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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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Autores principales: Gaitan-Quintero, Gustavo, Camargo-Camargo, Loida, López-Velásquez, Norman, González, Miguel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7806376/
https://www.ncbi.nlm.nih.gov/pubmed/33505744
http://dx.doi.org/10.1155/2021/6646115
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author Gaitan-Quintero, Gustavo
Camargo-Camargo, Loida
López-Velásquez, Norman
González, Miguel
author_facet Gaitan-Quintero, Gustavo
Camargo-Camargo, Loida
López-Velásquez, Norman
González, Miguel
author_sort Gaitan-Quintero, Gustavo
collection PubMed
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.
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spelling pubmed-78063762021-01-26 Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome Gaitan-Quintero, Gustavo Camargo-Camargo, Loida López-Velásquez, Norman González, Miguel Case Rep Neurol Med Case Report 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. Hindawi 2021-01-06 /pmc/articles/PMC7806376/ /pubmed/33505744 http://dx.doi.org/10.1155/2021/6646115 Text en Copyright © 2021 Gustavo Gaitan-Quintero et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Gaitan-Quintero, Gustavo
Camargo-Camargo, Loida
López-Velásquez, Norman
González, Miguel
Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome
title 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_short Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome
title_sort presentation of bilateral facial paralysis in melkersson–rosenthal syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7806376/
https://www.ncbi.nlm.nih.gov/pubmed/33505744
http://dx.doi.org/10.1155/2021/6646115
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