Melkersson-Rosenthal syndrome: a facial nerve center perspective

Abstract

Objectives: Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous granulomatous disorder of unknown etiology, characterized by the triad of facial palsy, lingua plicata (fissured tongue), and orofacial edema. Few articles in the literature report series with more than 20 patients or focus on the facial nerve dominant presentation of MRS.

Methods: We performed a retrospective review of the patients diagnosed with MRS at a university-based Facial Nerve Center.

Results: Twenty-one patients were identified from 1971 to 2010. The age of presentation ranged from 22 to 67 years (mean 44.1). Seven (33.3%) were male and 14 (66.7%) were female. All (100%) patients had facial paralysis. Fourteen (66.7%) patients who initially presented with unilateral paralysis subsequently developed metachronous contralateral paralysis (alternating unilateral facial paralysis). One (4.7%) patient had simultaneous bilateral facial paralysis. The number of episodes per patient ranged from 1 to 8 (mean 3.1). Laterality was relatively equal: 35 episodes occurred on the right side and 31 on the left. The patient with most episodes of facial paralysis had four on the left and four on the right (metachronous). This was followed by three patients with six episodes each. The age of first incidence of facial paralysis ranged from 2 to 60 years (mean 34.4, median of 39). The mean interval between episodes was 4.7 years (range 0-30, median 3). Six (28.5%) of the patients reported a family history of MRS.

Conclusions: MRS is a rare disease of unknown pathogenesis in which oligosymptomatic forms predominate. Patients with this disease may present to different specialties complaining of different symptoms, and frequently, not all the classic features of the triad will be present. In our series of facial paralysis patients diagnosed with MRS, a higher proportion had the full triad of symptoms than has been previously reported in the literature.

Keywords: Facial palsy; Facial paralysis; Fissured tongue; Lingua plicata; Melkersson–Rosenthal syndrome; Orofacial edema.