Ahn Soo Hyun, Ahn Seon-Jae, Kim Seung Ae, Lee Han Sang, Chu Kon
Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
Laboratory for Neurotherapeutics, Center for Medical Innovation, Biomedical Research Institute, Seoul National University Hospital, Seoul, Korea.
Encephalitis. 2024 Apr;4(2):40-46. doi: 10.47936/encephalitis.2024.00010. Epub 2024 Mar 28.
Eosinophilic meningoencephalitis is a rare inflammatory condition of the central nervous system. As a limited number of cases has been reported, debate remains on the optimal treatment. We present a case of idiopathic eosinophilic meningoencephalitis successfully treated with glucocorticoids and intravenous immunoglobulin (IVIG). After extensive evaluation to rule out other possible causes, the patient was treated with intravenous (IV) dexamethasone and showed significant improvement within a few days. However, neurologic impairment persisted, and follow-up lumbar puncture results showed only a mild decrease in pleocytosis. Even after an additional 5 days of IV methylprednisolone, cerebrospinal fluid (CSF) pleocytosis persisted, and brain magnetic resonance imaging (MRI) showed an increase in enhanced lesions, implying persistent neuroinflammation. The patient was maintained on high-dose oral prednisolone for 2 months, and additional immune-modulatory effects were treated with IVIG. Follow-up MRI at 2 months showed a significant decrease in the extent of multiple enhanced lesions and a normalized CSF profile. The patient was maintained on regular maintenance doses of IVIG for an additional 6 months without any neurologic signs or symptoms. Inflammation is the key pathophysiology underlying neurological damage in eosinophilic meningoencephalitis. A literature review revealed that corticosteroid treatment is the only anti-inflammatory treatment used in cases of idiopathic meningoencephalitis, resulting in sufficient response in most patients but only partial response or death in a few cases. This is the first case report of IVIG use in idiopathic eosinophilic meningoencephalitis, suggesting the possibility of a new treatment modality for refractory cases.
嗜酸性粒细胞性脑膜脑炎是一种罕见的中枢神经系统炎症性疾病。由于报告的病例数量有限，关于最佳治疗方案仍存在争议。我们报告一例特发性嗜酸性粒细胞性脑膜脑炎患者，经糖皮质激素和静脉注射免疫球蛋白（IVIG）成功治疗。在进行广泛评估以排除其他可能病因后，患者接受静脉注射地塞米松治疗，并在数天内显示出明显改善。然而，神经功能障碍仍然存在，后续腰椎穿刺结果显示仅细胞数轻度减少。即使在额外静脉注射甲泼尼龙5天后，脑脊液（CSF）细胞数仍持续存在，脑部磁共振成像（MRI）显示强化病灶增加，提示神经炎症持续存在。患者接受高剂量口服泼尼松龙治疗2个月，并使用IVIG进行额外的免疫调节治疗。2个月后的随访MRI显示多个强化病灶范围显著减小，CSF指标恢复正常。患者继续规律维持剂量的IVIG治疗6个月，未出现任何神经体征或症状。炎症是嗜酸性粒细胞性脑膜脑炎神经损伤的关键病理生理学机制。文献综述显示，皮质类固醇治疗是特发性脑膜脑炎唯一使用的抗炎治疗方法，大多数患者对此有充分反应，但少数患者仅有部分反应或死亡。这是首例关于IVIG用于特发性嗜酸性粒细胞性脑膜脑炎的病例报告，提示难治性病例可能有新的治疗方式。
