Complete Melkersson-Rosenthal syndrome: an exceptional cause of facial nerve palsy

Abstract

Melkersson-Rosenthal syndrome (MRS) is a rare disease, with unknown etiology characterized by oro-facial oedema, fissuring tongue and peripheral facial palsy. The mainstay treatment is corticosteroids. We report the case of a 38-year old female, who presented six recurrent episodes of left peripheral facial palsy associated with simultaneous oedema of the median frontal area, treated successfully by short course oral corticosteroids with neuromuscular facial re-education. However, the recovery of the last episode was incomplete and the patient kept a left House-Brackmann grade II facial palsy at six months. The median frontal area was slightly inflammatory, being the location of simultaneous repetitive oedema and the tongue was fissured. These cardinal symptoms realise the complete triad of Melkersson-Rosenthal syndrome (MRS). The histopathological examination of the lip biopsy showed lymphocytic inflammation around the blood vessels. Since the residual facial palsy was graded as mild dysfunction, the frontal oedema fully recovered and the fissured tongue was not painful, the treatment consisted on appropriate neuromuscular re-education. The 6 months follow-up showed no recurrence with a stable grade II left facial palsy. We present this case to supplement the rare literature data concerning the management of this rare entity. Patients should be prepared to the risk of recurrent episodes with longer duration of symptoms and more incomplete recoveries, which may indicate other therapeutic options.