Melkersson-Rosenthal综合征双侧面瘫的表现。

IF 0.9 Q4 CLINICAL NEUROLOGY
Case Reports in Neurological Medicine Pub Date : 2021-01-06 eCollection Date: 2021-01-01 DOI:10.1155/2021/6646115
Gustavo Gaitan-Quintero, Loida Camargo-Camargo, Norman López-Velásquez, Miguel González
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引用次数: 0

摘要

介绍。Melkersson-Rosenthal综合征(MRS)是一种神经粘膜皮肤疾病,其特征为以下经典症状三联征:周围面瘫、口面水肿和阴囊或舌裂。这种病很罕见,而且由于大多数患者都是少症状或单症状,因此很难诊断。临床病例。我们报告一位26岁男性患者,11岁时有镰状细胞特征病史,未经治疗的打鼾,并伴有左侧周围性面瘫。这是一项为期20天的临床研究,从左侧周围面瘫开始,伴有中等强度的枕部搏动性头痛。此外,患者有舌头感觉异常和唇部水肿的感觉。一周后,他表现为右侧周围性面瘫。体格检查显示双侧周围面瘫,轻度唇水肿,阴囊或舌裂。患者接受皮质类固醇治疗,水肿和面瘫得到改善。讨论。MRS是一种罕见的疾病,主要影响女性,通常从20多岁或30多岁开始。病因不明。然而,已经提出了一个涉及环境因素和遗传易感性的多因素起源,它导致局部免疫系统和自主神经系统(ANS)功能障碍以及嘴唇和舌头肉芽肿炎症的出现。面瘫通常在晚些时候出现;然而,它可以发生在临床首次亮相。没有治愈的方法。治疗的重点是调节患者的免疫反应,复发是频繁的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Presentation of Bilateral Facial Paralysis in Melkersson-Rosenthal Syndrome.

Presentation of Bilateral Facial Paralysis in Melkersson-Rosenthal Syndrome.

Introduction. Melkersson-Rosenthal syndrome (MRS) is a neuromucocutaneous disorder characterized by the following classic symptom triad: peripheral facial paralysis, orofacial edema, and scrotal or fissured tongue. It is rare, and since most of the patients are oligo- or monosymptomatic, it makes it difficult to diagnose. Clinical Case. We present a 26-year-old male patient with a history of sickle cell trait, untreated snoring, and left peripheral facial paralysis when he was 11 years old. This was an overall 20-day clinical profile that started with left peripheral facial paralysis, which was accompanied by moderate-intensity occipital pulsatile headaches. Additionally, the patient experienced paresthesias in the tongue and feelings of labial edema. After one week, he manifested peripheral facial paralysis on the right side. Physical examination revealed bilateral peripheral facial paralysis, mild labial edema, and a scrotal or fissured tongue. The patient received corticosteroids, which resulted in improvement of the edema and facial paralysis. Discussion. MRS is a rare disorder that predominantly affects women, typically starting in their 20s or 30s. The etiology is unknown. However, a multifactorial origin that involves environmental factors and a genetic predisposition has been proposed, which causes a dysfunction of the local immune system and autonomic nervous system (ANS) and an appearance of granulomatous inflammation in the lips and tongue. Facial paralysis usually appears later on; however, it can occur from its clinical debut. There are no curative treatments. Therapy is focused on modulating the patient's immune response, and relapses are frequent.

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