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Moebius Syndrome

Medically Reviewed by Dr. Lakshmi Venkataraman, MD on Oct 05, 2017


What is Moebius Syndrome?

Moebius syndrome is a condition that affects the development of the cranial nerves VI and VII, either on one or both the sides of the head. It is named after the neurologist P. J. Moebius who studied the condition in 1888. Moebius syndrome is estimated to affect around 1 in 50,000 to 1 in 500,000 newborns. Both male and female children are equally affected.


Anatomy of the Cranial Nerves

There are twelve pairs of cranial nerves, among which the first two arise from the cerebrum, while the remaining arise from the brainstem. The nerves are either sensory, that is, they carry sensations to the brain; motor, that is, they control muscle movements; or mixed, which contain both sensory and motor nerves. They supply to the head and neck region, except the tenth (vagus) nerve and a few fibers of the XIth nerve, which also supply the chest and the abdomen.

The VI cranial nerve is a motor nerve that innervates the lateral rectus, the muscle that causes the eyeball to move laterally, that is, towards the outer corners of the eyes. The VII cranial nerve is a mixed nerve i.e. it carries both sensory as well as motor impulses. The sensory part carries taste sensations from the outer 2/3rd of the tongue, and soft and hard palate which form the roof of the mouth, to the brain. The motor part of the facial nerve controls the muscles of facial expression. When the facial muscles contract, various emotions are apparent on the face like smiling, frowning, or wrinkling the forehead. In addition, the facial nerve also carries parasympathetic nerve fibers that control the secretion of the salivary and mucous glands of the mouth and nose, and the tear secretion of the lacrimal gland.

Based on the extent of the disease, Moebius syndrome can be classified as:

What are the Causes of Moebius Syndrome?

The exact cause of Moebius syndrome is not known. Genetic and environmental factors have been implicated.

What are the Symptoms and Signs of Moebius Syndrome?

The features of Moebius syndrome are present since the birth. They include the following:

Facial paresis or paralysis: Weakness or paralysis of the facial muscles is the primary feature of Moebius syndrome. As a result, the baby:


These two features could interfere with the mother-baby bonding in the initial period after birth.

The muscles of the eyelids and around the eyes are also affected. Older children cannot raise their eyebrows. Incomplete closure of the eyelids could result in the eyes going dry and the cornea getting damaged due to excessive exposure.

The lack of facial expressions could interfere with social communication.

Problems with eye movements: Due to the paralysis of the lateral rectus muscle, the eye cannot turn outward. As a result, the child has to move the head while reading or following movements of objects. Squint may be present. The vertical movements of the eyes are not affected.

Involvement of other cranial nerves: Features due to involvement of other cranial nerves may be present which could include the 5th, 8 th, 9 th, 10 th, 11 th, and/or the 12 th nerves

Intelligence is usually normal. Though developmental delays may be noted around 1 year of age, the children tend to catch up by the time they are around 5 years of age.

Associated congenital abnormalities

Patients with Moebius syndrome often suffer from congenital malformations. These may include:

How do you Diagnose Moebius Syndrome?

The diagnosis of Moebius syndrome is based on the clinical features. Tests that may be advised include the following:


Imaging tests: CT scan and MRI may be carried out to rule out any other causes of the symptoms. They may also detect calcifications in the region of the nucleus of the VIth cranial nerve. The brainstem may be underdeveloped and the VIth and VIIth nerves will be either underdeveloped or absent. These tests are, however, not necessary for the diagnosis of Moebius syndrome.

How do you Treat Moebius Syndrome?

Early diagnosis and rehabilitation of the child are necessary to ensure that the child does not suffer from the long-term consequences of Moebius syndrome. The rehabilitation process should be modified as the child grows in line with the improvements showed by the child.

Surgical Treatments

Severe cases may require surgery. Surgical treatments to manage the facial paralysis include the following:

Other deformities like the limb and jaw deformities may also require surgical treatment.

More severe cases may require surgery in the form of free-muscle transplant innervated with motor nerves.

References:

  1. Moebius Syndrome - (https://ghr.nlm.nih.gov/condition/moebius-syndrome)
  2. Picciolini O, Porro M, Cattaneo E, Castelletti S, Masera G, Mosca F, Bedeschi MF. Moebius syndrome: clinical features, diagnosis, management and early intervention. Italian Journal of Pediatrics 2016; 42:56 https://doi.org/10.1186/s13052-016-0256-5
  3. Magnifico M, Cassi D, Kasa I, Di Blasio M, Di Blasio A, Gandolfini M. Pre- and Postsurgical Orthodontics in Patients with Moebius Syndrome. Case Reports in Dentistry 2017, Article ID 1484065, https://doi.org/10.1155/2017/1484065

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