The purpose of this study was to investigate the impact of botulinum toxin (BoNT) treatment on the quality of life (QoL) for patients with prominent lingual dystonia (LD) using a disease-specific questionnaire, the oromandibular dystonia questionnaire-25 (OMDQ-25). This is a prospective, observational study of a cohort of 30 patients treated with BoNT injections for LD, with or without concurrent jaw dystonia. Primary efficacy outcome was the absolute difference between total OMDQ-25 baseline score and total OMDQ-25 scores 4 and 8 weeks after the treatment. Safety outcome was the occurrence of adverse effects. The mean total OMDQ-25 baseline score was 46.8 ± 17.8. After BoNT treatment, there was a significant reduction in the mean total OMDQ-25 score at 4 weeks (38.2 ± 17.6; p = 0.004), as well as at 8 weeks (39.6 ± 18.1; p = 0.008). At the multiple regression analysis, a jaw deviation pattern (JDD) and high questionnaire baseline total score were detected as predictors of a better outcome, whilst associated jaw tremor was a predictor of poor outcome. In patients with JDD, jaw-opening muscles were more frequently injected and genioglossus less frequently than in patients without JDD. No major adverse events were detected. A consistent and measurable improvement in QoL, with good safety and tolerability, can be achieved in patients with prominent LD by injecting BoNT into genioglossus and/or other muscles of the oromandibular region.

Background: Patients with neuromuscular diseases (NMDs) require long-term multidisciplinary care. Access to specialised NMD services is still variable across the UK. In 2012, an audit showed a high frequency of emergency admissions in this patient population, which were more likely to happen if care was poorly coordinated and not proactive in preventing NMDs complications. The audit was followed by several recommendations and a partnership approach project. Aims: To assess the impact of the previous Audit Methods: A re-audit was undertaken across the same specialised commissioning groups in Central and SouthEast England. Results: More patients were known to specialised centres in NMDs, and the majority of admissions were under the care of Neurosciences (77%) as compared to 2012 when only 14.9% were admitted under Neuroscience. Improvements included a reduction in preventable admissions directly related to known NMDs (from 63% to 32.8%) and reduction in re-admissions (from 25.1% to 12.4%). The mortality rate dropped from 4.5% to 0.3%. Patients known to a NMD specialised service had shorter hospital stay and fewer ITU admissions than patients who were not known to specialised services. Conclusion: The re-audit on unplanned hospital admissions in England highlighted the importance of coordination of care at specialised services for patients with neuromuscular disorders. It successfully documented improvements in the care provided to patients with NMDs in the assessed regions.

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