j - JOUR 1 -急性营养性轴索神经病患者的临床谱与预后[j] -神经科-神经科-神经科-神经科-神经科-神经内科- 10.1212/WNL.0000000000207215首页Sp - 10.1212/ wnl.0000000000207215AU - Johanna I Hamel AU - Eric L. Logigian Y1 - 2023/03/27 UR - http://n.首页neurology.org/content/early/2023/03/24/WNL.0000000000207215.abstract背景与目的:探讨急性营养性轴索神经病(ANAN)患者的临床、微量营养素、电生理谱及预后。方法:通过回顾性回顾我们的肌电图数据库和电子健康记录，在1999年至2020年期间确定ANAN患者，并根据临床和电诊断依据分为纯感觉、感觉运动或纯运动;以及风险因素(酒精使用障碍、减肥手术或厌食症)。实验室异常记录包括硫胺素、维生素B6、B12和E、叶酸和铜。最后随访时记录患者的动态和神经性疼痛状态。结果:在40例ANAN患者中，21例有酒精使用障碍，10例厌食症，9例最近接受了减肥手术。纯感觉神经病变14例(低硫胺素7例)，感觉运动神经病变23例(低硫胺素8例)，纯运动神经病变3例(低硫胺素1例)。维生素B1含量最低(85%)，其次是维生素B6(77%)和叶酸(50%)。危险因素和神经病变类型与特定微量营养素缺乏无关。 Of the 37 patients who were seen in follow-up, only 13 (35%) were walking independently, and only 8 (22%) were pain free at the last follow-up visit at a mean of 22 months (range: 2-88 months) from onset.Discussion: The spectrum of ANAN is wide ranging from: 1) a pure sensory neuropathy with areflexia, limb and gait ataxia, neuropathic pain and unevocable sensory responses to 2) a motor axonal neuropathy with low-amplitude motor responses without conduction slowing, block, or dispersion, and 3) a mixed sensorimotor axonal polyneuropathy. Specific micronutrient deficiencies or risk factors do not predict neuropathy subtype. The sub-group of ANAN patients with documented thiamine deficiency also range from pure sensory to pure motor, and only a minority have Wernicke’s encephalopathy. We do not know whether co-existent micronutrient deficiencies may help explain the wide clinical spectrum of thiamine-deficient ANAN. The prognosis of ANAN is guarded due to residual neuropathic pain and slow recovery of independent ambulation. Therefore, early recognition of patients at risk is important. ER -