Manfredi M, Bini G, Cruccu G, Accornero N, Berardelli A, Medolago L
Arch Neurol. 1981 Aug;38(8):507-11. doi: 10.1001/archneur.1981.00510080069010.
A 16-year-old boy had congenital absence of pain sensitivity and no impairment of other sensory modalities. Routine electrophysiologic investigation showed no abnormalities. The threshold and latency of electrically elicited corneal reflex and cortical potentials evoked by tooth pulp stimulation were normal, but suprathreshold electric stimulation of corneal mucosa and dental pulp, as well as electric stimulation of dorsal roots, did not elicit pain. The total CSF opioid activity was raised. However, naloxone hydrochloride administration failed to reverse the analgesia. The axon reflex to intradermal injection of histamine dihydrochloride was absent. Cutaneous nerve branches showed unspecific changes affecting part of unmyelinated axons. most of the unmyelinated as well as the myelinated axons were normal. We consider the case an example of congenital indifference to pain.
一名16岁男孩先天性无痛觉，其他感觉方式无损害。常规电生理检查未显示异常。电诱发角膜反射及牙髓刺激诱发的皮质电位的阈值和潜伏期正常，但角膜黏膜和牙髓的阈上电刺激以及背根电刺激均未引发疼痛。脑脊液中阿片类物质的总活性升高。然而，注射盐酸纳洛酮未能逆转镇痛效果。皮内注射二盐酸组胺的轴突反射缺失。皮神经分支显示非特异性改变，影响部分无髓鞘轴突。大多数无髓鞘以及有髓鞘轴突正常。我们认为该病例是先天性无痛觉的一个实例。
