Also known as Moebius syndrome, congenital facial diplegia is an extremely rare neurological disorder characterized by the inability to move eyes from side to side and facial paralysis. Born with complete facial paralysis, people with Moebius cannot form facial expressions or close their eyes.
Sometimes the lack of facial expression is taken as a sign of unfriendliness and dullness. The Moebius syndrome is not always affecting the person’s level of intelligence. Some studies reported a mild intellectual deficit in about 1% of the cases. The disorder is named after Paul Julius Moebius the neurologist who described the syndrome for the first time in 1988.
The syndrome is caused by the absence or underdevelopment of the 6-th and 7-th cranial nerves responsible with facial expressions and eye movement. There are a few other cranial nerves that might be affected as well such as the 12th, 11th, 9th, 8th, 5th, and the 3rd. The inability to suck is one of the first symptoms detected at birth.
Common symptoms might include excessive drooling, feeding, choking and swallowing problems, eye sensitivity, crossed eyes, lack of facial expression, motor delays, high or cleft palate, inability to smile, hearing problems and speech difficulties. Autism is also associated with the Moebius syndrome. Between 30% and 40% of children with Moebius syndrome are diagnosed with autism. Deformities of jaw, tongue and limbs occur sometimes in people with Moebius.
Four groups of Moebius syndrome were identified.
In the first group the brain stem nuclei responsible with the control of the cranial nerves are small or absent.
The second group defined by the degeneration and loss of neurons in the facial peripheral nerve.
The degeneration and loss in other brain cells and hardened tissue in the brainstem nuclei describes the third group.
The fourth group lacks lesions in the cranial nerves but the muscular symptoms are present.
The Moebius syndrome is poorly understood and the exact causes are not known yet. The scientists think it occurs during prenatal development and is the results of a vascular disruption in the brain. In a few families genetic links have been found although most cases do not seem to be genetic. For a btter understanding of the syndrome patients should look for the specialist assistance of a neurologist in aventura florida.
Lack of oxygen or interrupted or impaired blood flow to a developing fetus might occur due to maternal trauma. The Moebius syndrome could also be associated with the use of drugs like thalidomide or misoprostol during pregnancy. According to other researchers congenital agenesis or hypoplasia of the cranial nerve nuclei could be the underlying problem of the disorder.
There is no cure for the disease and no specific course of treatment. The disorder is not evolving and the treatment and the management is symptomatic. For a correct and sufficient nutrition feeding tubes and special bottles might be necessary to feed infants.
Strabismus can be corrected through surgery as well as jaw and limb deformities. Patients could get some level of mobility and ability to smile with muscle grafts. Speech and physical therapy could help patients with the syndrome improve their motor skills and coordination as well as their control over eating and speaking.