小纤维神经病之间的关系,年龄,Fabry病背景下的性别和疼痛强度仍不清楚。我们的目的是研究小纤维神经病变的相关性,年龄,Fabry患者的性别和疼痛强度。
我们通过记录每位受试者的右手浸入50°C热水浴中时疼痛性热刺激(WLPHS)的退缩潜伏期来评估C-纤维功能,并将该参数与患者的感知疼痛强度和生活质量相关联,通过简短的McGill疼痛问卷(SF-MPQ)在台湾大型Fabry家庭和正常对照中评估。
与正常对照相比,男性Fabry患者的WLPHS明显增加。此外,男性Fabry患者WLPHS增加与患者年龄呈正相关。男性Fabry患者的SF-MPQ随年龄呈钟形分布,在20岁出头和40岁出头之间检测到最大疼痛评分。相比之下,女性Fabry患者的WLPHS和SF-MPQ与年龄有不同的相关性.
我们提出了一种可能的机制,通过这种机制,随着年龄的增长,球形核糖神经酰胺(Gb3)或球形核糖鞘氨醇(lyso-Gb3)逐渐沉积到小神经束中。在年轻的男性法布里患者中引起持续的损伤并产生损伤放电以维持神经性疼痛。然而,一旦小纤维减少到一定程度,他们不再产生足够的有害放电来维持老年男性法布里患者的神经性疼痛,这导致这些患者的SF-MPQ评分较低。相比之下,女性Fabry患者有较少和可变的小纤维损伤,疼痛强度和临床体征/症状。
The relationships among small fiber neuropathy, age, sex and pain intensity in the context of Fabry\'s disease remain unclear. We aim to
study the correlations of small fiber neuropathy, age, sex and pain intensity in Fabry patients.
We evaluated C-fiber function by recording the withdrawal latencies to painful heat stimulus (WLPHS) when each subject\'s right hand was immersed in a 50 °C hot water bath and correlated this parameter with the patient\'s perceived pain intensity and quality of life assessed by the short-form McGill Pain Questionnaire (SF-MPQ) in a large Taiwanese Fabry family and normal controls.
Male Fabry patients showed a significantly increased WLPHS compared to that of normal controls. Furthermore, male Fabry patients showed a positive correlation of increased WLPHS with patient age. The SF-MPQ of male Fabry patients showed a bell distribution with age, and maximal pain scores were detected between the ages of the early 20s and late 40s. In contrast, the female Fabry patients had variable associations of WLPHS and SF-MPQ with age.
We proposed a probable mechanism by which globotriaosylceramide (Gb3) or globotriaosylsphingosine (lyso-Gb3) is gradually deposited into the small nerve bundles with increasing age, which induces continuous damage and produces injury discharges to sustain neuropathic pain in young male Fabry patients. However, once the small fibers are reduced to a certain degree, they no longer produce enough noxious discharges to sustain neuropathic pains in older male Fabry patients, which leads these patients to have lower SF-MPQ scores. In contrast, female Fabry patients had less and variable small fiber damage, pain intensity and clinical signs/symptoms.