The relation between small nerve fibre function, age, disease severity and pain in Fabry disease

Abstract

Background Small fibre neuropathy supposedly causes pain in Fabry patients, but the relationship between small nerve fibre function and pain severity is unclear. Methods A cohort of 15 male and 33 female Fabry patients was studied making use of a quantitative sensory testing protocol, disease severity measures and pain scales to investigate the relationship between nerve fibre function, age, disease severity and pain intensity. Results Male Fabry patients exhibited an abnormal cold detection threshold and thermal sensory limen at the upper and lower limb, indicating Aδ-fibre hypofunction. Female patients showed Z-scores within normal range for all modalities. Nerve fibre function was worse at older age and with more severe disease. The overall severity of pain was mild, without significant differences between males and females. No linear relationship between pain severity and small nerve fibre function was identified. Conclusions In Fabry disease, no linear relationship exists between pain and small nerve fibre function. With older age and more severe disease pain may abate as nerve fibre function further deteriorates.