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儿童托洛萨-亨特综合征的不寻常临床过程。

Unusual clinical course in pediatric Tolosa-Hunt syndrome.

机构信息

Department of Pediatrics, Chang Gung Memorial Hospital- Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan, Republic of China.

出版信息

Pediatr Neurol. 2011 Jun;44(6):471-4. doi: 10.1016/j.pediatrneurol.2011.01.010.

Abstract

We report on a 7-year-old immunocompetent boy initially presenting with right-sided frontal cephalalgia, painful ophthalmoplegia, and ptosis for 1 month. Initial cerebrospinal fluid analysis produced normal results. Magnetic resonance imaging revealed an inflammatory pseudotumor of the right cavernous sinus after intravenous gadolinium administration, indicating a rare idiopathic inflammatory disorder of the cavernous sinus, i.e., Tolosa-Hunt syndrome. Ptosis and cephalalgia resolved after steroid treatment, although right-sided ophthalmoplegia remained. Breakthrough headache, associated with signs of meningeal irritation, developed 6 weeks later. Follow-up contrast-enhanced computed tomography revealed no enhancing cavernous soft tissue mass. A further lumbar puncture disclosed central nervous system infection with Staphylococcus saprophyticus. After 6 weeks of vancomycin, the headache resolved completely, and neuroimaging produced normal results. A diagnosis of Tolosa-Hunt syndrome should be rendered cautiously, because the etiology may involve a rare but not "idiopathic" infection. Moreover, if clinical signs are not fully responsive to steroid treatment, the underlying problems should receive careful investigation.

摘要

我们报告了一例 7 岁免疫功能正常的男孩,最初表现为右侧额头痛、痛性眼肌麻痹和上睑下垂,持续了 1 个月。最初的脑脊液分析结果正常。静脉注射钆后磁共振成像显示右侧海绵窦炎性假瘤,提示海绵窦罕见的特发性炎症性疾病,即托洛萨-亨特综合征。经类固醇治疗后,上睑下垂和头痛缓解,但右侧眼肌麻痹仍存在。6 周后出现突破性头痛,伴有脑膜刺激征。随访增强 CT 显示海绵窦软组织无强化。进一步腰椎穿刺显示中枢神经系统感染表皮葡萄球菌。万古霉素治疗 6 周后,头痛完全缓解,神经影像学检查结果正常。托洛萨-亨特综合征的诊断应慎重,因为病因可能涉及罕见但并非“特发性”的感染。此外,如果临床症状对类固醇治疗反应不完全,应仔细调查潜在问题。

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