Melkersson-rosenthal综合征并发面瘫和颅内高压

Elis Penteado Arantes, Giuliana Vieira Pretti, Soo Yang Lee, F. Leme
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摘要

自1928年神经学家Melkersson[1]首次描述,并于1931年由Rosenthal C[2]补充以来,Melkersson[1]和Rosenthal[2]综合征(SMR)已被证明是一种罕见的疾病,仍需要特异性治疗[3,4]。我们描述的情况下,一个年轻的病人,谁在23岁有第一次发作周围面瘫的权利。当时,它以一种协议方式进行,使用强的松,扑热息痛和眼部护理,以及相关的物理治疗。面部不对称有所改善,在2006年,她展示了一种新的周围性面瘫,这次是在左边,用同样的方法进行。然后,在3年的时间间隔内,她又出现了2次面瘫,并伴有美学后遗症。2009年,在第5次麻痹发作时,她伴有面部水肿和唇炎,当时要求对上眼睑进行活检,但活检不明确。2012年,第8次面瘫后,再次行活检,发现炎性浸润,符合临床对SMR的怀疑。影像学检查(颅骨磁共振和血管磁共振)、面部神经肌电图预测预后和脑脊液检查均无异常。那一年,她开始出现右侧眼、口轮肌肌不自主、节律性收缩,除了视觉肌和颈后肌外,还出现面部半瘫,每4 ~ 6个月服用100 IU肉毒杆菌。在整个治疗期间,强的松10mg与地扎柯6mg交替使用,2019年,由于持续头痛,她进行了动脉和静脉颅血管共振的新共振,提示颅内高压(IH)的元素。目前,患者正在接受双侧面神经减压手术和IH治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
MELKERSSON-ROSENTHAL SYNDROME COMPLICATED WITH FACIAL HEMIESPASM AND INTRACRANIAL HYPERTENSION
Since it was first described in 1928 by neurologist Melkersson [1], and complemented in 1931 by Rosenthal C [2], the Melkersson [1] and Rosenthal [2] Syndrome (SMR) has been shown to be a rare disorder and still in need of specific treatment [3,4]. We describe the case of a young patient, who at 23 years old had the first episode of peripheral facial paralysis on the right. At the time, it was conducted in a protocol manner, with prednisone, paracetamol and eye care, as well as pertinent physiotherapy. There was an improvement in facial asymmetry, when in 2006 she presented a new peripheral facial paralysis, this time on the left, which was conducted in a similar way. Then, she presented 2 more episodes of facial paralysis in the interval of 3 years, already with aesthetic sequelae. In 2009, in the 5th episode of paralysis, she associated facial edema and cheilitis, when biopsy of the upper eyelid was requested, which was unspecific. In 2012, after the 8th facial paralysis, she underwent a new biopsy, in which an inflammatory infiltrate was demonstrated, consistent with clinical suspicion of SMR. Imaging exams (skull resonance and angioresonance), face electroneuromyography for prognosis, and CSF study, without abnormalities, were performed. That year she began to experience involuntary and rhythmic contractions of the orbicularis musculature of the eyes and mouth on the right, in addition to risory and platysma, featuring facial hemispasm, treated every 4 to 6 months with 100 IU Onabotulinum. Throughout the treatment period, alternating use of 10mg prednisone with deflazacort 6mg, and in 2019, due to persistent headache, she underwent a new resonance with arterial and venous cranial angioresonance, which brought elements suggestive of Intracranial Hypertension (IH). Currently, the patient is undergoing surgical programming for facial nerve decompression bilaterally and undergoing treatment for IH.
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