头痛、眼肌麻痹、"镜像模式带":两个病例报告

Jianhang He, Yazhou Ren, Weimin Qi, Juan Yang, Haining Li
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摘要

托洛萨-亨特综合征(Tolosa-Hunt Syndrome,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。虽然这种特发性肉芽肿性炎症过程的病理生理学尚不清楚,但有人认为脑脊液(CSF)分析有助于了解其潜在机制。我们报告了两例国际头痛疾病分类(ICHD-3)第 3 版分类的 THS 患者,他们表现为头痛和复视。在早期接受甲基强的松龙静脉注射治疗后,两名患者的症状得到了明显改善。在门诊随访中,两名患者均未复发。值得注意的是,等电聚焦显示出相同的脑脊液和血清寡克隆 IgG 带(OBs)。这种条带被称为 "镜像模式条带",是五种标准化模式之一(即 1 型 = 血清和 CSF 中均无条带;2 型 = CSF 中存在 OBs;3 型 = CSF 中存在 OBs,血清和 CSF 中均存在额外的相同 OBs,表明存在鞘内合成;4 型 = 血清和 CSF 中均存在相同的 OBs,即 "镜像模式";5 型 = 血清和 CSF 中均存在单克隆条带)。以往的研究仅在 THS(4 型)的脑脊液中发现了 OB。镜像模式带 "的发现非常有趣,因为它揭示了 THS 潜在的可能的全身免疫激活机制。
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Headache, Ophthalmoplegia, “Mirror Pattern Bands”: Two Case Reports

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.

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