Schuller Y, Linthorst G E, Hollak C E M, Van Schaik I N, Biegstraaten M
Department of Internal Medicine, Division Endocrinology and Metabolism, Academic Medical Centre, Room F5-166, Meibergdreef 9, Amsterdam, 1105 AZ, The Netherlands.
BMC Neurol. 2016 Feb 24;16:25. doi: 10.1186/s12883-016-0549-8.
Neuropathic pain is one of the key features of (classical) Fabry disease (FD). No randomized clinical trials comparing effectiveness of different pain management strategies have been performed. This review aims to give an overview of existing pain management strategies.
PubMed and Embase were searched up to September 2014 for relevant articles on treatment of neuropathic pain in FD.
Seven-hundred-thirty-one articles were identified of which 26 were included in the analysis. Studies reported on 55 individuals in total, with group-sizes ranging from 1 to 8. Carbamazepine appeared most beneficial: complete pain relief in 5/25, partial relief in 17/25, and no benefit in 3/25 patients. Phenytoin resulted in complete relief in 1/27, partial relief in 12/27 and no benefit in 6/27 patients. In 8 patients a significant reduction in the frequency of pain attacks was described. Gabapentin caused partial relief in 6/7 and no relief in 1/7 patients. Little evidence was reported for SSNRI's or treatment combinations. Adverse-effects were reported in all treatment strategies.
Only for carbamazepine, phenytoin and gabapentin there is evidence of effectiveness in neuropathic pain due to FD, but comparison of effectiveness between these drugs is lacking. In routine clinical practice adverse-effects may discourage use of carbamazepine and phenytoin in favor of second-generation antiepileptic drugs, but this is currently not supported by clinical evidence. This review suffers greatly from incomplete outcome reports and a predominance of case reports, which emphasizes the need for robust clinical trials and observational cohort studies.
神经性疼痛是（典型）法布里病（FD）的关键特征之一。尚未进行比较不同疼痛管理策略有效性的随机临床试验。本综述旨在概述现有的疼痛管理策略。
检索截至2014年9月的PubMed和Embase，以查找有关FD神经性疼痛治疗的相关文章。
共识别出731篇文章，其中26篇纳入分析。研究总共报道了55例个体，每组人数从1至8不等。卡马西平似乎最有益：25例中有5例疼痛完全缓解，17例部分缓解，3例无改善。苯妥英钠使27例中的1例完全缓解，12例部分缓解，6例无改善。8例患者的疼痛发作频率显著降低。加巴喷丁使7例中的6例部分缓解，1例无缓解。关于5-羟色胺再摄取抑制剂（SSNRI）或联合治疗的证据很少。所有治疗策略均报告有不良反应。
仅有证据表明卡马西平、苯妥英钠和加巴喷丁对FD所致神经性疼痛有效，但缺乏这些药物之间有效性的比较。在常规临床实践中，不良反应可能会使人们不愿使用卡马西平或苯妥英钠而倾向于第二代抗癫痫药物，但目前这一点尚无临床证据支持。本综述因结果报告不完整和病例报告占主导而受到很大影响，这凸显了开展有力的临床试验和观察性队列研究的必要性。
