Management of Neuropathic Pain in Fabry Disease

Abstract

Aims: The goal was to investigate the diagnostic yield of Lanss and DN4 scores which have not been used in Fabry Disease until now, to our knowledge. Material and Methods: Eighteen patients aged between 8 and 51 years were enrolled in this study. The diagnosis of Fabry was confirmed by mutation analysis. We also kept track of these tests in the followup. Besides, Beck Depression scale, Pittsburgh Sleep Quality Index, Fatigue Severity Scale and both physical and mental components of Short Form Health Survey were performed. Results: The pain scores before and after each and every two months of enzyme replacement therapies (the patients received enzyme replacement therapies every two weeks) regressed during ten months of therapy and this regression was statistically significant (p=0.001). Similar results were also obtained for depression, sleep quality, fatigue reduction and improvement in health survey scores (p<0.001). Conclusion: Lanss and DN4 scores seem to be reliable both in diagnosis and monitoring of neuropathic pain in Fabry disease. Another important point that this study yields is the therapeutic impact of enzyme replacement therapies on pain scores. The pain and life quality scores before and after enzyme replacement therapies show that, this therapy is effective in neuropathic pain of Fabry disease.