严重程度/频率:来自随机对照试验的优质证据表明,两种不同的 A 型肉毒杆菌毒素制剂(Xeomin 和保妥适)皆可显著提高 Jankovic 评定量表得分,使其远高于基线。[54]Jankovic J. Clinical efficacy and tolerability of Xeomin in the treatment of blepharospasm. Eur J Neurol. 2009 Dec;16 Suppl 2:14-8.http://www.ncbi.nlm.nih.gov/pubmed/20002742?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
严重程度/频率:来自随机对照试验的优质证据表明,两种不同的 A 型肉毒杆菌毒素制剂(Xeomin 和保妥适)皆可显著提高 Jankovic 评定量表得分,使其远高于基线。[54]Jankovic J. Clinical efficacy and tolerability of Xeomin in the treatment of blepharospasm. Eur J Neurol. 2009 Dec;16 Suppl 2:14-8.http://www.ncbi.nlm.nih.gov/pubmed/20002742?tool=bestpractice.com
症状缓解:来自 RCT 的中等质量证据表明,相比于安慰剂对照组, 苯海索可以显著缓解肌张力障碍症状。[41]Burke RE, Fahn S, Marsden CD. Torsion dystonia: a double-blind, prospective trial of high-dosage trihexyphenidyl. Neurology. 1986 Feb;36(2):160-4.http://www.ncbi.nlm.nih.gov/pubmed/3511401?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
症状缓解:来自 RCT 的中等质量证据表明,相比于安慰剂对照组, 苯海索可以显著缓解肌张力障碍症状。[41]Burke RE, Fahn S, Marsden CD. Torsion dystonia: a double-blind, prospective trial of high-dosage trihexyphenidyl. Neurology. 1986 Feb;36(2):160-4.http://www.ncbi.nlm.nih.gov/pubmed/3511401?tool=bestpractice.com
症状缓解:来自对照研究的中等质量证据表明,脑深部电刺激可以有效提高 Burke Fahn Marsden 肌张力障碍评定量表得分、SF-36 和 TWSTRS(西多伦多医院痉挛性斜颈评定量表)的严重性、残疾和疼痛分项分数。[57]Vidailhet M, Vercueil L, Houeto JL, et al. Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med. 2005 Feb 3;352(5):459-67.https://www.nejm.org/doi/full/10.1056/NEJMoa042187http://www.ncbi.nlm.nih.gov/pubmed/15689584?tool=bestpractice.com[58]Kupsch A, Benecke R, Müller J, et al. Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med. 2006 Nov 9;355(19):1978-90.https://www.nejm.org/doi/full/10.1056/NEJMoa063618http://www.ncbi.nlm.nih.gov/pubmed/17093249?tool=bestpractice.com[59]Kiss ZH, Doig-Beyaert K, Eliasziw M, et al. The Canadian multicentre study of deep brain stimulation for cervical dystonia. Brain. 2007 Nov;130(Pt 11):2879-86.https://academic.oup.com/brain/article/130/11/2879/330659http://www.ncbi.nlm.nih.gov/pubmed/17905796?tool=bestpractice.com[60]Mueller J, Skogseid IM, Benecke R, et al. Pallidal deep brain stimulation improves quality of life in segmental and generalized dystonia: results from a prospective, randomized sham-controlled trial. Mov Disord. 2008 Jan;23(1):131-4.http://www.ncbi.nlm.nih.gov/pubmed/17973330?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
症状缓解:来自对照研究的中等质量证据表明,脑深部电刺激可以有效提高 Burke Fahn Marsden 肌张力障碍评定量表得分、SF-36 和 TWSTRS(西多伦多医院痉挛性斜颈评定量表)的严重性、残疾和疼痛分项分数。[57]Vidailhet M, Vercueil L, Houeto JL, et al. Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med. 2005 Feb 3;352(5):459-67.https://www.nejm.org/doi/full/10.1056/NEJMoa042187http://www.ncbi.nlm.nih.gov/pubmed/15689584?tool=bestpractice.com[58]Kupsch A, Benecke R, Müller J, et al. Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med. 2006 Nov 9;355(19):1978-90.https://www.nejm.org/doi/full/10.1056/NEJMoa063618http://www.ncbi.nlm.nih.gov/pubmed/17093249?tool=bestpractice.com[59]Kiss ZH, Doig-Beyaert K, Eliasziw M, et al. The Canadian multicentre study of deep brain stimulation for cervical dystonia. Brain. 2007 Nov;130(Pt 11):2879-86.https://academic.oup.com/brain/article/130/11/2879/330659http://www.ncbi.nlm.nih.gov/pubmed/17905796?tool=bestpractice.com[60]Mueller J, Skogseid IM, Benecke R, et al. Pallidal deep brain stimulation improves quality of life in segmental and generalized dystonia: results from a prospective, randomized sham-controlled trial. Mov Disord. 2008 Jan;23(1):131-4.http://www.ncbi.nlm.nih.gov/pubmed/17973330?tool=bestpractice.com
症状缓解:来自优质观察性定期研究的质量不佳的证据表明,多巴反应性肌张力障碍患者在接受左旋多巴治疗后,症状有所缓解。[38]Nygaard TG, Marsden CD, Fahn S. Dopa-responsive dystonia: long-term treatment response and prognosis. Neurology. 1991 Feb;41(2 ( Pt 1)):174-81.http://www.ncbi.nlm.nih.gov/pubmed/1899474?tool=bestpractice.com[40]Bandmann O, Nygaard TG, Surtees R, et al. Dopa-responsive dystonia in British patients: new mutations of the GTP-cyclohydrolase I gene and evidence for genetic heterogeneity. Hum Mol Genet. 1996 Mar;5(3):403-6.https://academic.oup.com/hmg/article/5/3/403/601854http://www.ncbi.nlm.nih.gov/pubmed/8852666?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
症状缓解:来自优质观察性定期研究的质量不佳的证据表明,多巴反应性肌张力障碍患者在接受左旋多巴治疗后,症状有所缓解。[38]Nygaard TG, Marsden CD, Fahn S. Dopa-responsive dystonia: long-term treatment response and prognosis. Neurology. 1991 Feb;41(2 ( Pt 1)):174-81.http://www.ncbi.nlm.nih.gov/pubmed/1899474?tool=bestpractice.com[40]Bandmann O, Nygaard TG, Surtees R, et al. Dopa-responsive dystonia in British patients: new mutations of the GTP-cyclohydrolase I gene and evidence for genetic heterogeneity. Hum Mol Genet. 1996 Mar;5(3):403-6.https://academic.oup.com/hmg/article/5/3/403/601854http://www.ncbi.nlm.nih.gov/pubmed/8852666?tool=bestpractice.com
症状缓解:来自若干病例分析和回顾性研究的质量不佳的证据表明,口服巴氯芬治疗可以缓解肌张力障碍症状,特别是步态有明显改善。[43]Greene P. Baclofen in the treatment of dystonia. Clin Neuropharmacol. 1992 Aug;15(4):276-88.http://www.ncbi.nlm.nih.gov/pubmed/1516073?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
症状缓解:来自若干病例分析和回顾性研究的质量不佳的证据表明,口服巴氯芬治疗可以缓解肌张力障碍症状,特别是步态有明显改善。[43]Greene P. Baclofen in the treatment of dystonia. Clin Neuropharmacol. 1992 Aug;15(4):276-88.http://www.ncbi.nlm.nih.gov/pubmed/1516073?tool=bestpractice.com
痉挛症状缓解:来自若干非对照病例分析研究和病例报告的质量不佳的证据表明,鞘内注射巴氯芬治疗可以缓解痉挛症状,并提高 Burke Fahn Marsden 肌张力障碍评定量表得分。[45]Butler C, Campbell S. Evidence of the effects of intrathecal baclofen for spastic and dystonic cerebral palsy. AACPDM Treatment Outcomes Committee Review Panel. Dev Med Child Neurol. 2000 Sep;42(9):634-45.https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1469-8749.2000.tb00371.xhttp://www.ncbi.nlm.nih.gov/pubmed/11034458?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
痉挛症状缓解:来自若干非对照病例分析研究和病例报告的质量不佳的证据表明,鞘内注射巴氯芬治疗可以缓解痉挛症状,并提高 Burke Fahn Marsden 肌张力障碍评定量表得分。[45]Butler C, Campbell S. Evidence of the effects of intrathecal baclofen for spastic and dystonic cerebral palsy. AACPDM Treatment Outcomes Committee Review Panel. Dev Med Child Neurol. 2000 Sep;42(9):634-45.https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1469-8749.2000.tb00371.xhttp://www.ncbi.nlm.nih.gov/pubmed/11034458?tool=bestpractice.com
症状缓解:来自随机对照临床试验的低质量证据表明,A 型肉毒杆菌毒素和 B 型肉毒杆菌毒素在降低 TWSTRS(西多伦多医院痉挛性斜颈评定量表)得分方面的效力相同。[55]Pappert EJ, Germanson T; Myobloc/Neurobloc European Cervical Dystonia Study Group. Botulinum toxin type B vs. type A in toxin-naïve patients with cervical dystonia: randomized, double-blind, noninferiority trial. Mov Disord. 2008 Mar 15;23(4):510-7.http://www.ncbi.nlm.nih.gov/pubmed/18098274?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
症状缓解:来自随机对照临床试验的低质量证据表明,A 型肉毒杆菌毒素和 B 型肉毒杆菌毒素在降低 TWSTRS(西多伦多医院痉挛性斜颈评定量表)得分方面的效力相同。[55]Pappert EJ, Germanson T; Myobloc/Neurobloc European Cervical Dystonia Study Group. Botulinum toxin type B vs. type A in toxin-naïve patients with cervical dystonia: randomized, double-blind, noninferiority trial. Mov Disord. 2008 Mar 15;23(4):510-7.http://www.ncbi.nlm.nih.gov/pubmed/18098274?tool=bestpractice.com