The most common forms of dystonia are those that develop in adults and affect a relatively isolated region of the body. Although these adult-onset focal dystonias are most prevalent, knowledge of their etiologies and pathogenesis has lagged behind some of the rarer generalized dystonias, where the identification of genetic defects has facilitated both basic and clinical research. This summary provides a brief review of the clinical manifestations of the adult-onset focal dystonias, focussing attention on less well-understood clinical manifestations that need further study. It also provides a simple conceptual model for the similarities and differences among the different adult-onset focal dystonias, as a rationale for lumping them together as a class of disorders while at the same time splitting them into subtypes. The concluding section outlines some of the most important research questions for the future. Answers to these questions are critical for advancing our understanding of this group of disorders, and for developing novel therapeutics.

/pdf/the-focal-dystonias-current-views-and-challenges-for-future-2sue2q26kj.pdf

The Focal Dystonias: Current Views and Challenges for Future Research

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. A systematic literature review was conducted to identify rating scales that have either been validated or used in dystonia. Thirty-six potential scales were identified. Eight were excluded because they did not meet review criteria, leaving 28 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 7 scales met criteria for "listed." All the scales are individually reviewed in the online information. The task force recommends 5 specific dystonia scales and suggests to further validate 2 recommended generic voice-disorder scales in dystonia. Existing scales for oromandibular, arm, and task-specific dystonia should be refined and fully assessed. Scales should be developed for body regions for which no scales are available, such as lower limbs and trunk.

/pdf/dystonia-rating-scales-critique-and-recommendations-1wz5jeb3ro.pdf

Dystonia rating scales: critique and recommendations

Botulinun neurotoxin (BoNT) has emerged as one of the most multipurpose therapeutic agents in modern medicine with more clinical applications than any other drug currently on the market. Initially developed in the treatment of strabismus and neurologic movement disorders, the use of botulinun neurotoxin has been expanding during the past 3 decades to include the treatment of a variety of ophthalmologic, gastrointestinal, urologic, orthopedic, dermatologic, dental, secretory, painful, cosmetic, and other conditions. In addition to onabotulinumtoxinA (Botox), abobotulinumtoxinA (Dysport), incobotulinumtoxinA (Xeomin), and RimabotulinumtoxinB (Myobloc or NeuroBloc) there are other novel botulinun neurotoxin products currently in development. With a better understanding of the cellular mechanisms of botulinun neurotoxin and advances in biotechnology, future botulinun neurotoxin products will likely be even more effective and customized to the specific indication and tailored to the needs of the patients. © 2017 International Parkinson and Movement Disorder Society.

Botulinum toxin: State of the art

Hemifacial spasm: The past, present and future

Botulinum toxin (BoNT) has been used for the treatment of a variety of neurologic, medical and cosmetic conditions. Two serotypes, type A (BoNT-A) and type B (BoNT-B), are currently in clinical use. While considered safe and effective, their use has been rarely complicated by the development of antibodies that reduce or negate their therapeutic effect. The presence of antibodies has been attributed to shorter dosing intervals (and booster injections), higher doses per injection cycle, and higher amounts of antigenic protein. Other factors contributing to the immunogenicity of BoNT include properties of each serotype, such as formulation, manufacturing, and storage of the toxin. Some newer formulations with purified core neurotoxin devoid of accessory proteins may have lower overall immunogenicity. Several assays are available for the detection of antibodies, including both structural assays such as ELISA and mouse-based bioassays, but there is no consistent correlation between these antibodies and clinical response. Prevention and treatment of antibody-associated non-responsiveness is challenging and primarily involves the use of less immunogenic formulations of BoNT, waiting for the spontaneous disappearance of the neutralizing antibody, and switching to an immunologically alternate type of BoNT.

https://www.mdpi.com/2072-6651/11/9/491/pdf

Immunogenicity Associated with Botulinum Toxin Treatment.

Objective:Botulinum toxin is widely utilized as a first-line therapy for cervical dystonia (CD). Numerous studies have demonstrated the efficacy and safety of this treatment, but little data exist on patient satisfaction. To address this question, a structured patient survey was conducted in Germany, France, the US, and Canada (n = 136 patients with CD).Methods:Specific information was collected on the patients’ current and prior botulinum toxin treatment cycles and their overall quality-of-life (including completion of the Cervical Dystonia Impact Profile-58 [CDIP-58]).Results:Patients rated the mean onset of action for their previous injection as 3.8 days, with peak effect at 3.6 weeks and a decline in effects at 9.5 weeks. While most patients were satisfied with their current therapy, only 50.7% were very satisfied, 42.6% were somewhat satisfied, and 6.6% not at all satisfied with their current therapy. Patient satisfaction was lowest just prior to injection and highest at the time of peak effe...

Satisfaction with botulinum toxin treatment: a cross-sectional survey of patients with cervical dystonia.

Objective: Botulinum toxin is widely utilized as a first-line therapy for cervical dystonia (CD). Numerous studies have demonstrated the efficacy and safety of this treatment, but little data exist on patient satisfaction. To address this question, a structured patient survey was conducted in Germany, France, the US, and Canada (n ¼ 136 patients with CD).

https://informahealthcare.com/doi/pdf/10.3111/13696998.2011.653726

Original article Satisfaction with botulinum toxin treatment: a cross-sectional survey of patients with cervical dystonia

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Satisfaction with botulinum toxin treatment: a cross-sectional survey of patients with cervical dystonia.

Original article Satisfaction with botulinum toxin treatment: a cross-sectional survey of patients with cervical dystonia