Complete clinical spectrum of melkersson–Rosenthal syndrome with bilateral temporal optic disc pallor – A case report

Abstract

Melkersson Rosenthal syndrome (MRS) is a very rare neuro-mucocutaneous disease, the aetiology of which is not well defined. It is classically characterised by recurring facial paralysis, swelling of face, lips and presence of a furrowed tongue. The presentation is usually oligosymtomatic, with only about one-fourth of cases showing the classical triad. Here, we present a 32-year-old female with MRS, showing the complete clinical triad of symptoms. We report for the first time, an association of MRS with bilateral optic nerve involvement. A standard criterion for diagnosis and management is lacking. It is important to increase awareness of such a rare syndrome with various associated manifestations, so that it can be accurately identified and treated.