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...

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Main Authors: Gustavo Gaitan-Quintero, Loida Camargo-Camargo, Norman López-Velásquez, Miguel González
Format: Article
Language:English
Published: Hindawi Limited 2021-01-01
Series:Case Reports in Neurological Medicine
Online Access:http://dx.doi.org/10.1155/2021/6646115
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spelling doaj-5a00c2a1ab4e42258dba3e0a8398af9c2021-02-15T12:52:58ZengHindawi LimitedCase Reports in Neurological Medicine2090-66682090-66762021-01-01202110.1155/2021/66461156646115Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal SyndromeGustavo Gaitan-Quintero0Loida Camargo-Camargo1Norman López-Velásquez2Miguel González3Neurology Residency Program of Sinú University, Cartagena, ColombiaNeurology Residency Program of Sinú University, Cartagena, ColombiaUniversidad de La Costa, Barranquilla, ColombiaDuke University, Durham, NC, USAIntroduction. 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.http://dx.doi.org/10.1155/2021/6646115
collection DOAJ
language English
format Article
sources DOAJ
author Gustavo Gaitan-Quintero
Loida Camargo-Camargo
Norman López-Velásquez
Miguel González
spellingShingle Gustavo Gaitan-Quintero
Loida Camargo-Camargo
Norman López-Velásquez
Miguel González
Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome
Case Reports in Neurological Medicine
author_facet Gustavo Gaitan-Quintero
Loida Camargo-Camargo
Norman López-Velásquez
Miguel González
author_sort Gustavo Gaitan-Quintero
title Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome
title_short Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome
title_full Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome
title_fullStr Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome
title_full_unstemmed Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome
title_sort presentation of bilateral facial paralysis in melkersson–rosenthal syndrome
publisher Hindawi Limited
series Case Reports in Neurological Medicine
issn 2090-6668
2090-6676
publishDate 2021-01-01
description 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.
url http://dx.doi.org/10.1155/2021/6646115
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