Melkersson-Rosenthal syndrome is characterized by a triad of recurrent episodes of facial paralysis and facial edema along with a fissured tongue. Other neurologic sequelae have been documented, including headache, trigeminal neuralgia, cranial nerve dysfunction, and autonomic dysfunction. Although the etiology is unknown, recent investigation points toward a granulomatous disease leading to recurrent attacks of edema and inflammation of the face and oral cavity with a predilection for facial nerve involvement. Facial nerve involvement may be unilateral or bilateral, with varying frequencies of attacks and progression. No confirmatory blood test, histopathology, or radiographic evaluation is available, as the diagnosis is entirely clinical.
Treatment for the intermittent attacks of facial edema consists of topical, intralesional, or systemic corticosteroid therapy. Mixed results have also been found with various other therapies, including dapsone, clofazimine, sulfasalazine, and antihistamines (81). Recurrent attacks of facial paralysis may result in synkinesis or residual paralysis. For such cases, many investigators now propose surgical decompression as the treatment of choice. Initial surgical procedures decompressing only the horizontal and vertical segments of the facial nerve left patients with continued attacks of facial paralysis (82). Evidence has mounted that the likely site of involvement is within the labyrinthine segment, and current recommendations are for patients with an increasing frequency, duration, and severity of facial paralysis attacks to undergo a combined middle-fossa transmastoid decompression of the entire course of the intratemporal facial nerve and opening of the nerve sheath (81). This procedure has been shown in several series to prevent further attacks of facial paralysis (83).
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