Moebius Syndrome - What It Is, Causes, Symptoms and Treatmentsand much more is what you will learn from now, so continue with us and find out everything about theMoebius Syndrome, a disease that is so dangerous.
In additionMoebius Syndromeis an extremely rare neurological disorder. It follows from the abnormal development of the cranial nerves, the death of several brain cells that are the muscles and the face and therefore has as main characteristic the total or partial loss of the movements of the muscles of the face, responsible for the expressions and motricity ocular
What is Moebius Syndrome:THEMoebius Syndrome(SM) was described by Moebius in 1892. It is characterized by congenital and non-progressive paralysis of the VII and VI cranial nerves (NC), almost always bilateral, which produces a little expressive facial appearance and convergent strabismus. Often, other NCs are compromised, uni or bilaterally, determining palpebral ptosis, divergent strabismus, deafness, sensitivity in the territories innervated by trigeminal, dysphagia, dysphonia and tongue atrophy, which can be verified in different combinations.
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A significant number of casesMoebius Syndromeis accompanied by mental deficiency suggesting that the involvement of the nervous system may be more diffuse than that indicated by the massive presence of CN paralyzes. Skeletal malformations are present in a large number of cases, especially crooked feet. Micrognathia and pectoral aplasia are also observed in association withMoebius Syndrome, constituting the denominated syndrome of Polland. Other abnormalities have been described, such as defects of the extremities, teeth, heart and central respiratory dysfunction, among others less common.
Causes of Moebius Syndrome:THEMoebius Syndromeoccurs randomly in the general population and patients generally have no family history ofMoebius Syndrome. Mutations of chromosome 13 were associated withMoebius Syndrome, as well as the mutations in the PLXND1 and REV3L genes..
The Rare Family PatternsMoebius SyndromeThey have been observed. The predominance ofMoebius Syndromeis approximately one in 25, 00 births, but family cases make up only 2% of all cases documented. In these cases, theMoebius Syndromeis inherited as an autosomal dominant trait. This means a single copy of an abnormal gene can causeMoebius Syndromebecomes, and can be passed on to the offspring.
Symptoms of Moebius Syndrome:In addition to the facial and abducent nerves, in some cases the glossopharyngeal and hypoglossal nerves may also be affected byMoebius Syndrome. Since each nerve is entrusted by a facial region, according to the nerve involved in each case the set of clinical manifestations changes. However, the most frequent are:
- Lack of facial expression (face in mask);
- Inability to smile;
- Converging strabismus;
- Absence of lateral eye movement and blinking;
- Eyelid fissure;
- Problems with closing the eyes, with consequent drying of the cornea;
- Muscle weakness in the upper body;
- Mandibular and maxillary hypoplasia;
- Tall, narrow palate;
- Incorrectly formed language;
- Alterations of the dental arch;
- Changes in speech;
- Hearing problems;
- Polydactyly and syndactyly;
- Primary myopathy;
- Umbilical hernia;
- Mental retardation;
- Congenital crooked feet;
- Flexor contracture of the knee and ankles.
Treatments for Moebius Syndrome:There is no curative treatment ofMoebius Syndrome. The affected child care requires a multidisciplinary team should include specialists in Pediatrics, Clinical Genetics and Dysmorphology, Trauma / Orthopedics, Speech Dentistry / Orthodontics, Otorhinolaryngology, Ophthalmology, Neurology, Physiotherapy and Psychology. In the medium and long term, the work of educators (school, sports, leisure activities ...) and psychologist, will be very important for social and labor integration..
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A Canadian Plastic Surgeon, Dr. Ronald M. Zuker, has developed a plastic surgery operation intervention called Zuker facial animation, which is achieved by people withMoebius Syndromeyou will be able to mobilize a small facial muscle that allows them to perform better lip closure and smile movement. The ideal age for this type of intervention seems to be between 5-6 years, but it can be done at any age..
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