Moebius syndrome is an uncommon disorder which is typified by paralysis of multiple cranial nerves, usually the 6th and 7th nerves. Other cranial nerves may get afflicted too. The condition is congenital. It tends to develop randomly in most cases; on the other hand, in some cases a genetic component can be traced.
Signs and symptoms of moebius syndrome are:
- Facial paralysis which usually afflicts both sides of the face – 7th cranial nerve is affected.
- Paralysis of the lateral movement of the eyes – 6th cranial nerve is afflicted.
- Seldom, other cranial nerves may get impaired too – 5th, 8th, 9th, 10th, 11th, and 12th.
- Infants drool excessively and exhibit strabismus. The eyes cannot move laterally, hence, the child is forced to turn his head to follow the object.
- The child has a mask like face, and cannot smile or frown.
- Corneal ulcers develop due to the fact that the eyelids stay open even during sleep.
- Some kids have a short, malformed tongue, a small jaw and a cleft palate; consequently there will be feeding and breathing problems.Some children may have hearing loss too. Dental anomalies are common as well. Some children may have speech difficulties and delays in speech development.
- Skeletal malformations are seen in over half of the children who have Moebius syndrome. Clubbed feet, poorly developed lower legs; webbing of fingers, absence of fingers, and underdevelopment of the hand are seen. Scoliosis of the spine is fairly common as well.
- Some show a delay in attaining the milestones – crawling or walking, this invariably occurs because of an upper body weakness; on the other hand, most children in due course catch-up. The syndrome is often related to a host of social and psychological consequences. No facial expressions and incapacity to smile makes an observer misinterpret what a Moebius syndrome individual is thinking or feeling.
What Are The Causes Of Moebius Syndrome?
Most cases occur arbitrarily without any cause and in the absence of a family history of the condition. A sporadic mutation in PLXND1 and REV3L genes has been recognized in a lot of patients. In some cases, familial patterns have been reported.
The cause for Moebius syndrome is multi-factorial, which means that environmental as well as genetic factors have a role to play.
In familial cases, Moebius syndrome is inherited as an autosomal dominant condition. Dominant genetic conditions develop in an individual when only one copy of an anomalous gene is enough for the condition to manifest. The anomalous gene may be inherited either from one of the parent, or may be due to new mutation.
Treatment Options For Moebius Syndrome
The treatment is directed towards the particular anomalies in the individual. Usually A multidisciplinary team is required to manage the case successfully. Specialists comprise of: neurologists, pediatricians; plastic surgeons; ear, nose, and throat specialists speech pathologists; dentists, audiologists, ophthalmologists and orthopaedics.
- To deal with facial paralysis, a temporalis tendon transfer is done; it involves lifting the temporalis muscle, which is for chewing; and moving it to the angles of your mouth. A similar surgical intervention is used to enhance the closure of eyelids. The smile surgery is also carried out in some people; it involves relocating of the gracillis muscle from the thigh to the face and joining the nerves that supply the masseter muscle. This surgery demonstrates noteworthy results in terms of speech, facial mobility and self-worth.
- Physical therapy is necessary for those who suffer from with orthopedic anomalies.
- Speech therapy is necessary for the affected children.
- Strabismus can be remedied surgically, though some experts advise delaying these procedures given that, the condition occasionally improves as the child grows.