Department of Neurology, Chinese PLA General Hospital, No. 28, Fuxing Road, Beijing 100853, China.
J Clin Neurosci. 2013 Jul;20(7):993-5. doi: 10.1016/j.jocn.2012.10.009. Epub 2013 May 7.
Melkersson-Rosenthal syndrome (MRS) is a rare disorder consisting of a triad of persistent or recurrent orofacial edema, relapsing facial paralysis and fissured tongue. It is difficult to diagnose and treat. A retrospective review of our MRS patients was performed. The medical files, and treatment, radiologic and histopathologic records of these patients were reviewed. The study group consisted of seven male MRS patients. In four patients, the three classical manifestations were present simultaneously. Neuroimaging examinations found facial soft-tissue thickening and swelling in two patients. The indicators of the disease in our patients were as follows: two had family histories, two had elevated levels of protein in the cerebrospinal fluid, one had an increased immunoglobulin G level in the blood and one had reactive arthritis. All patients responded to systemic corticosteroid treatment. We discuss the genetic, infectious and immunologic factors in the etiology of MRS. Neuroimaging examinations were useful in determining the diagnosis and in determining whether the initial treatment of MRS should be with systemic corticosteroids.
梅尔基奥森-罗森塔尔综合征（MRS）是一种罕见的疾病，由持续性或复发性口面水肿、复发性面瘫痪和裂纹舌三联征组成。其诊断和治疗都具有一定难度。我们对 MRS 患者进行了回顾性研究。对这些患者的病历、治疗、影像学和组织病理学记录进行了回顾。研究组包括 7 名男性 MRS 患者。其中 4 名患者同时存在这三个典型表现。神经影像学检查发现 2 名患者存在面部软组织增厚和肿胀。我们患者的疾病指标如下：2 名患者有家族史，2 名患者的脑脊液蛋白升高，1 名患者的血液免疫球蛋白 G 水平升高，1 名患者患有反应性关节炎。所有患者均对全身皮质类固醇治疗有反应。我们讨论了 MRS 病因中的遗传、感染和免疫因素。神经影像学检查有助于确定诊断，并确定 MRS 的初始治疗是否应采用全身皮质类固醇。
