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[托洛萨·亨特综合征与自身免疫性多腺体综合征。一例罕见病例报告]

[Tolosa Hunt syndrome and autoimmune polyglandular syndrome. A rare case report].

作者信息

Cesareo R, Reda G, Verallo O

机构信息

Ambulatorio Divisionale di Endocrinologia, Ospedale S. Pertini, Roma.

出版信息

Minerva Endocrinol. 1995 Jun;20(2):149-52.

PMID:8531898
Abstract

The authors refer to a case report in which two rare clinical syndromes, the Tolosa Hunt Syndrome (THS) and the polyglandular auto-immune syndrome (PGA) co-exist in the same patient and make a detailed analysis of the literature regarding this kind of diseases. The THS is a rare clinical condition characterized by a painful ophthalmoplegia due to an involvement of the cranial nerves and of the sympathetic nerve-fibres going through the cavernous sinus and the superior orbit cavity. Nowadays the diagnosis of TSH is made by high resolution computerized tomography aimed at the cavernous sinus and the upper orbital cavity. This method allows to notice if at the basis of this syndrome there are vascular, neoplastic or inflammatory diseases or if, as in e case here presented, by exclusion, we must think of an inflammatory process with unknown pathogenesis. In this case report the patient under observation process to be affected by THS and also by auto-immune polyglandular syndrome type III, as she was affected by diabetes type I, Basedow disease and alopecia. The authors believe that an auto-immune mechanism can be at basis of the THS, and this fact has already been pointed out in the literature in two previous works. An important feature of this case report has been the definitive improvement of the symptoms by an antithyroid effect and, also, by an immune suppressor activity. The Authors hope that there will be reported a greater number of cases pointing to the coexistence of the THS with autoimmune diseases.

摘要

作者提及一份病例报告,其中两种罕见临床综合征,即托洛萨·亨特综合征(THS)和多腺体自身免疫综合征(PGA)在同一患者中共存,并对有关这类疾病的文献进行了详细分析。THS是一种罕见的临床病症,其特征为因穿过海绵窦和眶上腔的颅神经及交感神经纤维受累而导致疼痛性眼肌麻痹。如今,TSH的诊断通过针对海绵窦和眶上腔的高分辨率计算机断层扫描进行。这种方法能够发现该综合征的病因是否为血管性、肿瘤性或炎症性疾病,或者像在此呈现的病例中那样,通过排除法,我们是否必须考虑一种发病机制不明的炎症过程。在这份病例报告中,观察的患者不仅患有THS,还患有III型自身免疫性多腺体综合征,因为她患有I型糖尿病、格雷夫斯病和脱发。作者认为自身免疫机制可能是THS的病因,这一事实在之前的两篇文献中已有提及。这份病例报告的一个重要特征是,通过抗甲状腺作用以及免疫抑制活性,症状得到了明确改善。作者希望能报告更多表明THS与自身免疫性疾病共存的病例。

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