Zečević Penić Sandra, Lisak Marijana, Gregurić Tomislav, Hećimović Hrvoje, Bašić Kes Vanja
Sveti Ivan Psychiatry Hospital.
Clinical Department of Neurology, Sestre milosrdnice University Hospital Center, Zagreb, Croatia.
Acta Clin Croat. 2017 Jun;56(2):331-337. doi: 10.20471/acc.2017.56.02.18.
This case report presents a patient diagnosed with Tolosa-Hunt syndrome (THS) after an extensive neuro-diagnostic and neuroimaging evaluation. Diagnostic work-up included thorough physical and neurological examination, complete laboratory serum assessments, neuroendocrine and immunohistochemistry analysis, cerebrospinal fluid analysis, neurophysiology assessment, ophthalmologist examination and neuroimaging. Th e most important diagnostic tool in deriving the diagnosis of THS was neuroimaging evaluation that included baseline and follow-up cranial contrast enhanced magnetic resonance imaging (MRI). Baseline cranial contrast enhanced MRI detected a nonspecific inflammatory granulomatous lesion in the right cavernous sinus extending basally towards the right trigeminal cave (Meckel's cave) and anteriorly towards the apex of the right orbit. Systemic intravenous high-dose corticosteroid therapy was administered for 3 consecutive days and then tapered down to lower oral steroid doses. Following therapy, the patient experienced complete regression of symptoms. Follow-up cranial contrast enhanced MRI showed significant regression of inflammatory lesion in the area of right cavernous sinus, thus verifying the efficacy of the treatment applied. This paper shows that an extensive diagnostic schedule for THS must be conducted prior to therapeutic treatment, for the possibility of alternative diagnosis. Patients suspected of having THS require careful evaluation, appropriate treatment, and follow-up.
本病例报告介绍了一名患者,在经过广泛的神经诊断和神经影像学评估后被诊断为托洛萨-亨特综合征(THS)。诊断检查包括全面的体格和神经系统检查、完整的实验室血清评估、神经内分泌和免疫组织化学分析、脑脊液分析、神经生理学评估、眼科医生检查以及神经影像学检查。得出THS诊断的最重要诊断工具是神经影像学评估,其中包括基线和随访的头颅增强磁共振成像(MRI)。基线头颅增强MRI在右侧海绵窦发现一个非特异性炎性肉芽肿病变,该病变基部延伸至右侧三叉神经腔(梅克尔腔),前部延伸至右侧眶尖。连续3天给予全身静脉高剂量皮质类固醇治疗,然后逐渐减量至较低的口服类固醇剂量。治疗后,患者症状完全消退。随访头颅增强MRI显示右侧海绵窦区域的炎性病变明显消退,从而证实了所应用治疗的有效性。本文表明,在进行治疗之前,必须针对THS进行广泛的诊断程序,以排除其他诊断的可能性。疑似患有THS的患者需要仔细评估、适当治疗和随访。