Sławek Jarosław, Łobińska Iga Alicja, Schinwelski Michał, Kopcewicz-Wiśniewska Joanna, Castagna Anna
Division of Neurological-Psychiatric Nursing, Faculty of Health Sciences, Medical University of Gdańsk, 80-210 Gdańsk, Poland.
Department of Neurology & Stroke, St. Adalbert Hospital, Copernicus Ltd., 80-462 Gdańsk, Poland.
Toxins (Basel). 2025 Jul 29;17(8):375. doi: 10.3390/toxins17080375.
Axial truncal dystonia can present as either flexion or extension, often with a tendency toward lateral movement. Flexion dystonia is more common and may represent a clinical spectrum associated with parkinsonism. In contrast, extensor trunk dystonia is less frequent and exhibits a diverse range of causes. In this paper, we reviewed the literature on axial extensor trunk dystonia. We identified 11 studies involving 49 patients, of which only 10 had idiopathic trunk dystonia. Treatment with botulinum neurotoxin A (BoNT/A) emerged as the most effective therapy; however, many studies did not provide detailed descriptions of the treatment (4/11) and follow-up periods were not specified or short term (up to one-two years). We present four new, well-documented patients with the idiopathic form of extensor trunk dystonia who were treated with BoNT/A with moderate to significant effect according to Global Clinical Impression scale (GCI) and Burke-Fahn-Marsden (BFM) dystonia scale. These cases include long-term follow-up for three patients, all without any adverse events. While the diagnostic process and treatment can be challenging, we recommend using BoNT/A with adjusted doses tailored to the appropriate muscle groups as a first-line treatment.
轴性躯干肌张力障碍可表现为屈曲或伸展，常伴有向侧方运动的倾向。屈曲型肌张力障碍更为常见，可能代表一种与帕金森病相关的临床谱系。相比之下，伸展型躯干肌张力障碍较少见，病因多样。在本文中，我们回顾了关于轴性伸展型躯干肌张力障碍的文献。我们确定了11项研究，涉及49例患者，其中只有10例患有特发性躯干肌张力障碍。肉毒杆菌神经毒素A（BoNT/A）治疗成为最有效的疗法；然而，许多研究没有提供详细的治疗描述（4/11），随访期未明确或为短期（长达一至两年）。我们报告了4例新的、记录良好的特发性伸展型躯干肌张力障碍患者，根据全球临床印象量表（GCI）和伯克-法恩-马斯登（BFM）肌张力障碍量表，他们接受BoNT/A治疗后有中度至显著疗效。这些病例包括对3例患者的长期随访，所有患者均无任何不良事件。虽然诊断过程和治疗具有挑战性，但我们建议将针对适当肌肉群调整剂量的BoNT/A作为一线治疗方法。
