Post-Graduate Program of the School of Health Science, Superior School of Health Science (ESCS), Brasilia, DF, Brazil.
Post-Graduation Program at Health Science Faculty, University of Brasilia, Brasilia, DF, Brazil.
Am J Case Rep. 2023 Feb 9;24:e938670. doi: 10.12659/AJCR.938670.
BACKGROUND Recurrent facial nerve palsy, orofacial edema, and fissured tongue are a triad of manifestations that characterize a rare disorder named Melkersson-Rosenthal syndrome. It is important to consider this syndrome when diagnosing atypical, unilateral, or bilateral facial palsies with characteristics of familial prevalence. There is no established outcome prediction for this disease and the syndrome does not have a specific duration or prospective timeline. Recurrent facial paralysis can require surgery and a multidisciplinary approach with regular follow-up. CASE REPORT We describe a 38-year-old woman presenting with a third episode of facial paralysis and discuss her pedigree chart and the treatment course chosen. After conservative treatment with oral corticosteroids, antiviral therapy, and motor physical therapy with no significant improvements, the patient underwent facial nerve decompression surgery with outstanding results. Eight months after surgery and intense postoperative physical therapy, the patient improved from grade VI to grade II palsy on the House-Brackmann Scale. The patient's older brother also presented a fissured tongue and had a history of 2 episodes of facial paralysis. The patient's son, mother, and sister also presented tongue fissuring but did not have any other clinical signs of the syndrome. CONCLUSIONS Despite being rare, Melkersson-Rosenthal syndrome is associated with a family inheritance and its diagnosis has prognostic implications. Therefore, it is of the utmost importance to have suspicion of this disorder in order to improve quality of care and target the treatment accordingly. Surgical treatment in these cases seems to be an excellent choice to treat current facial paralysis and prevent further episodes.
复发性面神经麻痹、口面部肿胀和裂纹舌是一种罕见疾病——梅尔基奥尔森-罗森塔尔综合征的三联征表现。在诊断具有家族性发病倾向的非典型性、单侧或双侧面神经麻痹时,应考虑到这种综合征。目前还没有针对该疾病的既定预后预测,且该综合征没有特定的持续时间或前瞻性时间表。复发性面神经麻痹可能需要手术治疗和多学科方法,并定期进行随访。
我们描述了一位 38 岁女性出现第三次面神经麻痹的病例,并讨论了她的家族谱图和选择的治疗方案。在接受了保守治疗(包括口服皮质类固醇、抗病毒治疗和运动物理疗法)但无明显改善后,患者接受了面神经减压手术,效果显著。术后 8 个月,患者接受了密集的术后物理治疗,House-Brackmann 量表评级从 VI 级改善到 II 级。患者的哥哥也有裂纹舌,并有两次面神经麻痹病史。患者的儿子、母亲和姐姐也有舌裂,但没有该综合征的其他任何临床体征。
尽管罕见,但梅尔基奥尔森-罗森塔尔综合征与家族遗传有关,其诊断具有预后意义。因此,怀疑存在这种疾病对于提高护理质量和针对性治疗至关重要。对于这些病例,手术治疗似乎是治疗当前面神经麻痹和预防进一步发作的极佳选择。