Background: Presence of truncal dystonia usually points to a secondary cause of dystonia like exposure to dopamine receptor blockers or neurodegenerative illness. Rarely, it can occur as an idiopathic focal or segmental dystonia. Methods: Retrospective review of medical records and videos of patients of truncal dystonia presenting in the Botulinum Toxin Clinic of Department of Neurology at Post Graduate Institute of Medical Education and Research, Chandigarh between May 2016 and February 2019. Results: A total of 16 patients with predominant truncal dystonia were recruited. There were ten males and six females with mean age of 49.1 ± 15.1 years (range 22–70). Extensor truncal dystonia was the most common (12/16) followed by camptocormia (4/16). Various etiologies included Idiopathic Parkinson’s disease (4/16), Tardive dystonia (5/16), Neurodegeneration with brain iron accumulation (genetically confirmed) (2/16) and idiopathic (5/16). All patients were refractory to a combination of oral medications tried over a period of 1.82 ± 1.93 years. All patients received electromyographic-guided botulinum toxin in paraspinals or rectus abdominis muscles depending upon the type of dystonia. The mean dose of abobotulinum toxin used was 286.7 ± 108.6 units (range 200–500 units) for paraspinals and 297.5 ± 68.5 (range 200–350) for rectus abdominis muscles per session. Average subjective response after botulinum toxin injection session was 31.2 ± 21.5% (range 0–70). No adverse effects were reported. Conclusion: Botulinum toxin is an acceptable alternative to patients presenting with medically refractory truncal dystonia and may offer modest benefit.


中文翻译：

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躯干肌张力障碍谱和治疗反应：回顾性分析


背景：躯干肌张力障碍的存在通常表明肌张力障碍的次要原因，如暴露于多巴胺受体阻滞剂或神经退行性疾病。极少数情况下，它可以作为特发性局灶性或节段性肌张力障碍发生。方法：回顾性回顾 2016 年 5 月至 2019 年 2 月在昌迪加尔医学教育与研究研究生院神经内科肉毒杆菌毒素诊所就诊的躯干肌张力障碍患者的病历和视频。结果：共招募了 16 名主要躯干肌张力障碍患者。有 10 名男性和 6 名女性，平均年龄为 49.1 ± 15.1 岁（范围 22-70）。伸肌躯干肌张力障碍最常见（12/16），其次是弯曲性（4/16）。各种病因包括特发性帕金森病 (4/16)、迟发性肌张力障碍 (5/16)、伴有脑铁积累的神经退行性病变 (基因证实) (2/16) 和特发性 (5/16)。在 1.82 ± 1.93 年的时间里，所有患者都对口服药物组合无效。根据肌张力障碍的类型，所有患者都接受了肌电图引导下的椎旁肌或腹直肌肉毒杆菌毒素治疗。使用的肉毒杆菌毒素的平均剂量为 286.7 ± 108.6 单位（范围 200-500 单位），用于脊髓旁和 297.5 ± 68。5（范围 200-350）腹直肌每次训练。肉毒杆菌毒素注射后的平均主观反应为 31.2 ± 21.5%（范围 0-70）。没有报告任何不良反应。结论：肉毒杆菌毒素是医学上难治性躯干肌张力障碍患者的可接受替代品，并且可能提供适度的益处。