Möbius syndrome in children

A congenital anomaly caused by an abnormal structure of the cranial nerves is the Moebius syndrome. Consider its causes, symptoms, methods of diagnosis and correction.

Neurological disorders occupy a significant share among congenital pathologies. Moebius syndrome is characterized by the absence of mimic reactions. The patient can not smile, cry and express other emotions. This disorder refers to rare and poorly studied. Annually, it is detected in one of the 150,000 newborns.

The syndrome is included in the international classification of diseases of ICD-10 Class 10 - Congenital malformations (deformities), deformities and chromosomal abnormalities (Q00-Q99):

Q80-Q89 Other congenital malformations (malformations).

• Q87 Other specified syndromes of congenital anomalies (malformations) affecting several systems.

For the first time the disease was described in 1892 by a German psychiatrist and neurologist Paul Möbius. Pathology is systematically studied to this day, since its reliable causes have not been identified. Treatment, or rather, correction, consists of a variety of methods aimed at eliminating the various symptoms of the disease. With timely diagnosis and treatment, the patient has a chance for a normal social adaptation.

[1], [2], [3]

Epidemiology

Moebius syndrome is a fairly rare disease. Statistics indicate that it is diagnosed with a prevalence of 1 case per 150,000 newborns. Due to severe symptoms (violation or complete absence of facial expressions and sucking reflexes), complex treatment begins at an early stage of the disease. Modern microsurgical and conservative methods of correction make it possible to minimize complications of the disease.

[4], [5], [6]

Causes of the mobius syndrome

Many serious anomalies are formed in utero, transmitted at the genetic level or arising due to certain factors. The causes of Möbius syndrome, that is, the most severe form of paralysis of the facial nerves, are not fully known. The opinions of physicians and scientists were divided into such theories:

• Atrophy of the cranial nerves due to vascular disturbances in the embryonic period. Temporary interruption or change in blood supply, that is, oxygen starvation during fetal development, leads to damage to the central nervous system of varying severity. This can be manifested by abnormalities in the growth of a child or influencing the development of his brain.
• Destruction or damage to the cranial nerves due to external factors. It can be various infectious diseases of the mother, for example, rubella, hyperthermia, generalized hypoxia, gestational diabetes or the use of drugs in the first trimester of pregnancy
• Various disorders and pathological processes in the body, provoking problems with the brain.
• Muscular pathologies that cause degenerative lesions of the peripheral nervous system and brain.

This malformation of the cranial nerves manifests itself in various anomalies. Because of this, many patients experience unilateral or bilateral paralysis of facial muscles. With him completely missing or broken facial expressions. The person does not react to external stimuli, does not smile and does not frown. Children with the syndrome have poorly developed sucking reflex, they can not normally swallow food. Without comprehensive treatment, he leads to violations of social adaptation and a normal life in society.

[7], [8], [9]

Risk factors

According to the studies, Mobius syndrome is multifactorial. That is, the risk factors for the development of this pathology are related to both genetic and external causes.

In the case of a family history, the disease acts as an autosomal dominant trait. An abnormal gene can be inherited from both the mother and the father. The risk of transmission of an abnormal gene is 50% for each pregnancy and does not depend on the sex characteristics of the unborn child.

Other possible factors of the disease:

• Oxygen starvation during intrauterine development.
• Various diseases and injuries during pregnancy.
• Reception of narcotic substances or contraindicated drugs at early gestation.
• Congenital hypoplasia.

In addition to the above factors, the risk of development of pathology is associated with reduced protective properties of the immune system, the age of the woman (the older the mother is, the higher the risk of having a baby with pathologies), borne by viral and infectious diseases. In some cases, pathology was diagnosed in children whose mothers had rubella, flu or poisoning with quinine during pregnancy.

[10], [11], [12], [13]

Pathogenesis

The mechanism of development of Möbius syndrome is poorly understood. Pathogenesis is associated with vascular disorders during intrauterine development. The use of narcotic drugs, alcohol abuse and a number of other factors affect the appearance of a neurological disorder.

The disease is inherited by autosomal dominant type, but can also be autosomal recessive. Affects 6 and 7 cranial nerves. Because of their incomplete development, facial paralysis and impairment of the ocular musculature occurs. Other 12 pairs of cranial nerves can also be affected, each of which is responsible for such processes in the body:

1. Olfactory nerve - allows you to perceive odors.
2. Optical (visual) nerve - transmits visual information.
3. The oculomotor (oculomotor) nerve is responsible for the operation of the external muscles of the eyeball.
4. Block - provides the relationship between the upper oblique muscle of the eyeball and the central nervous system.
5. Triple - responsible for the innervation of masticatory and facial muscles.
6. Retractor - lateral movement of eyes and blinking.
7. Facial - controls the facial muscles of the face, contains an intermediate nerve that transmits the taste receptors of the anterior third of the tongue, sensations of the skin and toes.
8. The pre-old cochlear-is responsible for hearing.
9. Glossopharyngeal - controls the swallowing process, is responsible for the taste sensations.
10. Wandering - transfers sensory and motor signals to many organs, especially the thorax and abdomen.
11. Additional - responsible for the movement of the muscles of the neck, lifting the shoulder, bringing the shoulder blade to the spine.
12. Sublingual - responsible for the movement of the tongue.

Paralysis of the oculofacial congenital is very often manifested by maldevelopment of 3, 6, 7, 9, 12 pairs of cranial nerves. This may be due to the damaging effect of the cerebrospinal fluid, that is, the cerebrospinal fluid, to the nerves region of 4 pairs.

[14], [15], [16], [17], [18]

Symptoms of the mobius syndrome

Congenital neurological diseases are characterized by a complex of various disorders on the part of many organs and systems. Symptoms of Mobius syndrome are most often associated with paralysis of facial muscles, serious problems with the musculoskeletal system and deformity of the feet. Other symptoms include:

• Problems of speech and nutrition - in patients, the process of swallowing and chewing is broken because of the underdevelopment of the muscles of the jaw, tongue, throat and larynx. There are also problems with the speech device.
• Dental problems - due to incorrect movement of the tongue the food accumulates behind the teeth, provoking the development of caries and other diseases. The syndrome is characterized by a split sky.
• Hearing problems - hearing loss is observed in patients. There is a high risk of its complete loss due to frequent ear infections, arising from the anatomical features of the structure of the auricles.

People with the syndrome are born with a micrognathia (small chin) and a small oral cavity, with a short or irregularly formed tongue. The upper part of the oral cavity has an anomalous hole, that is, a split sky. Quite common and dental problems - the lack of teeth or the displacement of the dentition. Such anomalies cause difficulties with nutrition, breathing and speech. Patients suffer from frequent respiratory diseases, sleep disorders and sensory integration dysfunctions.

There is also a lack of tear secretion and impaired vestibular system. The patient's skin is stretched, the corners of the mouth are always lowered. Anomalies are characterized by various pathologies of the limbs: clubfoot, additional fingers, syndactyly and others.

First signs

The obvious first signs of Moebius syndrome are the absence of facial expression. Also for the disease characteristic of such symptoms:

• Anomalies in the structure of the body and limitation of mobility.
• Partial or complete dysphagia (swallowing disorders).
• Difficulty sucking and breathing.
• Deformed feet.
• Sensory perception disorders.
• Ophthalmic and otolaryngic disorders.
• Tightness of the skin of the face.
• Violations of the vestibular apparatus.
• Absence of tearful secretion.
• Chest deformation (Poland's syndrome).

As for the intellect, in children of age it can be lowered. But as he grows up, the mental abilities of the patient do not lag behind his peers.

[19], [20], [21]

Möbius syndrome in children

Young children express their emotions very actively, so a child with congenital oculofacial paralysis is immediately noticeable. Mobius syndrome in children is manifested by the immobility of the face and its clear asymmetry. Patients do not react to external stimuli, do not cry, do not laugh and do not even smile. Only by the sounds you can understand that the child needs something. The little eyes close their toes and the mouth is slightly opened, the movements of the head are difficult.

Genetic pathology is characterized by an autosomal recessive or less dominant type of inheritance. The disorder is determined immediately after the birth of the baby, since his facial expressions are not natural. There may also be other anomalies:

• Fingling or missing fingers.
• Deformation of the auricles.
• Increased salivation.
• Difficulty sucking, swallowing and breathing.
• Weak musculature weakness.
• Violation of the functions of the language.
• Difficult pronunciation.
• Underdevelopment or hypoplasia of the lower jaw.
• Ulceration of the cornea (eyelids remain half-open during sleep).
• Strabismus.
• Epicanthus.
• Hearing impairment.
• Violation of tearing.
• Significant reduction in the size of the eyeball.

Very timely diagnosis of the disease. Comprehensive examination of the child's body allows you to identify various abnormalities and violations, make a plan for their correction. There are no radical methods of treating the syndrome. Kids are symptomatic therapy, which consists of correcting strabismus, pronunciation disorders, and more.

It is possible to perform a surgical operation to eliminate the defect of atrophied facial muscles. The operation allows you to partially restore the facial abilities and adapt in the society. At the same time, the disease is not completely cured, as the child grows up, its symptoms become softer. In rare cases, about 10% of children experience delays in both mental and mental development.

Preventive measures to prevent this pathology to date does not exist. The prognosis of Mobius syndrome is favorable for life, since the disease is not prone to progression. With a comprehensive correction and compliance with all medical recommendations, most children lead a normal life, despite the fact that the disorder is associated with various psychological and social complications. Violation of facial expressions and inability to express emotions causes certain difficulties in communicating with others.

Can Mobius syndrome be without a cleft?

If there is a suspicion of the developmental disorder of the cranial nerves, the question often arises whether Mobius syndrome can be without cleft. Against the background of this anomaly, there are skeletal lesions and pathologies on the part of different systems of the organism, which disrupt its normal functioning.

Formation of the bones of the face and their fusion occurs on the 7-8th week of intrauterine development. When this process is broken, various facial pathologies arise. The development of the oral cavity runs simultaneously with the formation of the nasal cavity. The facial skull consists of five processes - paired maxillary and mandibular, single frontal. The upper sky is formed from the outgrowths on the inner surface of the maxillary processes. Due to the violation of this process, the upper sky is not inflated and a cleft (wolf mouth) is formed.

The upper sky consists of appendages and plates of the palatine bones, which are joined together by a seam. This part of the facial skull is an anatomical barrier separating the oral cavity from the nasal cavity. The sky consists of a hard and soft part.

Anatomical features of the upper sky:

• The hard palate is bone formation from the palatine plates. Its formation occurs during the embryonic period. The plates are joined together, forming a single dome-shaped bone. But because of genetic disorders, bone formation is irregular, that is, a cleft is formed.
• The soft palate is formed by soft tissues, that is, muscles (lingual, lingo-pharyngeal, lingual, raising and straining the palatal curtain) and palatine aponeurosis. Has front and back. If the defect affects all layers of the sky, that is, muscles and mucous membranes, then this is an open cleft. If only the muscles are affected, then this is a hidden cleft of the soft palate.

This sign of Mobius syndrome arises not only because of genetic factors, but also because of the harmful effects on the fetus of various viruses, bacteria and parasites. Teratogenic effect is possessed by some diseases, transferred by a woman during pregnancy: chicken pox, cytomegalia, measles, herpes.

There are several forms of wolf mouth, differing in the degree of nezrascheniya upper sky:

• Cleft palate cleft
• Cleft soft and part of hard palate
• One-sided cleft of the upper palate
• Bilateral defeat of the upper palate

For the treatment of clefts, surgical intervention is necessary. For the successful operation, a coordinated action of a number of medical specialists is shown: pediatrics, maxillofacial surgery, orthodontics, neurology, otolaryngology. Treatment is carried out in stages, the number of operations can exceed 5-7 pieces. Correction is indicated for children from 3 to 7 years of age.

If you leave the cleft palate without surgery, it leads to disruption of vital functions: swallowing and sucking problems, speech and respiratory disorders, gradual hearing loss, digestive disorders and dental diseases. Due to pathology, food and liquid enter the nasal cavity. Therefore, the patient is shown food with a special tube. As a child grows up, this defect becomes more pronounced, causing psychological disorders.

[22]

Smile with Mobius syndrome

Congenital facial diplegia is both bilateral and one-sided. Smile with Möbius syndrome without surgical correction is not possible. Paralysis of the facial muscles leads to the impossibility of expressing one's feelings with the help of facial expressions. The owners of this syndrome are called "children without a smile." Pathology is complicated by the fact that patients are unable to recognize and interpret other people's emotions, as the relationship between the neural imitation system and the limbic system is broken.

Impossibility to smile is the most characteristic symptom of the syndrome. Because of the split sky, there are problems with nutrition, as food enters the nasal cavity. There is also increased salivation and difficulty sucking.

In some cases, the congenital anomaly remains unnoticed for several weeks, and even the first months of the child's life. But the inability to smile and the lack of facial expressions during crying cause parents' concerns sooner or later. Many babies with the syndrome can not completely close their mouths because of anomalies of the jaw and chin. Because of facial paralysis in 75-90% of cases, there are problems with speech. Patients are unable to properly pronounce sounds and letters, which complicates the process of socialization.

Stages

For the first time the classification of Möbius syndrome was proposed in 1979, by scientists who were engaged in the study of this pathology. The stages of the disease are based on its pathological signs:

1. Simple hypoplasia or atrophy of the cranial nerves.
2. Primary lesions of the peripheral central nervous system. It is characterized by degenerative lesions of the facial nerves.
3. Focal necrosis in the nuclei of the brain stem. Microscopic damage to neurons and other brain cells.
4. Primary myopathy, both without and with CNS involvement. It is manifested by various muscular symptoms.

During the diagnosis of atrophy of the motor nuclei of the cranial nerves, the stage of the disease state is determined. Based on this, a plan for future treatment and prognosis is prepared.

[23], [24], [25]

Forms

The classification of neurological pathology has certain types, which depend on its symptoms:

1. Cognitive impairment - most people with the syndrome have normal intelligence, but 10-15% are diagnosed with moderate mental retardation. Many scientists associate the disease with autism. But this theory has not been confirmed, as the growth retardation normalizes as it grows up.
2. Breaches of the skeletal system - about 50% of patients have developmental limbs. The most commonly observed deformities of feet, clubfoot, absence or fusion of fingers. Some patients are diagnosed with scoliosis, underdevelopment of the muscles of the chest, problems from the vestibular apparatus.
3. Emotional and psychological symptoms - due to congenital facial paralysis, serious problems arise with communication and development of social skills. Patients are susceptible to depressive disorders, have learning difficulties and speech disorders.

In addition to the above symptom complexes, developmental problems are observed. Due to the inability to normally move the eyes, there is a violation of the spatial orientation and recognition of objects.

[26], [27], [28]

Complications and consequences

According to medical statistics, in 30% of cases, Mobius syndrome causes serious consequences and complications. Most often, patients face such problems:

• Severe forms of paralysis.
• Contracture and synkinesia.
• Painful sensations.
• Ophthalmic and stomatological pathologies.
• Degenerative changes in the limbs.
• Violation of the functioning of organs and systems.

In many patients, contractures are diagnosed because of increased muscle tone. Paralysis of the facial nerves provokes a serious contraction of the facial skin. Also can be observed synkinesia, that is, friendly muscle movements - lifting the corners of the mouth, wrinkling the forehead when closing the eyes and much more.

In most cases, complications are associated with improper repair of damaged muscle fibers. In 60% of patients, partial or complete vision loss, incomplete eye closure and various dental problems are diagnosed.

[29]

Diagnostics of the mobius syndrome

To date, the diagnosis of Mobius syndrome has no specific algorithm. The disease is identified based on the symptomatology characteristic of it. Paralysis of the facial nerves is one of the main signs of the syndrome. There are a number of specialized tests to exclude other causes of congenital facial diplegia.

Diagnosis is done by a neurologist and a number of other specialists. At the initial examination the doctor collects a hereditary anamnesis, appoints clinical analyzes of urine and blood. A complex of instrumental studies, mandatory CT, MRI and electroneuromyography. Differential diagnostics are also shown to compare the signs of the disease with other possible congenital pathologies.

[30]

Analyzes

Laboratory diagnosis in case of suspected congenital neurological disorder is aimed at determining the general condition of the body and its various complications. Analyzes allow to reveal latent infectious or inflammatory lesions and a number of other pathologies.

Patients are assigned general and biochemical blood tests, urinalysis, feces. In the presence of inflammatory processes there is an increase in the rate of erythrocyte sedimentation, an increase in the number of leukocytes, and a decrease in lymphocytes. Similar results allow to suspect such complications as otitis media, meningitis, tumor lesions and other.

[31]

Instrumental diagnostics

Instrumental diagnostics is shown to determine the degree of damage to the cranial nerves. Most often, patients are assigned such studies:

• Computer tomography and magnetic resonance imaging of the brain - these diagnostic methods are based on the ability of tissues to partially absorb X-rays. Irradiation is carried out from several points to obtain reliable information. The procedure allows you to identify areas of impaired blood circulation, tumor neoplasms, brain hematomas and other pathologies.
• Electro-neurology is the determination of the speed of propagation of an electrical signal through nerve fibers. To stimulate, use weak electrical impulses, measuring their activity at different points. Mobius syndrome is characterized by a decrease in the speed of impulses, the inability to transmit a signal to one of the branches of the nerve due to a break in the nerve fiber. If a decrease in the number of muscle fibers excited by electricity is detected, then there is a risk of muscular atrophy.
• Electromyography - this method is based on the study of electrical impulses arising in muscles (without stimulation by electric current). During the procedure, thin needles are introduced into different parts of the muscle, which determine the spread of pain impulses. The study is conducted with relaxed and tense muscles. Detects damaged nerves, their atrophy and contractures.

Instrumental methods are aimed at assessing the condition of the facial muscles. If necessary, the status of other possibly abnormal nerves is examined, for example, a large ear or gastrocnemius. Based on the results of diagnosis, the doctor makes a plan for correcting the painful condition.

Mobius syndrome with MRI

One of the most informative diagnostic studies in congenital oculofacial paralysis is magnetic resonance imaging. This method is based on the interaction of the magnetic field and hydrogen atoms. After the dose of irradiation, the particles of the atom give up energy, which is fixed by ultrasensitive sensors. Due to this, it is possible to obtain a layered image of the brain and other organs. The procedure reveals tumor lesions, inflammatory processes in the membranes of the brain, abnormal development of blood vessels.

Moebius syndrome in MRI is manifested by hypoplasia, that is, underdevelopment of 6 and 7 pairs of cranial nerves. With the help of visualization in many patients, the absence of medium cerebellar stems is determined. Violation of 6 and 7 pairs of nerves is associated with craniofacial, musculo-skeletal and cardiovascular defects, that is, symptoms of the disease.

Magnetic resonance imaging fully demonstrates the abnormal structure of the brain stem caused by the syndrome. The results of the study make it possible to draw up a plan for the correction of pathological manifestations of the disease.

Differential diagnosis

Since birth trauma and a number of other pathologies can be mistaken for the signs of Mobius syndrome, patients are shown differential diagnosis. A rare congenital anomaly is compared with such diseases:

• Bulbar paralysis.
• Traumatic lesion of the facial nerve during childbirth.
• Respiratory paralysis.
• Down Syndrome.
• Metabolic neuropathy.
• Neuromuscular pathology.
• Brain Syndromes.
• Poland's anomaly.
• Thrombosis of the basilar artery.
• Congenital muscular dystrophy.
• Focal muscular atrophy.
• Dorsal muscular atrophy.
• Toxic neuropathy.
• Cerebral palsy.

To carry out differential diagnostics use a set of laboratory and instrumental studies.

Treatment of the mobius syndrome

The optimal option for eliminating the developmental defects of cranial nerves is the complex treatment of Mobius syndrome. Radical methods have not been developed to date. The main task of doctors is symptomatic therapy, that is, correction of vision, pronunciation and other pathologies.

1. Nutrition - due to the cleft of the upper palate (wolf mouth) feeding newborns is significantly complicated. Sucking and swallowing reflexes are disturbed in children, therefore an alternative to breastfeeding is chosen. To do this, use special tubes, syringes and nutritional droppers.
2. Ophthalmic therapy - patients with the syndrome can not normally blink and completely close their eyes during sleep. Because of this, there is a risk of ulceration of the cornea and its increased dryness. Patients are prescribed special drops that eliminate inflammatory reactions. Tarsopharyngeal surgery is also indicated, a surgical operation aimed at partial suturing of the eyelid margins for normal blinking and protection of the cornea.
3. Dental and orthodontic treatment - due to a cleft palate in patients with an incorrect bite, the dentition is displaced or absent. There may also be problems with normal closure of the oral cavity, which causes various gum disease and increased dryness of the lips. For correction, various orthodontic devices are used that allow the formation of a normal position of the teeth. In especially severe cases, jaw surgery is performed.
4. Restoration of facial expressions and speech - to minimize the symptoms of paralysis of the facial nerves, a complex surgical procedure is performed. The operation is carried out at an early age, so that as the child grows up, the child does not experience problems in interacting with the outside world. Particular attention is paid to the correction of pronunciation, because because of the anatomically incorrect position of the language there are problems with speech.
5. Physical therapy - is prescribed for patients with various orthopedic deviations. Most often, corrective operations are performed in the deformation of feet, clubfoot, abnormalities of the hands and fingers, and underdevelopment of the thorax.
6. Psychological support - work with a psychologist / psychotherapist is needed both to the owners of the syndrome and to members of their families. Mental health professionals help to cope with feelings that can not be fully expressed because of facial paralysis. The doctor works on self-evaluation of the patient, helps to find constructive ways to talk about his problem in society.

Moebius syndrome is treated by pediatricians, neurologists, plastic surgeons, otolaryngologists, orthopedists, psychologists and a number of other medical specialists.

Medications

Congenital maldevelopment of the cranial nerves requires a comprehensive approach to treatment. Drug therapy is used to improve the blood supply to the brain and restore the functioning of its atrophied areas.

Medicines are chosen by the doctor, individually for each patient. When choosing a medication, the age of the patient, the stage of Mobius syndrome and other features of the disease are taken into account. Very often, patients are prescribed such drugs:

1. Cerebrolysin

Improves the metabolism of brain tissue. Contains peptides and biologically active amino acids, which are the main constituents of brain metabolism. The active substances of the drug penetrate the blood-brain barrier, that is, through blood and brain tissue. It improves the transmission of nervous excitation and regulates intracellular metabolism.

Increases the efficiency of energy production in the body in the presence of oxygen, improves intracellular protein synthesis, minimizes acidification processes due to the increased content of lactic acid. It protects the brain cells, increases the survival of neurons with insufficient oxygen supply and other damaging actions.

• Indications for use: diseases with violations of the central nervous system, cerebral circulation disorders, surgical operations on the brain, mental retardation in children. Various psychiatric pathologies with symptoms of absent-mindedness and memory impairment.
• Method of application: for violations of cerebral circulation, after severe craniocerebral trauma or neurosurgical operations, the drug is prescribed intravenously drip by 10-30 ml, diluted in 100-200 ml of isotonic sodium chloride solution. The medication is administered within 60-90 minutes. The course of treatment is 10-25 days. With a mild course of the disease, the drug is administered intramuscularly at 1-2 ml for 20-30 days.
• Side effects: a decrease or increase in body temperature, a temporary feeling of heat at the injection site.
• Contraindications: pregnancy, allergic reactions, severe violations of the liver.

The drug is issued in ampoules of 1 and 5 ml with a 5% solution.

2. Cortexin

A polypeptide drug with a balanced composition of neurotransmitters and polypeptides. It has a tissue-specific effect on the cerebral cortex, reduces the toxic effect of neurotropic substances, increases cognitive abilities. Active substances activate the reparative processes in the central nervous system, have cerebroprotective and anticonvulsant action. The drug promotes rapid recovery of CNS functions after various stressful effects.

Cortexin regulates the content of serotonin and dopamine. It restores bioelectrical activity of brain cells and GABA-ergic effect.

• Indications for use: delay in psychomotor and speech development in childhood, cerebral palsy, impaired thinking, memory and learning disability, various autonomic disorders, asthenic syndrome, encephalopathy of various etiologies, cerebral circulation disorders, comprehensive treatment of epilepsy.
• Method of administration: The drug is intended for intramuscular administration. The contents of the vial are dissolved in 1-2 ml of the solvent (water for injection, 0.9% solution of sodium chloride or 0.5% solution of procaine). The procedure is carried out once a day, the course of treatment is 5-10 days. Dosage is calculated individually for each patient.
• Side effects: in rare cases, there are reactions of hypersensitivity. There have been no cases of overdose.
• Contraindications: allergic reactions to the components of the drug, pregnancy and lactation.

Cortexin is available in the form of a lyophilized powder in 10 mg vials for the preparation of an intramuscular solution.

3. Dibasol

A drug from the pharmacotherapeutic group of peripheral vasodilators. Has vasodilator and spasmolytic properties, reduces pressure. It improves the functioning of the spinal cord, restores the activity of the peripheral nervous system. The active substances stimulate the synthesis of interferon, providing a moderate immunostimulating effect.

• Indications for use: spasms of the smooth muscle layer of blood vessels, diseases of the nervous system, a syndrome of flaccid paralysis. The drug is effective in hypertension, spasms of smooth muscles of internal organs and intestinal colic.
• The method of administration and dosage depend on the form of the preparation. When parenteral administration, 2.5-10 mg of the agent is used. Tablets are prescribed for patients over 12 years of 20-50 mg 2-3 times a day. The maximum single dose of 50 mg, the maximum daily dose of 150 mg.
• Side effects: arterial hypotension, dizziness, skin allergic reactions. With local administration, pain can occur at the injection site.
• Contraindications: individual intolerance to the components of the drug, severe renal dysfunction, peptic ulcer of the stomach and duodenum, gastrointestinal bleeding. Diabetes mellitus, severe heart failure, pregnancy and lactation.
• Overdose: headaches and dizziness, development of a feeling of heat, nausea, increased sweating, hypotension. There is no specific antidote, symptomatic therapy, gastric lavage and reception of enterosorbents are indicated.

Dibazol is available in the form of tablets of 10 pieces per pack and in the form of a solution for injection of 1 5 ml of the drug in ampoules, 10 ampoules per package.

4. Nivalin

The cholinesterase inhibitor with the active substance is galantamine. Improves the conduct of pulses between neuromuscular synapses. Increases the excitation of the reflex zones of the spinal cord and brain. Stimulates and tones up the contraction of the skeletal musculature. Strengthens the secretion of sweat glands and glands of the digestive system, restores the functioning of muscle tissue. Reduces intraocular pressure.

• Indications for use: pathology of the nervous system and the myoneural synapse, muscular dystrophy, neuritis, myasthenia gravis, cerebral palsy. The use of the drug is effective for spinal cord injuries, after the transferred poliomyelitis, myelitis, as well as spinal muscular atrophy.
• The method of use depends on the form of release of the drug. The solution is administered intramuscularly and intravenously. With a short course of treatment, parenteral administration is possible. On average, the initial dose is 2.5 mg per day with a gradual increase. The maximum daily dosage is 20 mg.
• Side effects: hypersensitivity reactions, hives, itching, anaphylactic shock. Headaches and dizziness, nausea and tremors of the extremities, narrowing of the pupils. On the background of therapy, it is possible to develop insomnia, bronchial spasms, diarrhea, increased peristalsis, abdominal pain, frequent urination. In rare cases, an increase in the production of nasal and bronchial secretions is observed.
• Contraindications: allergic reactions to the components of the drug, bronchial asthma, bradycardia, angina pectoris, heart failure, severe impairment of kidney and liver, pregnancy and lactation. The solution is not prescribed for children under 1 year old, and tablets up to 9 years.
• Overdose: nausea and vomiting, colic in the abdomen, hypotension, diarrhea, bronchial spasms, convulsions and coma, bradycardia.

Nivalin is available in the form of oral tablets and a medicament solution.

5. Tivortin

A medicinal preparation with an active substance-amino acid from the discharge partially synthesized in the body. Arginine is a regulator of intracellular metabolic processes that affects the vital functions of organs and tissues. It has antioxidant, anti-asthenic and membrane-stabilizing properties.

• Indications for use: acute disturbance of cerebral circulation and its complications, atherosclerotic changes in cerebral vessels with signs of hypoxia, various metabolic disorders, asthenic conditions, decreased function of the thymus gland, liver and respiratory diseases. Pregnancy during pregnancy, pre-eclampsia.
• Method of application: the solution is administered intravenously drip with an initial rate of 10 drops per minute. The daily dosage is 4.2 g, that is, 100 ml after dilution with an isotonic solution. For oral administration, a single dose is 1 g, a maximum daily dose of 8 g. The duration of treatment is 10-14 days.
• Side effects: burning sensation at the injection site, headaches and dizziness, local inflammation of the vein at the injection site, various allergic reactions.
• Contraindications: hypersensitivity to the components of the drug, the age of patients under three years old, severe renal and hepatic insufficiency. Use of the drug during pregnancy is possible only for medical purposes.
• Overdose: increased sweating, weakness, anxiety, tachycardia, tremor of extremities. It is also possible to develop allergic reactions, metabolic acidosis. To eliminate such conditions it is necessary to stop taking the medicine and conduct desensitizing therapy.

Tivortin is available in the form of a 4.2% solution for infusions in 100 ml vials.

6. Taufon

It is prescribed for ophthalmologic complications of Mobius syndrome. Medication with a sulfur-containing amino acid, which under normal conditions is formed during the transformation of cysteine. It improves energy processes, has neurotransmitter properties, inhibits synaptic transmission. Possesses anticonvulsant properties, stimulates reparative processes in dystrophic conditions in the body.

Indications for use: disruption of tissue nutrition, damage to the retina of the eye, corneal dystrophy, increased intraocular pressure. The drug is used as installations for 2-3 drops in each eye 2-4 times a day, for 2-3 months. Side effects, contraindications and signs of an overdose have not been identified. Taufon is available in the form of a 4% solution in 5 ml bottles and 1 ml ampoules.

The use of medicines should take place under strict medical supervision and only as part of a comprehensive therapy of neurological pathology.

Vitamins

A rare congenital non-progressive abnormality with atrophy of the motor nuclei of the cranial nerves requires complex treatment. Vitamins with Möbius syndrome are prescribed from the first days of diagnosis and taken throughout life.

For the harmonious work of the brain and the health of the nervous system, such vitamins are recommended:

• A - fat-soluble substance, protects cells from the negative effects of free radicals. Increases brain activity, normalizes sleep, improves concentration of attention.
• C - ascorbic acid increases the protective properties of the immune system and protects cells from free radicals. Improves brain activity and the nervous system. It improves mood, helps to cope with stress and nervous overstrain.
• D - protects blood vessels from cholesterol, prevents oxygen starvation of the brain, improves memory. Minimizes nervousness.
• E - strengthens blood vessels and capillaries, protects the body from the effects of free radicals. Keeps the brain from Alzheimer's, improves memory and mood.

The most important for the normal functioning of the brain are vitamins B:

• B1 - antidepressant, has strengthening, soothing and restorative properties. Has a positive effect on mental abilities and cognitive functions, reduces anxiety.
• B2 - participates in the synthesis of cells, maintains the tone and elasticity of blood vessels, improves sleep. Relieves fatigue, weakness and struggles with frequent mood swings.
• В3 - improves cerebral blood supply and microcirculation in the vessels of the brain, restores energy metabolism in cells. Improves memory and attention, relieves chronic fatigue.
• B5 - regulates and maintains a normal connection between neurons. It prevents the development of a depressed state, increased fatigue and problems with sleep.
• B6 - is involved in the production of serotonin. It is necessary for the assimilation of essential amino acids for the normal functioning of the brain. Increases the level of intelligence, improves sleep, memory and attention.
• B9 - takes an active part in the functioning of the nervous system (inhibition, stimulation). It increases the protective properties of the body, it is necessary to maintain short-term and long-term memory, normal speed of thinking.
• B11 - increases the protective properties of the immune system. Strengthens and restores the functioning of the nervous system, brain, heart and muscle tissue. Reduces anxiety.
• В12 - provides cognitive function of the brain, supports a normal change of sleep and wakefulness. Minimizes the increased irritability, improves memory and sleep.

Microelements for strengthening the nervous system and maintaining the normal functioning of the brain:

• Iron - supports and regulates the work of the brain. It improves concentration of attention, contributes to the speed of reactions.
• Iodine - has a positive effect on the level of hormones, improves memory.
• Phosphorus - supports the normal functioning of the brain, central nervous system and the whole body.
• Magnesium - supports the normal operation of the muscular system at all levels.
• Potassium - responsible for the interaction of nerves with muscles.
• Calcium - responsible for the normal conduct of nerve impulses from the muscles to the nervous system.

Provide the body with useful vitamins and minerals can be through a balanced diet. The most useful for neurological disorders are milk, meat (pork, beef, liver, chicken) and eggs, seafood, cereals (wheat, buckwheat, oats), fruits (citrus fruits, avocados, strawberries, bananas), vegetables (beans, spinach, greens, tomatoes), nuts, fish oil. Also, there are ready-made vitamin complexes, which can be purchased at the pharmacy. For children, such drugs can be prescribed: Multitabs, Pikovit, Alphabet, Vitrum and others.

Physiotherapeutic treatment

The complex therapy of congenital facial diplegia necessarily includes physiotherapy. Physiotherapy is an area of medicine that studies the therapeutic effect on the body of natural and artificially created physical factors.

Such treatment significantly improves the central regulation of vital body functions. It normalizes the conductivity and excitability of the neuromuscular system, the central nervous system and raises energy resources. Also, metabolic processes are accelerated, pathological autoimmune changes are corrected, blood supply, trophism and microcirculation of tissues are improved.

The procedures are selected individually for each patient. The doctor takes into account the stage and severity of the disease, the history of pathology, the physical and mental condition of the patient, age and a number of other factors. Most often, in Mobius syndrome patients are prescribed such physioprocedures:

• Ultrasound therapy

The therapeutic effect of the procedure is based on mechanical micromassage of tissues and cells. There is an energy effect of ultrasound waves on the body with the formation of heat and physicochemical effects. Ultrasound acts as a catalyst for biochemical, physico-chemical reactions and processes in the body. The procedure is combined with medications that penetrate through histological barriers and have a therapeutic effect on pathological foci.

Ultrasound therapy is prescribed for pathological symptoms of the syndrome from the musculoskeletal system, peripheral nervous system, ENT pathologies, dental diseases, trauma, and gastrointestinal disorders. This method is contraindicated for patients under 5 years of age, with angina pectoris, arrhythmia, hypertension, thrombophlebitis.

• Phonophoresis

The procedure is based on the introduction of a medicament in the body with a certain frequency of the ultrasound field. This method is effective both at the initial stage of the disease, and when it is running. Applied drugs enhance the processes of regeneration of damaged tissues.

• Electrosleep

It is based on the reflex influence of the current on the brain. The mechanism of action is associated with irritation of the reflexogenic zone of the eye sockets and the upper eyelid. Irritation is transmitted through a reflex arc into the thalamus and cortex of the brain. The procedure restores the vegetative, humoral and emotional balance. Electrosleep is effective in conditions after traumatic brain injuries, with neurological diseases, neurosis, cerebral atherosclerosis, vegetovascular dystonia, hypertension.

• Magnetotherapy

A therapeutic procedure in which affected tissues are affected by an alternating low-frequency or constant magnetic field. This method is aimed at the activation of physicochemical processes in blood elements. Increases the rate of chemical reactions by 10-30%, increases the number of cytokines, prostaglandins and tocopherol in tissues. Reduces the excitability of neurons with spontaneous impulse activity.

Magnetotherapy has spasmolytic, vasodilating, toning and immunomodulating properties. The procedure is prescribed for the violation of microcirculatory processes in the body, inflammatory diseases in their acute phase, pathologies from the peripheral nervous system, hypertension, vegetovascular dystonia.

• Local darsonvalization

This is a method of affecting the body with a weak pulsed alternating current of medium frequency and high voltage. The procedure causes irritation of terminal areas of sensitive nerve fibers of the skin, changing their excitability and activating local blood circulation. Darsonvalization has a therapeutic effect on the vascular walls, provides a disinfecting effect due to the destruction of the envelopes of viruses and bacteria.

• Electromyostimulation (myoneurostimulation, myostimulation)

The mechanism of action of this method is based on electrical stimulation of nerves and muscles by transferring a current with given characteristics through electrodes from the myostimulator to the body. The procedure is prescribed for patients with diseases of the central and peripheral nervous systems, as well as for rehabilitation after trauma.

• Massage

Mechanical effect on the affected tissue with strengthening and restoring properties. Stimulates blood supply. It is prescribed for neurological diseases, orthopedics and other pathological conditions.

• Therapeutic physical training (LFK)

This is a complex of therapeutic methods for the recovery of the organism after the transferred diseases and for their prevention. LFK improves blood circulation and lymph circulation, stimulates the processes of regeneration and passage of nerve impulses.

High efficiency of physiotherapeutic treatment for neurological disorders is based on the processes of regeneration of nerve tissues, improvement of its conductivity, minimization of pain sensation, edema. Physiotherapy is contraindicated in case of tumor lesions of the brain and peripheral nerves, infectious diseases of the central nervous system, epilepsy with frequent attacks, psychoses.

Alternative treatment

The developmental disorder of the cranial nerves requires a serious and comprehensive medical approach. Alternative treatment for Mobius syndrome is possible only to minimize certain pathological symptoms and only with the appropriate medical clearance. To eliminate facial paralysis, the following procedures are shown:

• Take a glass of table salt or clean sand and reheat it well in a skillet. Put everything in a pouch of dense fabric. Apply it to warm the affected tissues daily before bedtime. The course of treatment should be at least 1 month. Heat accelerates the repair of damaged nerves and improves the condition of muscle fibers.
• Every day before the first meal, drink ½ cup of fresh nettle juice, in an hour ½ cup of dandelion juice and another ½ cup of celery juice. An hour after socoterapia, you should have breakfast. The procedure is carried out once every 10-15 days. Vegetable juices promote the removal of residual metabolic products from the body, which accumulate in large quantities, stimulate metabolic processes, purify the blood and activate the functions of digestion.
• Birch sap has an increased biological value for neurological disorders. It is necessary to take on a daily basis ½ cup of juice. It has calming properties, normalizes metabolic and energy processes in the body.
• Take 50 ml of tinctures of motherwort, peony, calendula and hawthorn. Such a plant remedy normalizes the state of the nervous system. Tincture should be taken 1 teaspoon for 2-3 months.

In addition to the above alternative recipes, it is possible to rub fir oil into the affected tissues, which has a warming effect and stimulates local blood supply.

[32], [33], [34]

Herbal Treatment

Another option of an unconventional approach to the elimination of congenital facial diplegia is herbal treatment.

• Acquire a 10% solution of mummy in the pharmacy for grinding. A small amount of the drug is applied to a cotton swab and massaging movements to treat the affected area of the face. Massage should last at least 5 minutes, after which you should drink 20 mg of mummy, dissolved in a glass of warm milk with a teaspoon of honey. Mumiye has medicinal properties in relation to peripheral nerves, strengthens immunity. Duration of treatment is 10-14 days.
• For grinding, you can use a white acacia tincture. 4-5 tablespoons of plant flowers, pour 250 ml of vodka and let it brew for 10 days. The finished product is used for grinding 2 times a day for a month.
• Spoon a tablespoon of petals of a red rose with a glass of steep boiling water. The product should be infused for an hour. The medicine is taken 3-4 times a day for 200 ml. The course of treatment should be at least a month. Essential oils and trace elements that are contained in the rose improve the work of the nervous system and the conductivity of nerve impulses.
• To reduce the inflammation, frequent spasms and swelling of the affected tissue, it is recommended to use chamomile tea. Take a couple of teaspoons of dried chamomile, pour boiling water and let it brew until it cools. Tea should be drunk, and the remaining vegetable raw materials used as compresses on the face under the cellophane and warm cloth.
• Take 2 tablespoons of black poplar buds and the same amount of butter. The vegetable part needs to be crushed thoroughly and mixed with butter. A ready-made ointment is applied to the skin for warming up. Essential oils and resins that are contained in the kidneys have an analgesic and anti-inflammatory effect. The effect is noticeable after 5-7 days of therapy.

The severe form of facial paralysis requires prolonged treatment. The first results of therapy will be noticeable in a couple of months.

Homeopathy

Elimination of the symptoms of the developmental cranial nerves with the help of homeopathic medicines is used extremely rarely. Homeopathy is an alternative and rather controversial method, the effectiveness of which has not yet been confirmed by official medicine. Consider the means used for neurological disorders:

• Agaricus - stiffness and tension of the muscles of the face, their twitching, itching and burning. In the course of nerves, ice needles, painful sensations of a prickling character run through.
• Kalmia - neuralgic pain in the form of lumbago with frequent paresthesias. Discomfort occurs in the area of the tongue, jaws, bones of the facial skeleton. Pain can decrease after eating.
• Cedron - periodic neuralgic pain around the eyes, in the nose. Discomfort is worse at night and in a supine position.
• Verbascum - affects the lower branch of the trigeminal nerve, respiratory system, ears. Stops pain in the temporomandibular joint, painful lacrimation.
• Magnesium phosphoricum - sharp shooting pains, giving in the upper jaw and teeth. Local muscular paralysis, spasms.
• Mezereum - sharp painful lumbago with lacrimation, numbness of certain areas of the body.
• Xantoxylum - shooting neuralgic pain, increasing in experience and stress.
• Spigelia - sharp sharp pains that increase when you touch the affected area. Pain in the eyeballs and orbits, can spread to cheekbones, teeth, temples and cheeks.
• Viola odorata - the tension of the skull covers, the pain is localized above the eyebrows. Pulsates under the eyes and temples.

Before using the above drugs, you should consult a homeopath. The doctor will prescribe the appropriate remedy, its dosage and the duration of treatment.

Surgery

Congenital maldevelopment of the nuclei of the facial nerve requires complex therapy. Surgical treatment for Mobius syndrome is carried out by such methods:

• Restoration of the functioning of the facial nerve: decompression, neurolisis, stitching of damaged tissues, plastic with a free graft.
• Restoration of the functions of facial muscles with the help of surgical intervention on the sympathetic nervous system.
• Reinnervation of facial muscles with the help of sewing of the facial nerve with other muscle fibers (sublingual, diaphragmatic).

Operational intervention makes it possible to create an artificial smile, facilitating the process of social communication with others. Such microsurgery is technically difficult, as it consists of graft transplantation from the hip muscles to the face and can cause a number of complications.

In addition to creating a smile, multidisciplinary surgical therapy is aimed at correcting ophthalmic abnormalities, deformations of the jaw and limbs. Before the operation, the patient undergoes a comprehensive diagnostic examination to assess the severity of the disorders and choose the optimal method of treatment.

Prevention

Methods of preventing congenital genetic pathologies are based on pregnancy planning and prenatal diagnosis. Prevention of Mobius syndrome and other congenital neurological disorders consists of:

1. Pregnancy planning and habitat improvement.

• The optimal reproductive age is 20-35 years. More late or early conception significantly increases the risk of having a baby with chromosomal and congenital anomalies.
• At high risk of hereditary diseases, childbirth should be abandoned. This is recommended in marriages between blood relatives and between heterozygous carriers of the pathological gene.
• Improvement of the habitat is necessary to prevent gene mutations caused by external factors. This is especially important for the prevention of somatic genetic pathologies (malformations, immunodeficiency states, malignant neoplasms).

2. Reducing risk factors that increase the possibility of developing congenital anomalies.

• A healthy lifestyle and timely treatment of any diseases. Abandonment of bad habits and minimization of nervous overstresses, stresses. Compliance with normal sleep, rest.
• Physical activity to maintain the normal operation of nerves, vessels and the whole body.
• Healthy nutrition, rich in vitamins and minerals for the full functioning of all organs and systems.

At high risk of a birth of the child with deviations the abortion of pregnancy is shown. Recommendations for abortion are based on prenatal diagnosis. This is not the easiest method of prevention, but it prevents most of the severe and fatal genetic defects.

[35], [36], [37], [38], [39], [40]

Forecast

Moebius syndrome refers to the non-progressive malformation of the cranial nerve nuclei. The prognosis of a painful condition depends entirely on its symptoms, that is, on the severity of birth defects. With a comprehensive approach to treatment, doctors minimize pathological symptoms, correct paralysis of facial nerves, limb defects, split skies and other anomalies. Due to this, most patients with the syndrome lead a normal life, but may experience a number of difficulties in the process of socialization.