Melkersson-Rosenthal Syndrome (MRS) is a rare condition of the neurological system distinguished by a classic triad of symptoms that comprises of long-term and recurring facial swelling especially affecting the lips, development of folds in the tongue (fissured tongue), and muscular weakness of face (facial palsy). Some of the patients may have all three symptoms while others suffer from only two. Symptoms start appearing in early adolescence for the first time and then recurs for life.
No cause has been established for Melkersson-Rosenthal Syndrome (MRS). However, a genetic link has been discovered in some families including cases where both twins are affected. Other possible risk factors include Crohn’s disease, sarcoidosis, particular allergens and some dietary factors.
Signs and Symptoms
Patients of Melkersson-Rosenthal Syndrome (MRS) usually present with the following classic triad:
- Facial Palsy: Episodic recurrence in which weakness and twitching of the facial muscles occur on one or both sides of the face.
- Fissured Tongue: Folds and grooves in the tongue
- Facial Swelling: Swelling of the face occurs. Usually, one or both lips are also involved. Lips may become hard and cracked with a reddish brown pigmentation which is known as granulomatous cheilitis. Facial swelling is also episodic and recurs within days to years following the previous attack.
Other non-specific signs and symptoms include visual disturbances, headache and fever during an episode. Sometimes, taste sensation is also lost and salivary production is reduced leading to dryness of the mouth.
Diagnosis of Melkersson-Rosenthal Syndrome is based mostly on the classic triad of symptoms. However, it should be differentiated from conditions such as Bell’s Palsy in which only facial paralysis and weakness occurs. Other conditions such as Crohn’s Disease and sarcoidosis should be ruled out as well through laboratory tests. Biopsy of the lips is performed in some cases to diagnosis granulomatous cheilitis which is one of the manifestations of Melkersson-Rosenthal Syndrome.
Treatment of Melkersson-Rosenthal Syndrome is focused on managing the symptoms.
- Drug Therapy: Steroids and non-steroidal anti-inflammatory drugs (NSAIDs) in the form of oral tablets and injections are available to reduce swelling. Immunosuppressants and antibiotics are also used.
- Surgical Treatment: Reduction cheiloplasty has been performed on some patients of MRS who had recurrent and painful lip swelling with fruitful results.
Other surgeries can reduce pressure on the facial nerves by removing parts of swollen tissue but they are not always effective and may carry the risk of complications.
- Miscellaneous: Electrical stimulation and massage therapy has been used to control symptoms of MRS without clinically proven efficacy.