以痛性三叉神经病为首发表现的AQP4-IgG阴性的视神经脊髓炎谱系疾病一例并文献复习

A case of AQP4-IgG-negative neuromyelitis optica spectrum disorders with initial clinical manifestation of painful trigeminal neuropathy and literature review

目的探讨以痛性三叉神经病为首发表现的水通道蛋白-4抗体(AQP4-IgG)阴性的视神经脊髓炎谱系疾病(NMOSD)的临床、影像特征。方法对西南医科大学附属医院神经内科1例AQP4-IgG阴性的NMOSD患者症状、体征、影像学检查及诊治过程进行报道,并结合文献进行讨论。结果该患者第一次发作表现为右侧面部疼痛起病,伴左侧肢体节段性感觉异常,第二次发作表现为顽固性呃逆、呕吐起病,伴左侧肢体乏力及感觉异常。头颅及脊髓核磁共振平扫提示病灶累及延髓、颈髓,两次血清、脑脊液AQP4-IgG、髓鞘少突胶质细胞糖蛋白(MOG)抗体、髓鞘碱性蛋白(MBP)抗体、寡克隆区带(OCB)均呈阴性,诊断为AQP4-IgG阴性的NMOSD。经激素抗炎、止痛等治疗后,患者面部疼痛、感觉异常、呃逆、呕吐、肢体乏力明显好转。结论痛性三叉神经病可能是AQP4-IgG阴性的NMOSD的首发临床表现,仅表现为三叉神经分布区疼痛的NMOSD易误诊、漏诊,核磁共振检查有助于早期诊断。

Objective To explore the clinical manifestations and imaging of neuromyelitis optica spectrum disorders(NMOSD)with negative aquaporin-4 immunoglobulin G antibody(AQP4-IgG)with initial clinical manifestation of painful trigeminal neuropathy.Methods The symptoms,signs,imaging examinations,diagnosis and treatment of a case of AQP4-IgG-negative NMOSD in neurology department of Affiliated Hospital of Southwest Medical University were reported and the relevant literatures were reviewed.Results In the first episode,the patient started with right-sided facial pain with segmental paresthesia in the left limb.And In the second episode,the patient started with intractable hiccups and vomiting with weakness and paresthesia in the left limb.Magnetic resonance imaging of the skull and spinal cord revealed that the lesion involved the medulla oblongata and cervical medulla.AQP4 antibody,myelin oligodendrocytes glycoprotein(MOG)antibody,myelin basic protein(MBP)antibody and oligoclonal bands(OCB)of serum and cerebrospinal fluid were negative.The clinical diagnosis was AQP4-IgG-negative NMOSD.After treatments with hormonal anti-inflammatory and analgesic therapy,the patient′s facial pain,sensory abnormalities,hiccups,vomiting and limb weakness improved significantly.Conclusions Painful trigeminal neuropathy may be the initial clinical manifestation of AQP4-IgG-negative NMOSD.NMOSD of presenting solely with painful trigeminal neuropathy is easy to be misdiagnosed and missed.Magnetic resonance examination is helpful for the early diagnosis of NMOSD.