Department of Neurology, Loma Linda University Medical Center, Loma Linda, CA.
J Clin Neuromuscul Dis. 2023 Dec 1;25(2):59-62. doi: 10.1097/CND.0000000000000464.
Anti-neurofascin-155 IgG4 (NF-155) antibody disease has previously been associated with a subset of patients with chronic inflammatory demyelinating polyradiculoneuropathy. We report a case of NF-155 positive polyneuropathy that initially presented as an acute inflammatory demyelinating polyradiculoneuropathy. The patient responded appropriately to treatment but subsequently progressed over a 3-month period, resulting in quadriplegia, areflexia, and oculobulbar paralysis.
Case report and literature review.
A 40-year-old male presented with acute bilateral arm and thigh weakness, areflexia, and distal sensory loss. Treatment with intravenous immunoglobulin (IVIg) for acute acquired demyelinating neuropathy resulted in initial improvement but subsequent decline. Lack of response to additional IVIg and plasmapheresis (PLEX) prompted testing for NF-155. Treatment with rituximab and steroids resulted in virtually complete recovery.
Early testing for nodal and paranodal proteins is indicated in patients who present with acute acquired demyelinating neuropathy but fail to respond to conventional treatments, such as IVIg or PLEX. Identification of nodal and paranodal antibodies should prompt treatment with rituximab and steroids to increase likelihood of recovery.
抗神经束蛋白-155 IgG4（NF-155）抗体病以前与慢性炎症性脱髓鞘性多发性神经病的一部分患者有关。我们报告了一例 NF-155 阳性多发性神经病，最初表现为急性炎症性脱髓鞘性多发性神经病。该患者对治疗有适当反应，但随后在 3 个月内进展，导致四肢瘫痪、反射消失和眼肌瘫痪。
病例报告和文献复习。
一名 40 岁男性出现急性双侧手臂和大腿无力、反射消失和远端感觉丧失。急性获得性脱髓鞘性神经病的静脉注射免疫球蛋白（IVIg）治疗最初有改善，但随后病情恶化。对额外的 IVIg 和血浆置换（PLEX）没有反应促使检测 NF-155。利妥昔单抗和类固醇治疗导致几乎完全恢复。
对于出现急性获得性脱髓鞘性神经病但对 IVIg 或 PLEX 等常规治疗无反应的患者，应早期检测神经结和神经旁蛋白。识别神经结和神经旁抗体应提示使用利妥昔单抗和类固醇进行治疗，以提高恢复的可能性。
