Samadzadeh Sara, Brauns Raphaela, Hefter Harald
Department of Neurology, University Hospital of Düsseldorf, Moorenstrasse 5, D-40225 Düsseldorf, Germany.
Toxins (Basel). 2020 Dec 31;13(1):22. doi: 10.3390/toxins13010022.
The response to BoNT is not uniform; a broad spectrum of responses and side-effects usually occurs. This study aimed to show special cervical dystonia cases with therapy response very different to normal treatment course which indicate the extreme ends of therapy spectrum.
Clinical data and course of treatment of five long-term treated patients with cervical dystonia out of therapy response norms are presented: a patient who was supersensitive to standard dose and has required dose adjustment to lower dose of BoNT; one patient who worsened under a standard dose, but responded excellently to twice the standard dose; one insensitive patient who responded poorly for years to a dose well above the standard dose, but responded when dose was further increased; and two patients with a totally different response pattern to BoNT/A preparation 1, but the development of a neutralizing antibody induced secondary treatment failure in both cases and a totally different response after switch to BoNT/A preparation 2.
These five patients indicate that the response of a patient to a BoNT preparation may be unexpected. Therefore, cautious onset of BoNT therapy is recommended as well as consequent dose adjustment later on and even switch to another BoNT/A preparation when a patient has already developed NABs against BoNT/A.
肉毒毒素（BoNT）的反应并不一致；通常会出现广泛的反应和副作用。本研究旨在展示特殊的颈部肌张力障碍病例，其治疗反应与正常治疗过程非常不同，代表了治疗范围的极端情况。
呈现了5例长期接受治疗但治疗反应不符合规范的颈部肌张力障碍患者的临床数据和治疗过程：1例对标准剂量超敏感，需要将BoNT剂量调整至低于标准剂量；1例在标准剂量下病情恶化，但对两倍标准剂量反应良好；1例不敏感患者多年来对远高于标准剂量的剂量反应不佳，但在剂量进一步增加时出现反应；还有2例对BoNT/A制剂1的反应模式完全不同，但均因产生中和抗体导致继发性治疗失败，在换用BoNT/A制剂2后反应完全不同。
这5例患者表明，患者对BoNT制剂的反应可能出乎意料。因此，建议谨慎开始BoNT治疗，并随后进行剂量调整，甚至当患者已经产生针对BoNT/A的中和抗体时，换用另一种BoNT/A制剂。
