2019, Number 2
Melkersson-Rosenthal syndrome
Romero-Martínez DG, Vales-Hidalgo LO, Vega-Alarcón A, Palma-Díaz M, Morales-Del Ángel JA, Diplan JM
Language: Spanish
References: 0
Page: 52-60
PDF size: 197.22 Kb.
ABSTRACT
Background: Melkersson-Rosenthal syndrome is a rare disease that affects the nervous system and skin (granulomatous neuromucocutaneous disease), its etiology is unknown. Melkersson-Rosenthal syndrome may be characterized by three main features: recurrent facial nerve palsy, episodes of swelling of the face and lips, and fissuring of the tongue. The majority of people with Melkersson-Rosenthal syndrome only have one or two of these features.Objective: To introduce the experience in the Facial Nerve department of Neurology and Neurosurgery National Institute Manuel Velasco Suarez, Mexico City, about Melkersson-Rosenthal syndrome.
Material and Method: A retrospective study of patients with Melkersson-Rosenthal syndrome was done between 1983-2015. We analyzed different features that let us know the role of this disease.
Results: We identified 40 patients with Melkersson-Rosenthal syndrome, 27 were females and 13 were males; 100% of patients presented with recurrent facial nerve palsy. The onset of the disease was an average of 22.2 years old. The average number of facial nerve palsy was 3.3 per patient, with predominance of the left side. At this moment, 50% with intact facial function (House-Brackmann score I), 47.5% with House-Brackmann score II; 35% of patients presented full triad and 65% the olygosymptomatic form.
Conclusion: The olygosymptomatic form is predominant in Melkersson-Rosenthal syndrome. In patients with recurrent facial nerve palsy is mandatory to investigate episodes of swelling of the face and lips, and fissuring of the tongue.