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17 January 2023 : Case report  Brazil

[In Press] Surgical Treatment Outcome for Familial Melkersson-Rosenthal Syndrome

Unusual setting of medical care, Congenital defects / diseases

Tannara Nobile Alencar1ABEF, Marcela Machado Botelho2BD, Natália Carasek2EF, Fayez Bahmad Jr.2BE

DOI: 10.12659/AJCR.938670

Am J Case Rep In Press; DOI: 10.12659/AJCR.938670  

Available online: 2023-01-17, In Press, Corrected Proof

Publication in the "In-Press" formula aims at speeding up the public availability of the pending manuscript while waiting for the final publication. The assigned DOI number is active and citable. The availability of the article in the Medline, PubMed and PMC databases as well as Web of Science will be obtained after the final publication according to the journal schedule

Abstract

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.

Keywords: Facial Paralysis; Melkersson-Rosenthal Syndrome; Microvascular Decompression Surgery; Tongue, Fissured

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923