Arthur Anupriya, Sivadasan Ajith, Mannam Pavitra, Prabakhar A T, Aaron Sanjith, Mathew Vivek, Karthik M, Benjamin Rohith Ninan, Iqbalahmed Shaikh Atif, Rynjah Gideon Lyngsyun, Alexander Mathew
Department of Ophthalmology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
Department of Neurological Sciences, Neurology Unit, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
Ann Indian Acad Neurol. 2020 Mar-Apr;23(2):201-205. doi: 10.4103/aian.AIAN_368_18. Epub 2020 Feb 25.
Tolosa-Hunt Syndrome (THS) is one of the causes of cavernous sinus syndrome causing painful ophthalmoplegia. Literature on long-term outcome of this rare condition is scarce.
The aim is to study the recurrence and role of steroid-sparing agents in THS.
All cases of THS treated at a tertiary-level teaching hospital during a 10-year period were studied. Clinical and radiological profile, response to treatment and recurrences were noted.
A total of 44 cases were studied. The mean age was 49.5 years, Males constituted 23/44 (52%). The first symptom was pain in 90%. Ptosis with ophthalmoplegia was the most common deficit 29/44 (66%). Lesions confined to cavernous sinus 27/44 (61%) was the most frequent magnetic resonance imaging finding. All patients received steroids as the initial treatment and 15/44 (34%) received steroid-sparing agents. Follow-up ranged from 6 to 120 months (Mean 39 months). Two patients had alternative diagnosis of leptomeningeal malignancy and hypertrophic pachymeningitis on follow-up. Recurrences occurred in 18/37 (48.6%). Time for recurrence varied from 8 months to 7 years. (Mean 18 months). No clinical or radiological predictors for recurrence were identified. Patients who received steroid-sparing agents had a significantly lower recurrence 3/15 (20%) versus 14/26 (53.8%)P < 0.034.
Around 50% of patients with THS can have recurrence. Steroid-sparing agents appear to prevent recurrence. A prospective multicenter randomized controlled trial may help to evaluate the risk and benefits of steroid-sparing therapy and to identify any possible predictors for recurrence.
托洛萨-亨特综合征（THS）是海绵窦综合征导致疼痛性眼肌麻痹的病因之一。关于这种罕见疾病长期预后的文献较少。
研究THS中复发情况及激素替代药物的作用。
对一家三级教学医院在10年期间治疗的所有THS病例进行研究。记录临床和影像学特征、治疗反应及复发情况。
共研究44例病例。平均年龄49.5岁，男性占23/44（52%）。90%的患者首发症状为疼痛。上睑下垂伴眼肌麻痹是最常见的缺陷，占29/44（66%）。磁共振成像最常见的表现是病变局限于海绵窦，占27/44（61%）。所有患者均接受激素作为初始治疗，15/44（34%）接受激素替代药物治疗。随访时间为6至120个月（平均39个月）。2例患者在随访中被诊断为软脑膜恶性肿瘤和肥厚性硬脑膜炎。18/37（48.6%）出现复发。复发时间从8个月到7年不等（平均18个月）。未发现复发的临床或影像学预测因素。接受激素替代药物治疗的患者复发率显著较低，为3/15（20%），而未接受者为14/26（53.8%），P<0.034。
约50%的THS患者会复发。激素替代药物似乎可预防复发。一项前瞻性多中心随机对照试验可能有助于评估激素替代疗法的风险和益处，并确定任何可能的复发预测因素。
