmacological, surgical, and rehabilitative therapeutic options for adult dystonia. Finally, we propose a hospital-territory network model for adult dystonia management.

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“…Clinical details of the study population have been presented several times 10,11,15–18 . Briefly, mean age was 60.6 ± 10.4 years (range 18 to 92), and 70.9% were females.…”

Section: Resultsmentioning

confidence: 99%

An Empirical Comparison of Commonly Used Universal Rating Scales for Dystonia

Boz,

Kilic‐Berkmen,

Perlmutter

et al. 2023

Movement Disord Clin Pract

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BackgroundThere are several widely used clinical rating scales for documenting the severity and distribution of various types of dystonia.ObjectivesThe 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.MethodsGlobal 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.ResultsThere 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.ConclusionsThe 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.This article is protected by copyright. All rights reserved.

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Clinical and Demographic Characteristics of Upper Limb Dystonia

Cited by 10 publications

References 34 publications

Predictive modeling of spread in adult‐onset isolated dystonia: Key properties and effect of tremor inclusion

Predictive modeling of spread in adult‐onset isolated dystonia: Key properties and effect of tremor inclusion

Diagnostic and therapeutic recommendations in adult dystonia: a joint document by the Italian Society of Neurology, the Italian Academy for the Study of Parkinson’s Disease and Movement Disorders, and the Italian Network on Botulinum Toxin

An Empirical Comparison of Commonly Used Universal Rating Scales for Dystonia

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