16例Tolosa-Hunt综合征的临床分析

目的:对Tolosa-Hunt综合征的临床表现、影像学特点及治疗转归进行分析总结，以提高该病的诊治水平。方法:收集海军总医院2006年4月—2014年1月收治的16例Tolosa-Hunt综合征患者的临床资料，总结其临床表现、实验室检查、影像学特点及预后，并结合文献资料进行分析。结果:16例多为急性或亚急性起病，病程从3 d至8个月不等。症状以头痛和同侧眼肌麻痹为主，多累及Ⅲ、Ⅳ、Ⅴ1、Ⅵ多组颅神经。激素冲击治疗后疼痛症状迅速好转，颅神经麻痹症状在随后的1周至3个月内恢复，1例病程较长患者遗留眼肌麻痹。头颅MRI发现海绵窦、眶尖区软组织影，在T1加权像呈低信号或等信号，T2加权像呈稍高或等信号。结论:Tolosa-Hunt综合征临床表现以头痛、眼肌麻痹为主，对激素治疗敏感，颅脑增强MRI可为诊断提供客观的影像学依据，对患者定期随诊并复查颅脑增强MRI，可以减少误诊及漏诊。

Clinical study of 16 patients with Tolosa-Hunt syndrome

ObjectiveTo analyze the clinical manifestation, imaging, and outcome of Tolosa-Hunt syndrome for an insight into this syndrome. MethodsSixteen cases of Tolosa-Hunt syndrome were diagnosed in our hospital from April 2006 to January 2014. Clinical manifestation, laboratory examination, neuroimaging findings, treatment and outcome were reviewed and summarized. ResultsMost patients developed disease in acute or subacute mode. The most frequent sign was unilateral orbital pain and oculomotor paresis with prompt relief of symptoms to steroid treatment. Cranial nerve palsy were recovered in the following one to three months. One patient with long duration remained ophthalmoplegia. ConclusionThe main clinical manifestations of painful ophthalmoplegia is hea dache, ophthalmoplegia and good responses to steroid treatment. The MR imaging findings of brain can provide accordance in diagnosing the disease. Since diagnosis and differential diagnosis are difficult for the patients with normal MR imaging, MRI enhancement of brain is needed for accurate diagnosis.