Abstract:This document presents a consensus on the diagnosis and classification of isolated cervical dystonia (iCD) with a review of proposed terminology. The International Parkinson and Movement Disorder Society Dystonia Study Group convened a panel of experts to review the main clinical and diagnostic issues related to iCD and to arrive at a consensus on diagnostic criteria and classification. These criteria are intended for use in clinical research, but also may be used to guide clinical practice. The benchmark is e… Show more
“…Unlike the Dystonia Coalition database, 17 the CD PROBE registry 9 protocol with data collected from 2009 to 2012 did not require data to be collected on involvement of body regions beyond head and neck or on presence of other phenomenology. This limits our ability to classify subjects in the CD PROBE registry using 2023 criteria 4 . It is possible that a poor response to sensory trick could be because those subjects did not have idiopathic isolated CD.…”
Section: Discussionmentioning
confidence: 99%
“…Sensory tricks are thought to be most effective early on in the disease course and may completely alleviate posturing and associated disability in some individuals 2,3 . Their importance in CD is demonstrated by their inclusion as a supportive diagnostic criterion 4 . Imaging and neurophysiology studies suggest that sensory tricks act via modulation of the abnormal network by peripheral sensory feedback, in particular by involvement of the sensorimotor cortex 5 .…”
mentioning
confidence: 99%
“…2,3 Their importance in CD is demonstrated by their inclusion as a supportive diagnostic criterion. 4 Imaging and neurophysiology studies suggest that sensory tricks act via modulation of the abnormal network by peripheral sensory feedback, in particular by involvement of the sensorimotor cortex. 5 A study using functional MRI showed increased functional connectivity of sensorimotor network in CD subjects without a sensory trick, while an effective sensory trick was associated with a decrease in connectivity in the same network.…”
BackgroundSensory tricks (STs) are voluntary maneuvers that dampen the abnormal movement in cervical dystonia (CD).ObjectivesTo investigate the effect of ST on CD severity and treatment.MethodsData on 1039 individuals with a modified Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) score were extracted from the CD Patient Registry for Observation of OnabotulinumtoxinA (onabotA) Efficacy study. Univariate and multivariate models evaluated the direct and indirect impact of ST on CD severity and treatment, while controlling for confounds.ResultsComplete ST was associated with a 10% lower mean onabotA dose. Absence of complete ST was associated with a higher onabotA dose after controlling for dystonia severity (OR = 1.37, p = .04). ST moderated the relationship between dystonia severity and toxin dose (β = −.16, p = .02).ConclusionsST is related to lower CD severity and toxin dose. It may have a direct effect on lowering toxin dose, independent of CD severity.This article is protected by copyright. All rights reserved.
“…Unlike the Dystonia Coalition database, 17 the CD PROBE registry 9 protocol with data collected from 2009 to 2012 did not require data to be collected on involvement of body regions beyond head and neck or on presence of other phenomenology. This limits our ability to classify subjects in the CD PROBE registry using 2023 criteria 4 . It is possible that a poor response to sensory trick could be because those subjects did not have idiopathic isolated CD.…”
Section: Discussionmentioning
confidence: 99%
“…Sensory tricks are thought to be most effective early on in the disease course and may completely alleviate posturing and associated disability in some individuals 2,3 . Their importance in CD is demonstrated by their inclusion as a supportive diagnostic criterion 4 . Imaging and neurophysiology studies suggest that sensory tricks act via modulation of the abnormal network by peripheral sensory feedback, in particular by involvement of the sensorimotor cortex 5 .…”
mentioning
confidence: 99%
“…2,3 Their importance in CD is demonstrated by their inclusion as a supportive diagnostic criterion. 4 Imaging and neurophysiology studies suggest that sensory tricks act via modulation of the abnormal network by peripheral sensory feedback, in particular by involvement of the sensorimotor cortex. 5 A study using functional MRI showed increased functional connectivity of sensorimotor network in CD subjects without a sensory trick, while an effective sensory trick was associated with a decrease in connectivity in the same network.…”
BackgroundSensory tricks (STs) are voluntary maneuvers that dampen the abnormal movement in cervical dystonia (CD).ObjectivesTo investigate the effect of ST on CD severity and treatment.MethodsData on 1039 individuals with a modified Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) score were extracted from the CD Patient Registry for Observation of OnabotulinumtoxinA (onabotA) Efficacy study. Univariate and multivariate models evaluated the direct and indirect impact of ST on CD severity and treatment, while controlling for confounds.ResultsComplete ST was associated with a 10% lower mean onabotA dose. Absence of complete ST was associated with a higher onabotA dose after controlling for dystonia severity (OR = 1.37, p = .04). ST moderated the relationship between dystonia severity and toxin dose (β = −.16, p = .02).ConclusionsST is related to lower CD severity and toxin dose. It may have a direct effect on lowering toxin dose, independent of CD severity.This article is protected by copyright. All rights reserved.
“…This study provides a recommendation for cervical dystonia, in which is proposed to reserve the diagnosis of focal cervical dystonia for those patients in which only muscles that move the neck are involved [6 ▪ ] (Table 1). Recently, the MDS has published a consensus statement regarding the classification of idiopathic cervical dystonia, differentiating between the two levels ‘definite’ and ‘probably’ idiopathic cervical dystonia [8 ▪▪ ]. Supportive criteria and exclusion criteria for cervical dystonia diagnosis were identified (Tables 2 and 3).…”
Section: Clinical Features and Recommendations For Diagnosismentioning
confidence: 99%
“…Supportive criteria and exclusion criteria for cervical dystonia diagnosis were identified (Tables 2 and 3). Additionally, required criteria are also provided for diagnosis of genetic and acquired cervical dystonia [8 ▪▪ ].…”
Section: Clinical Features and Recommendations For Diagnosismentioning
Purpose of review
The aim of this review is to showcase the recent developments in the field of diagnosis and treatment of adult-onset focal dystonia.
Recent findings
Accurate phenotyping of focal dystonia is essential in the process of finding an underlying cause, including acquired, genetic, and idiopathic causes. Motor symptoms as well as the associated nonmotor symptoms and their detrimental impact on quality of life have received increased interest over the last years. The diagnostic process is complicated by the steadily increasing numbers of newly discovered genes associated with dystonia. Recent efforts have been aimed at further developing recommendations and algorithms to aid in diagnosis and in navigating the use of diagnostic tools. In terms of treatment, research on DBS is advancing towards a better understanding of the most effective stimulation locations within the globus pallidus. Moreover, with the introduction of the LFP-recording devices, the search continues for an accurate electrophysiological biomarker for dystonia.
Summary
Accurate phenotyping and (sub)classification of patients with dystonia is important for improving diagnosis, subsequent treatment effect and population-based study outcomes in research. Medical practitioners should be attentive to the presence of nonmotor symptoms in dystonia.
BackgroundThalamic deep brain stimulation (Th‐DBS) is established for medically refractory tremor syndromes and globus pallidus stimulation (GPi‐DBS) for medically refractory dystonia syndromes. For combined tremor and dystonia syndromes, the best target is unclear.ObjectivesWe present four patients with two different profiles whose clinical course demonstrates that our current analysis of clinical symptomatology is not a sufficient predictor of surgical success.MethodsOutcome parameters were assessed with observer‐blinded video ratings and included the Fahn‐Tolosa‐Marin‐Tremor Rating Scale (FTM‐TRS) and the Unified Dystonia Rating Scale (UDRS).ResultsTwo patients with ‘predominant lateralized action tremor’ of the hands and mild cervical dystonia showed no relevant tremor improvement after GPi‐DBS, but UDRS improved (mean: 45%). Rescue Vim‐DBS electrodes were implanted and both patients benefited significantly with a mean tremor reduction of 51%.Two other patients with ‘axial‐predominant action tremor of the trunk and head’ associated with cervical dystonia underwent bilateral Vim DBS implantation with little effect on tremor (24% reduction in mean FTM‐TRS total score) and no effect on dystonic symptoms. GPi rescue DBS was implanted and showed a significant effect on tremor (63% reduction in mean FTM‐TRS) and dystonia (49% reduction in UDRS).ConclusionsThe diagnosis of dystonic tremor alone is not a sufficient predictor to establish the differential indication of Gpi‐ or Vim‐DBS. Further criteria (e.g., proximal‐distal distribution of tremor/dystonia) are needed to avoid rescue surgery in the future. On the other hand, the course of our patients encourages rescue surgery in such severely disabled patients if the first target fails.This article is protected by copyright. All rights reserved.
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