Effectiveness of Unilateral Pallidotomy for Meige Syndrome Confirmed by Motion Analysis

Background Many rating scales have been applied to the evaluation of dystonia, but only few have been assessed for clinimetric properties. The Movement Disorders Society commissioned this task force to critique existing dystonia rating scales and place them in the clinical and clinimetric context. Methods A systematic literature review was conducted to identify rating scales that have either been validated or used in dystonia. Results Thirty six potential scales were identified. Eight were excluded because they did not meet review criteria, leaving twenty-eight scales that were critiqued and rated by the task force. Seven scales were found to meet criteria to be “recommended”: the Blepharospasm Disability Index is recommended for rating blepharospasm; the Cervical Dystonia Impact Scale and the Toronto Western Spasmodic Torticollis Rating Scale for rating cervical dystonia; the Craniocervical Dystonia Questionnaire for blepharospasm and cervical dystonia; the Voice Handicap Index (VHI) and the Vocal Performance Questionnaire (VPQ) for laryngeal dystonia; and the Fahn-Marsden Dystonia Rating Scale for rating generalized dystonia. Two “recommended” scales (VHI and VPQ) are generic scales validated on few patients with laryngeal dystonia, whereas the others are disease-specific scales. Twelve scales met criteria for “suggested” and seven scales met criteria for “listed”. All the scales are individually reviewed in the online appendix. Conclusion The task force recommends five specific dystonia scales and suggests to further validate in dystonia two recommended generic voice-disorder scales. Existing scales for oromandibular, arm and task-specific dystonia should be refined and fully assessed. Scales should be developed for body regions where no scales are available, such as lower limbs and trunk.

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“…The FMDRS has been used in numerous studies to determine the treatment effects of deep brain stimulation, including childhood‐onset dystonia …”

Section: Resultsmentioning

confidence: 99%

Dystonia rating scales: Critique and recommendations

Albanese

Sorbo

Comella³

et al. 2013

Movement Disorders

161

136

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“…Severe cases of DT in the setting of a generalised dystonia have been successfully treated with DBS of the globus pallidus internus (Gpi),49 although none of the larger DBS trials for dystonia have specifically taken into account the effect on tremor. Seven cases were treated with only GPi stimulation or ablation: two had dramatic improvements,50 51 four had moderate improvement,52 whereas one with cervical DT did not improve 53. Stimulation of the thalamic ventralis intermedius nucleus (Vim) or ablations within the same region improved tremor in all cases in which they were employed: in 11 cases as unique target54–58 and in seven cases combined with GPi DBS 58–61.…”

Section: Resultsmentioning

confidence: 99%

“…However, in Japan where calligraphy is an important occupation, neurosurgery has been successfully performed for this condition, as it can threaten the professional career of the patient 68. Taking into account the potential ameliorative effect on the other dystonic features, GPi should be viewed as the preferred target for either stimulation50 or ablation51; thalamic stimulation may be added in cases in which the benefit is insufficient,59–61 because it can also alleviate the tremor but can occasionally lead to worsening of dystonia itself 54 56–58. Stimulation of thalamic VLp is considered as less efficacious in dystonia and tremor treatment than GPi DBS 62.…”

Section: Discussion and Recommendationsmentioning

confidence: 99%

The treatment of dystonic tremor: a systematic review

Fasano

Bove

Lang

2013

Journal of Neurology, Neurosurgery & Psychiatry

115

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Tremor is one of the clinical manifestations of dystonia; however, there are no specific therapeutic trials evaluating the efficacy of treatments for dystonic tremor (DT), tremor associated with dystonia or primary writing tremor (PWT). We systematically reviewed the literature available up to July 2013 on the treatment of these tremors and retrieved the data of 487 patients published in 43 papers detailing the effects of given interventions on tremor severity. Treatment outcome was highly variable, depending on the specific type of intervention and tremor distribution. No specifically designed studies were available for the treatment of tremor associated with dystonia. As for the other tremors, drug efficacy was generally disappointing and a moderate effect was only found with anticholinergics, tetrabenazine, clonazepam, β-blockers and primidone; levodopa was only efficacious on tremor due to dopa-responsive dystonia. The largest amount of data was available for botulinum toxin injections, which provided a marked improvement, particularly for the management of axial tremors (head or vocal cords). In refractory DTs, deep brain stimulation of several targets was attempted. Deep brain stimulation of globus pallidus internus, thalamus or subthalamic area led to a marked improvement of dystonic axial or appendicular tremors in most cases refractory to other treatments. Few other non-invasive treatments, for example, orthotic device in PWT, have been used with anecdotal success. In conclusion, considering the lack of good-quality studies, future randomised controlled trials are needed. In absence of evidence-based guidelines, we propose an algorithm for the treatment of DT based on currently available data.

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“…This occurs in the following settings: (1) advanced dystonia, in which body habitus and extreme dystonic contractions predispose to hardware‐related complications, and life‐threatening status dystonicus or “dystonic storm,” in which the time necessary for repeated programming sessions is disadvantageous; (2) early childhood dystonia, in which, again, body‐related issues preclude a neurostimulator system; and/or (3) pre‐existing neurological deficits caused by dystonia, including speech disturbances (such as advanced dysphonia and dysarthria and/or dysphagia with feeding tube), which make the risk of such effects from bilateral pallidotomies less of a concern . Second , some patients may not wish to have an implanted neurostimulator system because of hardware considerations, or it is not feasible to implant 1 due to decreased access to a programming center. Unilateral pallidotomy is reasonable in these situations, but careful deliberation is necessary before contralateral pallidotomy given the risk (albeit small but still not well quantified) of speech and swallowing complications.…”

Section: When Is Medical Therapy Not Enough?mentioning

confidence: 99%

What's new in surgical treatment for dystonia?

2013

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It is now established that pallidal deep brain stimulation (DBS) is effective in the treatment of generalized and segmental primary dystonia, although there is still insufficient evidence to support its benefit in focal and secondary dystonia. Because several studies have demonstrated that pallidal DBS improves quality of life (QoL), reduced QoL and disability that are nonresponsive to medical treatment are probably the main factors guiding the decision to consider surgery. Some studies have indicated that young patients with primary dystonia who have shorter disease duration and less severe dystonia are likely to have the best outcome from DBS. Therefore, surgery should not be delayed when disability and QoL are impaired to the extent that justifies the surgical risk. A case-by-case approach is recommended in patients who have secondary dystonia. The globus pallidus internus is considered the best target for dystonia. There are still not enough data about the effectiveness of thalamic, subthalamic nucleus, and premotor cortex stimulation. Targeting with multiple electrodes and intra-individual comparisons of outcomes may help determine which target would be more beneficial. With regard to the role of lesions, pallidotomy for dystonia is still performed in several countries and can play a role in selected patients. New technologies are already available to improve the stimulation programming for DBS patients and to increase battery longevity. In the near future, it is possible that we will be able to shape stimulation settings according to disease type and symptoms. © 2013 Movement Disorder Society.

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Effectiveness of Unilateral Pallidotomy for Meige Syndrome Confirmed by Motion Analysis

Cited by 19 publications

References 41 publications

Dystonia rating scales: Critique and recommendations

Dystonia rating scales: Critique and recommendations

The treatment of dystonic tremor: a systematic review

What's new in surgical treatment for dystonia?

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