Brandt B R, Rosén I
Center of Child and Youth Habilitation, Lund, Sweden.
Neuropediatrics. 1998 Jun;29(3):124-6. doi: 10.1055/s-2007-973547.
The Prader-Willi syndrome (PWS) is associated with a tendency to self-injury and a reduced sensitivity to painful stimuli. Somatosensory functions were studied in 5 children aged 11-13 years with PWS. Tactual perception in the hands (stereognosis) was apparently normal in 4 of them. Sensory nerve conduction velocities in the median nerve and latencies for sensory evoked potentials were similar in the PWS subjects and in 10 healthy controls indicating a preserved myelinisation of sensory nerve fibers in PWS. Sensory nerve action potential amplitudes in the PWS group were on an average only 40-50% of normal size (p = 0.03), suggesting a reduced number of normal axons in the median nerve. The results may be relevant for the impaired pain sensitivity in PWS because similar neurographic findings and a low density of peripheral nerve fibers have been reported in patients with hereditary or congenital insensitivity to pain.
普拉德-威利综合征（PWS）与自伤倾向及对疼痛刺激的敏感性降低有关。对5名年龄在11至13岁的PWS患儿进行了躯体感觉功能研究。其中4名患儿手部的触觉感知（实体觉）明显正常。PWS患儿与10名健康对照者的正中神经感觉神经传导速度及感觉诱发电位潜伏期相似，表明PWS患儿感觉神经纤维的髓鞘化保存完好。PWS组感觉神经动作电位幅度平均仅为正常大小的40%至50%（p = 0.03），提示正中神经中正常轴突数量减少。这些结果可能与PWS患儿疼痛敏感性受损有关，因为在遗传性或先天性痛觉缺失患者中也报告了类似的神经电生理 findings 及外周神经纤维密度降低的情况。
