title: Pain management strategies for neuropathic pain in Fabry disease--a systematic review.
abstract: BACKGROUND
  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.
  
  
  METHODS
  PubMed and Embase were searched up to September 2014 for relevant articles on treatment of neuropathic pain in FD.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Internal Medicine, Division Endocrinology and Metabolism, Academic Medical Centre, Room F5-166, Meibergdreef 9, Amsterdam, 1105 AZ, The Netherlands. y.schuller@amc.nl.
authors.forename: Y
authors.initials: Y
authors.lastname: Schuller
authors.affiliation: Department of Internal Medicine, Division Endocrinology and Metabolism, Academic Medical Centre, Room F5-166, Meibergdreef 9, Amsterdam, 1105 AZ, The Netherlands. g.e.linthorst@amc.nl.
authors.forename: G E
authors.initials: GE
authors.lastname: Linthorst
authors.affiliation: Department of Internal Medicine, Division Endocrinology and Metabolism, Academic Medical Centre, Room F5-166, Meibergdreef 9, Amsterdam, 1105 AZ, The Netherlands. c.e.hollak@amc.nl.
authors.forename: C E M
authors.initials: CE
authors.lastname: Hollak
authors.affiliation: Department of Internal Medicine, Division Endocrinology and Metabolism, Academic Medical Centre, Room F5-166, Meibergdreef 9, Amsterdam, 1105 AZ, The Netherlands. i.n.vanschaik@amc.nl.
authors.forename: I N
authors.initials: IN
authors.lastname: Van Schaik
authors.affiliation: Department of Internal Medicine, Division Endocrinology and Metabolism, Academic Medical Centre, Room F5-166, Meibergdreef 9, Amsterdam, 1105 AZ, The Netherlands. m.biegstraaten@amc.nl.
authors.forename: M
authors.initials: M
authors.lastname: Biegstraaten
mesh_terms: D000588:Amines / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use*; D002220:Carbamazepine / Q000627:therapeutic use; D003509:Cyclohexanecarboxylic Acids / Q000627:therapeutic use; D000795:Fabry Disease / Q000188:drug therapy*; D000077206:Gabapentin; D006801:Humans; D009437:Neuralgia / Q000188:drug therapy* / Q000209:etiology; D010672:Phenytoin / Q000627:therapeutic use; D005680:gamma-Aminobutyric Acid / Q000627:therapeutic use
chemical_list: D000588:Amines; D000927:Anticonvulsants; D003509:Cyclohexanecarboxylic Acids; D002220:Carbamazepine; D005680:gamma-Aminobutyric Acid; D010672:Phenytoin; D000077206:Gabapentin