Optic, trigeminal, and facial neuropathy related to anti-neurofascin 155 antibody.

Optic, trigeminal, and facial neuropathy related to anti-neurofascin 155 antibody.
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DOI:
10.1002/acn3.51220
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发表时间:
2020-11
影响因子:
5.3
通讯作者:
Kira JI
Kira JI
中科院分区:
医学2区
文献类型:
--
作者:
Ogata H;Zhang X;Inamizu S;Yamashita KI;Yamasaki R;Matsushita T;Isobe N;Hiwatashi A;Tobimatsu S;Kira JI

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通过神经影像学和电生理学表征IgG 4抗神经成束蛋白155抗体阳性(NF 155+)慢性炎性脱髓鞘性多发性神经病(CIDP)中视神经、三叉神经和面神经受累的频率和模式。13例平均发病年龄为34岁的IgG 4 NF 155 + CIDP患者(11例男性)接受了神经系统检查、眨眼反射和视觉诱发电位(VEP)测试,以及轴向和/或冠状位T2加权头部磁共振成像(MRI)。在13名患者中,分别有3名(23.1%)、2名(15.4%)和2名(15.4%)患者观察到面部感觉障碍、面部无力和明显视力障碍。所有12例受试者均存在眨眼反射异常:11例(91.7%)R1缺失和/或延迟,10例(83.3%)R2缺失和/或延迟。R1潜伏期与血清抗NF 155抗体水平(双侧r = 0.9,P ≤ 0.0001)以及正中神经和尺神经的远端和F波潜伏期呈强正相关。在10/13例(76.9%)患者和17/26例(65.4%)眼中观察到VEP缺失和/或延长。在MRI上,分别在9/13(69.2%)和10/13(76.9%)例患者中检测到三叉神经肥大和高信号强度,而所有患者的视神经均正常。冠状面上眶内三叉神经宽度与病程呈显著正相关。亚临床脱髓鞘经常发生在IgG 4 NF 155 + CIDP的视神经、三叉神经和面神经中,这表明颅神经的中枢和外周髓鞘结构都参与了这种情况,而神经肥大仅发生在有髓鞘的外周神经纤维中。
To characterize the frequency and patterns of optic, trigeminal, and facial nerve involvement by neuroimaging and electrophysiology in IgG4 anti‐neurofascin 155 antibody‐positive (NF155+) chronic inflammatory demyelinating polyneuropathy (CIDP). Thirteen IgG4 NF155+ CIDP patients with mean onset age of 34 years (11 men) were subjected to neurological examination, blink reflex, and visual‐evoked potential (VEP) testing, and axial and/or coronal T2‐weighted head magnetic resonance imaging (MRI). Among 13 patients, facial sensory impairment, facial weakness, and apparent visual impairment were observed in three (23.1%), two (15.4%), and two (15.4%) patients, respectively. All 12 patients tested had blink reflex abnormalities: absent and/or delayed R1 in 11 (91.7%), and absent and/or delayed R2 in 10 (83.3%). R1 latencies had strong positive correlations with serum anti‐NF155 antibody levels (r = 0.9, P ≤ 0.0001 on both sides) and distal and F wave latencies of the median and ulnar nerves. Absent and/or prolonged VEPs were observed in 10/13 (76.9%) patients and 17/26 (65.4%) eyes. On MRI, hypertrophy, and high signal intensity of trigeminal nerves were detected in 9/13 (69.2%) and 10/13 (76.9%) patients, respectively, whereas optic nerves were normal in all patients. The intra‐orbital trigeminal nerve width on coronal sections showed a significant positive correlation with disease duration. Subclinical demyelination frequently occurs in the optic, trigeminal, and facial nerves in IgG4 NF155+ CIDP, suggesting that both central and peripheral myelin structures of the cranial nerves are involved in this condition, whereas nerve hypertrophy only develops in myelinated peripheral nerve fibers.
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