Tolosa-Hunt syndrome (THS) is an idiopathic, granulomatous inflammation in the cavernous sinus, supraorbital fissure or orbit. Although the pathophysiology of this idiopathic granulomatous inflammatory process is not clear, it has been suggested that cerebrospinal fluid (CSF) analyses could be helpful in understanding the underlying mechanisms. We report two cases of the 3rd Edition of the International Classification of Headache Disorders (ICHD-3)-classified THS who presented with headache and diplopia. After early treatment with intravenous methylprednisolone, the symptoms of two patients were significantly improved. On outpatient follow-up, neither patient had a relapse. It is worth noting that the isoelectric focusing showed identical CSF and serum oligoclonal IgG bands (OBs). Such bands, “mirror pattern bands”, are one of five standardized patterns (i.e., type 1 = absence of bands in serum and CSF; type 2 = presence of OBs in CSF; type 3 = presence of OBs in CSF and additional identical OBs in both serum and CSF, indicative of intrathecal synthesis; type 4 = presence of identical OBs in both serum and CSF, “mirror pattern”; and type 5 = presence of a monoclonal band in serum and CSF). Previous studies have only found OBs in the CSF of THS (type 4). The finding of “mirror pattern bands” is interesting as it reveals an underlying possible systemic immune activation mechanism in THS.

Tolosa-Hunt 综合征 (THS) 是一种发生在海绵窦、眶上裂或眼眶的特发性肉芽肿性炎症。尽管这种特发性肉芽肿炎症过程的病理生理学尚不清楚，但有人认为脑脊液（CSF）分析可能有助于理解潜在机制。我们报告了两例根据《国际头痛疾病分类》第三版(ICHD-3) 分类的 THS 病例，均表现为头痛和复视。经过早期静脉注射甲泼尼龙治疗后，两名患者的症状明显改善。在门诊随访中，两名患者均未出现复发。值得注意的是，等电聚焦显示相同的脑脊液和血清寡克隆 IgG 带 (OB)。此类条带，即“镜像图案条带”，是五种标准化图案之一（即，类型 1 = 血清和脑脊液中不存在条带；类型 2 = 脑脊液中存在 OB；类型 3 = 脑脊液中存在 OB 和其他相同的 OB）血清和脑脊液中均存在单克隆带，表明鞘内合成；4 型 = 血清和脑脊液中存在相同的 OB，“镜像模式”；5 型 = 血清和脑脊液中存在单克隆带）。之前的研究仅在 THS（4 型）的脑脊液中发现了 OB。“镜像模式带”的发现很有趣，因为它揭示了 THS 中潜在的可能的系统免疫激活机制。