What is Melkersson-Rosenthal Syndrome?
Melkersson-Rosenthal Syndrome, also termed as Miescher-Melkersson-Rosenthal syndrome, is an extremely rare neurological disorder, where the patient experiences recurrent facial weakness or paralysis/palsy, facial swelling and swelling of the lips, most often the upper lip. Patient often has recurrent attacks of this syndrome, which can range from several days to even years of time period in between the attacks. There is development of fissures and folds on the tongue. This disease manifests usually during childhood or in early adolescence. This condition is named after the physicians Dr. Ernst Melkersson and Dr. Curt Rosenthal.
Treatment consists of symptomatic treatment comprising of NSAIDs, steroids, antibiotics, immunosuppressants and surgery if needed.
Signs & Symptoms of Melkersson-Rosenthal Syndrome
- The three main characteristic symptoms of Melkersson-Rosenthal syndrome is recurrent facial swelling, relapsing paralysis of the face, and a cracked and furrowed tongue. Though all the patients may not develop this classic triad of symptoms and may only have one or two of the above symptoms.
- Orofacial swelling is the most recurrent and dominant symptom of Melkersson-Rosenthal syndrome.
- The orofacial swelling can be unilateral or asymmetric.
- Patient experiences pain in the affected region along with redness.
- Other structures, such as palate, gingiva, larynx and pharynx can also be involved.
- Patient often has recurrent attacks of Melkersson-Rosenthal syndrome, which can range from several days to even years of time period in between the attacks.
- There is edema on the face of the patient which is firm to touch.
- The tongue becomes hard and cracked with fissures and folds on it.
- The swelling remains constant and persists and slowly increases and gradually becomes a permanent feature in a patient's life.
- The affected lip becomes hard to touch and is fissured and cracked with a reddish-brown discoloration.
Causes for Melkersson-Rosenthal Syndrome
The exact cause of Melkersson-Rosenthal syndrome is not known; however, according to the research, a genetic link may predispose a patient to this condition. Melkersson-Rosenthal syndrome can also indicate the development of sarcoidosis or Crohn's disease. Certain ethnic groups in Bolivia are more prone to develop this syndrome.
Treatment for Melkersson-Rosenthal Syndrome
- Symptomatic treatment is commonly done for Melkersson-Rosenthal syndrome and includes medications, such as NSAIDs (Nonsteroidal Anti-Inflammatories) and corticosteroids. These medicines help in reducing the swelling.
- Antibiotics are given in case of infection.
- Immunosuppressants are also prescribed by some doctors for Melkersson-Rosenthal syndrome.
- Surgery for Melkersson-Rosenthal syndrome is done to relieve the pressure on the facial nerves, which in turn relieves the pain if any. Surgery is also done to excise the swollen tissue to correct any disfiguration to the face; however, the effectiveness of this procedure has not been established yet.
- Electrical stimulation and massage can also be done for Melkersson-Rosenthal syndrome.
- Melkersson-Rosenthal syndrome tends to recur intermittently after the first attack. It commonly becomes a chronic disorder. This syndrome should be closely followed as it can develop into Crohn's disease or sarcoidosis.
- Research is being done on neurological disorders like Melkersson-Rosenthal syndrome. The aim of the research is to increase knowledge as well as awareness of this disease and other rare neurological disorders, and to find ways to prevent, treat and cure them.