提示Miller Fisher综合征中三叉神经和前庭蜗神经受累的MRI表现

文献信息

中文摘要

背景： Miller Fisher综合征（MFS）是吉兰-巴雷综合征的一种变异型，以眼肌麻痹、共济失调和反射消失三联征为特征，通常与抗GQ1b抗体相关。虽然高分辨率MRI有时可见脑神经强化，但三叉神经（CN V）和前庭蜗神经（CN VIII）受累罕见。方法：我们报告一名62岁女性，表现为复视，起病前有眼痛、鼻塞和眩晕。结果：神经系统评估显示右侧上睑下垂、完全性眼肌麻痹、瞳孔反射迟钝、复视但无眼震、双下肢共济失调、反射减弱以及振动觉和关节位置觉受损。血清学抗GQ1b抗体阳性。增强MRI显示提示CN V和CN VIII受累的信号改变。尽管脑脊液未见蛋白细胞分离，她仍被诊断为MFS。她接受了免疫治疗，包括静脉注射免疫球蛋白治疗。虽然她的眩晕、眼痛和共济失调显著改善，但眼肌麻痹持续存在。患者于住院第44天出院，遗留眼球运动缺陷。结论：在该患者中，伴有颅部自主神经特征的眼眶疼痛（眼痛、鼻塞）和定位不明的眩晕成为误导性的早期体征。本病例说明了一种不寻常的MFS表现，即前驱性CN V和VIII症状先于典型特征出现，强调当感觉和前庭症状先于典型特征出现时需要仔细的临床评估。

英文摘要

Miller Fisher syndrome (MFS) is a variant of Guillain-Barré syndrome characterized by a triad of ophthalmoplegia, ataxia, and areflexia, typically associated with anti-GQ1b antibodies. While cranial nerve enhancement is sometimes seen on high-resolution MRI, involvement of the trigeminal (CN V) and vestibulocochlear (CN VIII) nerves is rare. We report a 62-year-old woman presenting with diplopia, preceded by ophthalmalgia, nasal congestion, and vertigo. Neurological assessment revealed right-sided ptosis, total ophthalmoplegia, sluggish pupillary reflexes, diplopia without nystagmus, bilateral limb ataxia, hyporeflexia, and impaired vibration and joint-position sensation. Serology was positive for anti-GQ1b antibodies. Contrast-enhanced MRI demonstrated signal changes suggestive of CN V and CN VIII involvement. Although cerebrospinal fluid showed no albuminocytologic dissociation, she was diagnosed with MFS. She received immunotherapy, including intravenous immunoglobulin therapy. While her vertigo, ophthalmalgia and ataxia significantly improved, ophthalmoplegia persisted. The patient was discharged on hospitalization day 44 with residual ocular motility deficits. In this patient, orbital pain with cranial autonomic features (ocular pain, nasal congestion) and vertigo of uncertain localization acted as misleading early signs. This case illustrates an unusual MFS presentation where prodromal CN V and VIII symptoms preceded classic features, highlighting the need for careful clinical evaluation when sensory and vestibular symptoms precede classic features.