Mobius syndrome in children

A congenital anomaly caused by abnormal structure of cranial nerves is Moebius syndrome. Let's consider its causes, symptoms, diagnostic and correction methods.

Neurological disorders account for a significant share of congenital pathologies. Moebius syndrome is characterized by the absence of facial reactions. The patient cannot smile, cry, or express other emotions. This disorder is rare and poorly studied. It is detected in one out of 150,000 newborns each year.

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

Q80-Q89 Other congenital anomalies (malformations).

• Q87 Other specified syndromes of congenital malformations involving multiple systems.

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

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Epidemiology

Moebius syndrome is a rather rare disease. Statistics indicate that it is diagnosed with a prevalence of 1 case per 150,000 newborns. Due to the pronounced symptoms (impaired 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 allow to minimize complications of the disease.

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Causes Moebius syndrome

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

• Atrophy of the cranial nerves due to vascular disorders during the embryonic period. Temporary interruption or change in blood supply, i.e. oxygen starvation during fetal development, leads to damage to the central nervous system of varying severity. This may manifest itself as anomalies in the child's growth or affect the development of its brain.
• Destruction or damage of cranial nerves due to external factors. These may include various infectious diseases of the mother, such as rubella, hyperthermia, generalized hypoxia, gestational diabetes, or drug use in the first trimester of pregnancy
• Various disorders and pathological processes in the body that provoke problems with the brain.
• Muscle pathologies causing degenerative lesions of the peripheral nervous system and brain.

This cranial nerve development defect manifests itself in various anomalies. Because of this, many patients experience unilateral or bilateral paralysis of the facial muscles. With it, facial expressions are completely absent or impaired. The person does not react to external stimuli, does not smile or frown. Babies with the syndrome have a poorly developed sucking reflex, they cannot swallow food normally. Without complex treatment, it leads to disruptions in social adaptation and normal life in society.

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Risk factors

According to the conducted studies, Moebius syndrome is multifactorial. That is, the risk factors for the development of this pathology are associated with both genetic and external causes.

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

Other possible factors of the disease:

• Oxygen starvation during intrauterine development.
• Various diseases and injuries during pregnancy.
• Taking narcotic substances or contraindicated medications in the early stages of pregnancy.
• Congenital hypoplasia.

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

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Pathogenesis

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

The disease is inherited in an autosomal dominant manner, but can also be autosomal recessive. It affects the 6th and 7th cranial nerves. Their incomplete development causes facial paralysis and disorders of the eye muscles. The other 12 pairs of cranial nerves can also be affected, each of which is responsible for the following processes in the body:

1. Olfactory nerve – allows you to perceive smells.
2. Optic nerve – transmits visual information.
3. The oculomotor nerve is responsible for the work of the external muscles of the eyeball.
4. Block - provides a connection between the superior oblique muscle of the eyeball and the central nervous system.
5. Trigeminal - responsible for the innervation of the chewing and facial muscles.
6. Abductor – lateral eye movement and blinking.
7. Facial - controls the facial muscles, contains the intermediate nerve, which transmits taste buds of the anterior third of the tongue, sensations of the skin and toes.
8. The vestibulocochlear plexus is responsible for hearing.
9. Glossopharyngeal – controls the swallowing process and is responsible for taste sensations.
10. Vagus nerve - carries sensory and motor signals to many organs, especially the chest and abdomen.
11. Accessory – responsible for the movement of the neck muscles, raising the shoulder, bringing the scapula to the spine.
12. Hyoid - responsible for tongue movements.

Congenital oculofacial paralysis is often manifested by underdevelopment of the 3rd, 6th, 7th, 9th, 12th pairs of cranial nerves. This may be due to the damaging effect of the cerebrospinal fluid, i.e. liquor, in the area of the 4th pair of nerves.

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Symptoms Moebius syndrome

Congenital neurological diseases are characterized by a complex of various disorders in many organs and systems. Symptoms of Moebius syndrome are most often associated with facial paralysis, serious problems with the musculoskeletal system and foot deformities. Other symptoms include:

• Speech and nutrition problems – patients have problems swallowing and chewing due to underdevelopment of the jaw muscles, tongue, throat and larynx. Problems with the speech apparatus are also observed.
• Dental problems – due to incorrect tongue movement, food accumulates behind the teeth, causing caries and other diseases. The syndrome is characterized by a cleft palate.
• Hearing problems – patients have decreased hearing. There is a high risk of its complete loss due to frequent ear infections that occur due to the anatomical features of the structure of the auricles.

People with the syndrome are born with micrognathia (small chin) and a small mouth, with a short or malformed tongue. The roof of the mouth has an abnormal opening, i.e. a cleft palate. Dental problems are also quite common - missing teeth or misaligned teeth. Such anomalies cause difficulties with eating, breathing and speech. Patients suffer from frequent respiratory diseases, sleep disorders and sensory integration dysfunctions.

There is also a lack of tear secretion and disturbances in the vestibular system. The patient's skin is taut, the corners of the mouth are always lowered. The anomaly is characterized by various pathologies of the limbs: clubfoot, extra fingers, syndactyly, etc.

First signs

The obvious first signs of Moebius syndrome are the absence of facial expressions. The disease is also characterized by the following symptoms:

• Anomalies in body structure and limited mobility.
• Partial or complete dysphagia (swallowing disorders).
• Difficulty sucking and breathing.
• Deformed feet.
• Sensory perception disorders.
• Ophthalmological and otolaryngological disorders.
• Tightness of facial skin.
• Vestibular disorders.
• Absence of tear secretion.
• Chest wall deformity (Poland syndrome).

As for intelligence, it may be lower in pediatric patients. But as they grow older, the patient's mental abilities do not lag behind their peers.

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Moebius syndrome in children

Small children are especially active in expressing their emotions, so a child with congenital oculofacial palsy is immediately noticeable. Moebius syndrome in children is manifested by facial immobility and its clear asymmetry. Patients do not react to external stimuli, do not cry, do not laugh, and do not even smile. Only by sounds can you understand that the child needs something. Babies have difficulty closing their eyes and their mouths are slightly open, head movements are difficult.

The genetic pathology is characterized by an autosomal recessive or, less commonly, dominant type of inheritance. The disorder is determined immediately after the baby is born, since his facial expressions are not natural. Other anomalies may also occur:

• Fusion or absence of fingers.
• Deformation of the auricles.
• Increased salivation.
• Difficulty sucking, swallowing and breathing.
• Weakness of the chewing muscles.
• Impairment of language functions.
• Difficulty in pronunciation.
• Underdevelopment or hypoplasia of the lower jaw.
• Corneal ulceration (eyelids remain half open during sleep).
• Strabismus.
• Epicanthus.
• Hearing impairment.
• Impaired lacrimation.
• Significant reduction in the size of the eyeball.

Timely diagnosis of the disease is very important. A comprehensive examination of the child's body allows you to identify various deviations and disorders, and make a plan for their correction. There are no radical methods for treating the syndrome. Children undergo symptomatic therapy, which consists of correcting strabismus, pronunciation disorders, etc.

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

There are currently no preventive measures to prevent this pathology. The prognosis for Moebius syndrome is favorable for life, since the disease is not prone to progression. With complex 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 the inability to express emotions cause certain difficulties in communicating with others.

Can you have Moebius syndrome without a cleft?

When a cranial nerve malformation is suspected, the question often arises whether Moebius syndrome can occur without a cleft. Against the background of this anomaly, skeletal lesions and pathologies from various body systems are observed, disrupting its normal functioning.

The formation of facial bones and their fusion occurs at 7-8 weeks of intrauterine development. When this process is disrupted, various facial pathologies occur. The development of the oral cavity occurs simultaneously with the formation of the nasal cavity. The facial skull consists of five processes - paired maxillary and mandibular, a single frontal. The upper palate is formed from outgrowths on the inner surface of the maxillary processes. Due to disruption of this process, the upper palate does not close and a cleft (cleft palate) is formed.

The upper palate consists of the processes and plates of the palatine bones, which are connected to each other by a suture. This part of the facial skull is an anatomical barrier separating the oral cavity from the nasal cavity. The palate consists of a hard and soft part.

Anatomical features of the upper palate:

• The hard palate is a bone formation made up of the palatine plates. Its formation occurs during the embryonic period. The plates grow together to form a single dome-shaped bone. However, due to genetic disorders, the bones are not formed correctly, i.e. a cleft is formed.
• The soft palate is formed by soft tissues, i.e. muscles (lingual, palatopharyngeal, palatoglossal, raising and tensing the soft palate) and the palatine aponeurosis. It has anterior and posterior parts. If the defect affects all layers of the palate, i.e. muscles and mucous membrane, then this is an open cleft. If only muscles are affected, then this is a hidden cleft of the soft palate.

This symptom of Moebius syndrome occurs not only due to genetic factors, but also due to the harmful effects of various viruses, bacteria and parasites on the fetus. Some diseases suffered by a woman during pregnancy have a teratogenic effect: chickenpox, cytomegalovirus, measles, herpes.

There are several forms of cleft palate, differing in the degree of non-closure of the upper palate:

• Cleft soft palate
• Cleft soft palate and part of the hard palate
• Unilateral cleft palate
• Bilateral lesion of the upper palate

Surgical intervention is necessary to treat the cleft. For a successful operation, coordinated action of a number of medical specialists is indicated: pediatrics, maxillofacial surgery, orthodontics, neurology, otolaryngology. Treatment is carried out in stages, the number of operations can exceed 5-7. Correction is indicated for pediatric patients from 3 to 7 years old.

If a cleft palate is left without surgical intervention, it leads to disruption of vital functions: problems with swallowing and sucking, speech and breathing disorders, gradual hearing loss, digestive disorders and dental diseases. Due to the pathology, food and liquid enter the nasal cavity. Therefore, patients are shown nutrition with the help of a special tube. As the child grows older, this defect becomes more pronounced, causing psychological disorders.

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Smile with Moebius syndrome

Congenital facial diplegia can be both bilateral and unilateral. A smile in Moebius syndrome is impossible without surgical correction. Facial muscle paralysis leads to the inability to express feelings through facial expressions. People with this syndrome are called "children without a smile." The pathology is complicated by the fact that patients are unable to recognize and interpret other people's emotions, since the relationship between the neural imitation system and the limbic system is disrupted.

The inability to smile is the most characteristic symptom of the syndrome. The cleft palate causes problems with feeding, as food enters the nasal cavity. Increased salivation and difficulty sucking are also observed.

In some cases, the congenital anomaly remains unnoticed for several weeks or even the first months of the child's life. But the inability to smile and the lack of facial expressions when crying sooner or later raises concern among parents. Many babies with the syndrome cannot completely close their mouths due to jaw and chin anomalies. Facial paralysis causes speech problems in 75-90% of cases. Patients are unable to pronounce sounds and letters normally, which complicates the socialization process.

Stages

The classification of Moebius syndrome was first proposed in 1979 by scientists who were studying this pathology. The stages of the disease are based on its pathological signs:

1. Simple hypoplasia or atrophy of cranial nerves.
2. Primary lesions of the peripheral central nervous system. Characterized by degenerative lesions of the facial nerves.
3. Focal necrosis in the nuclei of the brainstem. Microscopic damage to neurons and other brain cells.
4. Primary myopathy, both without and with CNS damage. Manifested by various muscle symptoms.

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

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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. However, this theory has not been confirmed, since developmental delays normalize as people grow older.
2. Skeletal system disorders – about 50% of patients have limb malformations. Most often, foot deformities, clubfoot, missing or fused fingers are observed. Some patients are diagnosed with scoliosis, underdeveloped chest muscles, and problems with the vestibular system.
3. Emotional and psychological symptoms – due to congenital facial paralysis, there are serious problems with communication and the development of social skills. Patients are prone 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 move the eyes normally, spatial orientation and object recognition are impaired.

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Complications and consequences

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

• Severe forms of paralysis.
• Contractures and synkinesis.
• Painful sensations.
• Ophthalmological and dental pathologies.
• Degenerative changes in the limbs.
• Disruption of the functioning of organs and systems.

Many patients are diagnosed with contractures due to increased muscle tone. Facial nerve paralysis causes severe tightening of the facial skin. Synkinesis may also be observed, that is, associated muscle movements - raising the corners of the mouth, wrinkling the forehead when closing the eyes, and much more.

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

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Diagnostics Moebius syndrome

To date, there is no specific algorithm for diagnosing Moebius syndrome. The disease is detected based on its characteristic symptoms. Facial nerve paralysis is one of the main symptoms of the syndrome. There are a number of specialized tests that allow excluding other causes of congenital facial diplegia.

A neurologist and a number of other specialists perform diagnostics. During the initial examination, the doctor collects a hereditary anamnesis and prescribes clinical urine and blood tests. A set of instrumental studies is carried out, including mandatory CT, MRI and electroneuromyography. Differential diagnostics are also indicated to compare the signs of the disease with other possible congenital pathologies.

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Tests

Laboratory diagnostics in case of suspected congenital neurological disorder is aimed at determining the general condition of the body and its various complications. Tests allow to detect hidden infectious or inflammatory lesions and a number of other pathologies.

Patients are prescribed general and biochemical blood tests, urine and stool tests. In the presence of inflammatory processes, an increase in the erythrocyte sedimentation rate, an increase in the number of leukocytes and a decrease in lymphocytes are observed. Such results allow us to suspect complications such as otitis, meningitis, tumor lesions and others.

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Instrumental diagnostics

To determine the extent of damage to the cranial nerves, instrumental diagnostics are indicated. Most often, patients are prescribed the following 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 determine areas of impaired blood circulation, tumors, brain hematomas and other pathologies.
• Electroneurography is the determination of the speed of propagation of an electrical signal along nerve fibers. Weak electrical impulses are used for stimulation, measuring their activity at different points. Moebius syndrome is characterized by a decrease in the speed of impulse conduction, the inability to transmit a signal to one of the nerve branches due to a rupture of the nerve fiber. If a decrease in the number of electrically excited muscle fibers is detected, there is a risk of muscle atrophy.
• Electromyography – this method is based on the study of electrical impulses that occur in muscles (without stimulation by electric current). During the procedure, thin needles are inserted into different areas of the muscle, determining the spread of pain impulses. The study is carried out with relaxed and tense muscles. It reveals damaged nerves, their atrophy and contractures.

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

Möbius syndrome on MRI

One of the most informative diagnostic studies for congenital oculofacial paralysis is magnetic resonance imaging. This method is based on the interaction of a magnetic field and hydrogen atoms. After a dose of radiation, the atomic particles release energy, which is recorded by ultra-sensitive sensors. Thanks 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, and abnormal development of blood vessels.

Moebius syndrome on MRI is manifested by hypoplasia, i.e. underdevelopment of the 6th and 7th pairs of cranial nerves. With the help of visualization, many patients are found to have an absence of the middle cerebellar stalks. Disruption of the 6th and 7th pairs of nerves is associated with craniofacial, musculoskeletal and cardiovascular defects, i.e. 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 correcting the pathological manifestations of the disease.

Differential diagnosis

Since birth injuries and a number of other pathologies can be mistaken for signs of Moebius syndrome, patients are shown differential diagnostics. The rare congenital anomaly is compared with the following diseases:

• Bulbar palsy.
• Traumatic injury of the facial nerve during childbirth.
• Respiratory paralysis.
• Down syndrome.
• Metabolic neuropathy.
• Neuromuscular pathologies.
• Brain syndromes.
• Poland Anomaly.
• Basilar artery thrombosis.
• Congenital muscular dystrophy.
• Focal muscular atrophy.
• Spinal muscular atrophy.
• Toxic neuropathy.
• Cerebral palsy.

To carry out differential diagnostics, a range of laboratory and instrumental studies are used.

Treatment Moebius syndrome

The best option for eliminating cranial nerve malformations is a comprehensive treatment of Moebius 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 palate (cleft palate), feeding newborns is significantly complicated. Children have impaired sucking and swallowing reflexes, so an alternative to breastfeeding is selected. For this, special tubes, syringes and feeding droppers are used.
2. Ophthalmological therapy – patients with the syndrome cannot blink normally and completely close their eyes during sleep. Because of this, there is a risk of corneal ulceration and increased dryness. Patients are prescribed special drops that eliminate inflammatory reactions. Tarsopharynx is also indicated – a surgical operation aimed at partially suturing the edges of the eyelids for normal blinking and protecting the cornea.
3. Dental and orthodontic treatment – due to a cleft palate, patients have an incorrect bite, the teeth are displaced or missing. There may also be problems with normal closure of the oral cavity, which causes various gum diseases and increased dryness of the lips. For correction, various orthodontic devices are used that allow the normal position of the teeth to be formed. In especially severe cases, jaw surgery is performed.
4. Restoration of facial expressions and speech – a complex surgical intervention is performed to minimize the symptoms of facial nerve paralysis. The operation is performed at an early age so that as the child grows up, he does not experience problems interacting with the outside world. Particular attention is paid to the correction of pronunciation, since there are problems with speech due to the anatomically incorrect position of the tongue.
5. Physical therapy – prescribed to patients with various orthopedic deviations. Most often, corrective surgeries are performed for foot deformities, clubfoot, hand and finger anomalies, and chest underdevelopment.
6. Psychological support – working with a psychologist/psychotherapist is necessary for both those with the syndrome and their family members. Mental health professionals help cope with feelings that cannot be fully expressed due to facial paralysis. The doctor works on the patient’s self-esteem, helps find constructive ways to talk about their problem in society.

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

Medicines

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

The doctor selects the medications individually for each patient. When choosing a medication, the patient's age, stage of Moebius syndrome and other features of the disease are taken into account. Very often, patients are prescribed the following medications:

1. Cerebrolysin

Improves the metabolism of brain tissue. Contains peptides and biologically active amino acids, which are the main components of brain metabolism. The active substances of the drug penetrate the blood-brain barrier, that is, through the blood and brain tissue. Improves the transmission of nerve 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 increased lactic acid content. Protects brain cells, increases the survival of neurons in the presence of insufficient oxygen supply and other damaging effects.

• Indications for use: diseases with disorders of the central nervous system, cerebrovascular accidents, brain surgery, mental retardation in children. Various psychiatric pathologies with symptoms of absent-mindedness and memory loss.
• Method of administration: in case of cerebrovascular accidents, after severe craniocerebral injuries or neurosurgical operations, the medicine is administered intravenously by drip, 10-30 ml, diluted in 100-200 ml of isotonic sodium chloride solution. The medicine is administered over 60-90 minutes. The course of treatment is 10-25 days. In case of mild disease, the medicine is administered intramuscularly, 1-2 ml for 20-30 days.
• Side effects: decrease or increase in body temperature, temporary feeling of heat at the injection site.
• Contraindications: pregnancy, allergic reactions, severe liver dysfunction.

The drug is available 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, and improves cognitive abilities. The active substances activate reparative processes in the central nervous system, and have a cerebroprotective and anticonvulsant effect. The drug promotes rapid restoration of central nervous system functions after various stressful effects.

Cortexin regulates the content of serotonin and dopamine. Restores the bioelectrical activity of brain cells and GABA-ergic influence.

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

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

3. Dibazol

A drug from the pharmacotherapeutic group of peripheral vasodilators. It has vasodilatory and antispasmodic properties, reduces blood pressure. 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, flaccid paralysis syndrome. The drug is effective in hypertension, spasms of the smooth muscles of internal organs and intestinal colic.
• The method of administration and dosage depend on the form of the drug. For parenteral administration, 2.5-10 mg of the drug is used. Tablets are prescribed for patients over 12 years old at 20-50 mg 2-3 times a day. The maximum single dose is 50 mg, the maximum daily dose is 150 mg.
• Side effects: arterial hypotension, dizziness, skin allergic reactions. With local administration, pain at the injection site is possible.
• Contraindications: individual intolerance to the components of the drug, severe renal dysfunction, gastric ulcer and duodenal ulcer, gastrointestinal bleeding. Diabetes mellitus, severe heart failure, pregnancy and lactation.
• Overdose: headaches and dizziness, development of a feeling of heat, nausea, increased sweating, arterial hypotension. There is no specific antidote, symptomatic therapy, gastric lavage and intake of enterosorbents are indicated.

Dibazol is available in the form of tablets, 10 pieces per package, and as a solution for injections of 1.5 ml of the drug in ampoules, 10 ampoules per package.

4. Nivalin

Cholinesterase inhibitor with the active substance – galantamine. Improves the conduction of impulses between neuromuscular synapses. Increases excitation of reflex zones of the spinal cord and brain. Stimulates and tones the contraction of skeletal muscles. Enhances the secretion of sweat glands and digestive glands, restores the functioning of muscle tissue. Reduces intraocular pressure.

• Indications for use: pathologies of the nervous system and myoneural synapse, muscular dystrophy, neuritis, myasthenia, cerebral palsy. The use of the drug is effective in cases of spinal cord injury, after poliomyelitis, myelitis, and also in cases of spinal muscular atrophy.
• The method of administration depends on the form of the drug. The solution is administered intramuscularly and intravenously. In case of 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, urticaria, itching, anaphylactic shock. Headaches and dizziness, nausea and tremor of the extremities, constriction of the pupils. Insomnia, bronchial spasms, diarrhea, increased peristalsis, abdominal pain, and frequent urination may develop during therapy. In rare cases, increased production of nasal and bronchial secretions is observed.
• Contraindications: allergic reactions to the components of the drug, bronchial asthma, bradycardia, angina, heart failure, severe renal and hepatic dysfunction, pregnancy and lactation. The solution is not prescribed for children under 1 year old, and tablets under 9 years old.
• Overdose: nausea and vomiting, abdominal colic, hypotension, diarrhea, bronchial spasms, convulsions and coma, bradycardia.

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

5. Tivortin

A medicinal product with an active substance - an amino acid from the category of partially synthesized in the body. Arginine is a regulator of intracellular metabolic processes, affecting the vital functions of organs and tissues. It has antioxidant, antiasthenic and membrane-stabilizing properties.

• Indications for use: acute cerebrovascular accident and its complications, atherosclerotic changes in the cerebral vessels with signs of hypoxia, various metabolic disorders, asthenic conditions, decreased function of the thymus gland, liver and respiratory diseases. Gestosis during pregnancy, preeclampsia.
• Method of administration: the solution is administered intravenously by drip at an initial rate of 10 drops per minute. The daily dosage is 4.2 g, i.e. 100 ml after dilution with isotonic solution. When administered orally, a single dose is 1 g, the maximum daily dose is 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, patients under three years of age, severe renal and hepatic insufficiency. Use of the drug during pregnancy is possible only on medical prescription.
• Overdose: increased sweating, weakness, anxiety, tachycardia, tremor of the extremities. Allergic reactions and metabolic acidosis may also develop. To eliminate such conditions, it is necessary to stop taking the drug and conduct desensitizing therapy.

Tivortin is available as a 4.2% solution for infusion in 100 ml vials.

6. Taufon

Prescribed for ophthalmological complications of Moebius syndrome. A medication with a sulfur-containing amino acid, which under normal conditions is formed during the transformation of cysteine. Improves energy processes, has neurotransmitter properties, inhibits synaptic transmission. Has anticonvulsant properties, stimulates reparative processes in dystrophic conditions in the body.

Indications for use: tissue malnutrition, retinal lesions, corneal dystrophy, increased intraocular pressure. The drug is used as an instillation of 2-3 drops in each eye 2-4 times a day for 2-3 months. No side effects, contraindications or signs of overdose have been identified. Taufon is available as a 4% solution in 5 ml vials and 1 ml ampoules.

The use of drugs should take place under strict medical supervision and only as part of complex therapy for neurological pathology.

Vitamins

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

For the well-coordinated functioning of the brain and the health of the nervous system, the following vitamins are recommended:

• A is a fat-soluble substance that protects cells from the negative effects of free radicals. Increases brain activity, normalizes sleep, and improves concentration.
• C – ascorbic acid increases the protective properties of the immune system and protects cells from free radicals. Improves brain activity and the functioning of the nervous system. Improves mood, helps cope with stress and nervous tension.
• 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. Protects the brain from Alzheimer's disease, improves memory and mood.

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

• B1 is an antidepressant, has strengthening, calming and restorative properties. It has a positive effect on mental abilities and cognitive functions, reduces anxiety.
• B2 – participates in cell synthesis, maintains vascular tone and elasticity, improves sleep. Relieves fatigue, weakness and combats frequent mood swings.
• B3 – improves cerebral blood supply and microcirculation in the brain vessels, restores energy metabolism in cells. Improves memory and attention, relieves chronic fatigue.
• B5 – regulates and maintains normal communication between neurons. Prevents the development of depression, increased fatigue and sleep problems.
• B6 – participates in the production of serotonin. Necessary for the absorption of essential amino acids for normal functioning of the brain. Increases intelligence, improves sleep, memory and attention.
• B9 – takes an active part in the functioning of the nervous system (inhibition, excitation). Increases the protective properties of the body, is necessary for maintaining 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.
• B12 – ensures cognitive function of the brain, maintains normal sleep-wake cycles. Minimizes increased irritability, improves memory and sleep.

Microelements to strengthen the nervous system and maintain normal functioning of the brain:

• Iron – supports and regulates brain function. Improves concentration, promotes quick reactions.
• Iodine – has a positive effect on hormone levels, improves memory.
• Phosphorus – maintains normal functioning of the brain, central nervous system and the entire body as a whole.
• Magnesium – maintains normal functioning of the muscular system at all levels.
• Potassium is responsible for the interaction of nerves with muscles.
• Calcium is responsible for the normal transmission of nerve impulses from muscles to the nervous system.

You can provide your body with useful vitamins and minerals with a balanced diet. The most useful products for neurological disorders are: dairy, meat (pork, beef, liver, chicken) and eggs, seafood, cereals (wheat, buckwheat, oats), fruits (citrus fruits, avocado, strawberries, bananas), vegetables (legumes, spinach, greens, tomatoes), nuts, fish oil. There are also ready-made vitamin complexes that can be purchased at the pharmacy. For children, the following drugs can be prescribed: Multitabs, Pikovit, Alphabet, Vitrum and others.

Physiotherapy treatment

Physiotherapy is a mandatory part of the complex therapy of congenital facial diplegia. Physiotherapy is a field of medicine that studies the therapeutic effect of natural and artificially created physical factors on the body.

This treatment significantly improves the central regulation of vital functions of the body. Normalizes conductivity and excitability of the neuromuscular apparatus, the central nervous system and increases energy resources. Metabolic processes are also accelerated, pathological autoimmune shifts are corrected, blood supply, trophism and tissue microcirculation 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 the pathology, the physical and mental state of the patient, age and a number of other factors. Most often, with Moebius syndrome, patients are prescribed the following physiotherapy procedures:

• Ultrasound therapy

The therapeutic effect of the procedure is based on mechanical micromassage of tissues and cells. There is an energetic effect of ultrasound waves on the body with the formation of heat and physical and chemical effects. Ultrasound acts as a catalyst for biochemical, physical and chemical reactions and processes in the body. The procedure is combined with drugs that penetrate 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, injuries, 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 medicinal product into the body using a certain frequency of the ultrasound field. This method is effective both at the initial stage of the disease and in its advanced course. The drugs used enhance the regeneration processes of damaged tissues.

• Electrosleep

Based on the reflex effect of current on the brain. The mechanism of action is associated with irritation of the reflexogenic zone of the eye sockets and upper eyelid. The irritation is transmitted along the reflex arc to the thalamus and cerebral cortex. The procedure restores vegetative, humoral and emotional balance. Electrosleep is effective in conditions after craniocerebral injuries, neurological diseases, neurosis, cerebral atherosclerosis, vegetative-vascular dystonia, hypertension.

• Magnetic therapy

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

Magnetotherapy has antispasmodic, vasodilatory, tonic and immunomodulatory properties. The procedure is prescribed for disorders of microcirculatory processes in the body, inflammatory diseases in their acute phase, pathologies of the peripheral nervous system, hypertension, vegetative-vascular dystonia.

• Local darsonvalization

This is a method of influencing the body using weak pulsed alternating current of medium frequency and high voltage. The procedure irritates the 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 disinfectant effect due to the destruction of the membranes of viruses and bacteria.

• Electromyostimulation (myoneurostimulation, myostimulation)

The mechanism of action of this method is based on electrical stimulation of nerves and muscles by transmitting current with specified 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 injuries.

• Massage

Mechanical action on damaged tissues with strengthening and restorative properties. Stimulates blood supply. Prescribed for neurological diseases, orthopedics and other pathological conditions.

• Therapeutic physical training (LFK)

This is a complex of treatment methods for the recovery of the body after illnesses and for their prevention. Physical therapy improves blood supply and lymph circulation, stimulates regeneration processes and the passage of nerve impulses.

The high efficiency of physiotherapy treatment for neurological disorders is based on the processes of regeneration of nerve tissue, improvement of its conductivity, minimization of pain and swelling. Physiotherapy is contraindicated in tumor lesions of the brain and peripheral nerves, infectious diseases of the central nervous system, epilepsy with frequent attacks, psychosis.

Folk remedies

The developmental defect of cranial nerves requires a serious and comprehensive medical approach. Folk treatment of Moebius syndrome is possible only to minimize certain pathological symptoms and only with the appropriate medical permission. The following procedures are indicated to eliminate facial paralysis:

• Take a glass of table salt or clean sand and heat it well in a frying pan. Put everything in a bag made of thick fabric. Apply it to warm up the affected tissues every day before going to bed. The course of treatment should be at least 1 month. Heat accelerates the restoration of damaged nerves and improves the condition of muscle fibers.
• Every day before your first meal, drink ½ cup of fresh nettle juice, an hour later ½ cup of dandelion juice, and an hour later ½ cup of celery juice. An hour after juice therapy, you should have breakfast. The procedure is performed once every 10-15 days. Plant juices help remove residual metabolic products from the body that accumulate in large quantities, stimulate metabolic processes, purify the blood, and activate digestive functions.
• Birch sap has increased biological value in neurological disorders. It is necessary to take ½ glass of such sap daily. It has calming properties, normalizes metabolic and energy processes in the body.
• Take 50 ml of motherwort, peony, calendula and hawthorn tinctures. This herbal remedy normalizes the state of the nervous system. The tincture should be taken 1 teaspoon for 2-3 months.

In addition to the above folk recipes, fir oil can be rubbed into the affected tissues, which has a warming effect and stimulates local blood circulation.

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Herbal treatment

Another alternative approach to treating congenital facial diplegia is herbal treatment.

• Buy a 10% solution of mumiyo for rubbing at the pharmacy. Apply a small amount of the product to a cotton swab and massage the affected area of the face. The massage should last at least 5 minutes, after which you should drink 20 mg of mumiyo dissolved in a glass of warm milk with a teaspoon of honey. Mumiyo has healing properties for peripheral nerves, strengthens the immune system. The duration of treatment is 10-14 days.
• For rubbing, you can use white acacia tincture. Pour 250 ml of vodka over 4-5 tablespoons of plant flowers and let it brew for 10 days. The finished product is used for rubbing 2 times a day for a month.
• Pour a glass of boiling water over a tablespoon of red rose petals. Let the mixture sit for an hour. Take 200 ml of the medicine 3-4 times a day. The course of treatment should be at least a month. Essential oils and microelements contained in the rose improve the functioning of the nervous system and the conductivity of nerve impulses.
• To reduce inflammation, frequent spasms and swelling of the affected tissues, it is recommended to use chamomile tea. Take a couple of teaspoons of dry chamomile, pour boiling water over it and let it brew until it cools. Drink the tea, and use the remaining plant material as compresses on the face under cellophane and a warm cloth.
• Take 2 tablespoons of black poplar buds and the same amount of butter. The plant part must be thoroughly crushed and mixed with butter. The finished ointment is applied to the skin for warming. Essential oils and resins contained in the buds have an analgesic and anti-inflammatory effect. The effect is noticeable after 5-7 days of therapy.

Severe facial paralysis requires long-term treatment. The first results of therapy will be noticeable in a couple of months.

Homeopathy

Elimination of symptoms of cranial nerve malformations with homeopathic remedies is used extremely rarely. Homeopathy is an alternative and rather controversial method, the effectiveness of which has not yet been confirmed by official medicine. Let's consider the remedies used for neurological disorders:

• Agaricus – stiffness and tension of the facial muscles, their twitching, itching and burning. Icy needles run along the nerves, painful sensations of a stabbing nature.
• Kalmia – neuralgic pains in the form of shooting pains with frequent paresthesias. Discomfort occurs in the tongue, jaws, and facial bones. The pains may decrease after eating.
• Cedron – periodic neuralgic pain around the eyes, in the nose. Discomfort increases at night and in a lying 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 radiating to the upper jaw and teeth. Local muscle paralysis, spasms.
• Mezereum – sharp shooting pains with lacrimation, numbness of certain parts of the body.
• Xantoxylum – shooting neuralgic pains that become more frequent during experiences and stress.
• Spigelia – sharp, acute pain that intensifies when touching the affected area. Pain in the eyeballs and eye sockets, can spread to the cheekbones, teeth, temples and cheeks.
• Viola odorata – tension of the scalp, pain localized above the eyebrows. Pulsating under the eyes and in the temples.

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

Surgical treatment

Congenital underdevelopment of the facial nerve nuclei requires complex therapy. Surgical treatment for Moebius syndrome is carried out using the following methods:

• Restoration of facial nerve function: decompression, neurolysis, suturing of damaged tissues, free graft plastic surgery.
• Restoration of facial muscle function using surgical intervention on the sympathetic nervous system.
• Reinnervation of facial muscles by suturing the facial nerve with other muscle fibers (hyoid, diaphragmatic).

The surgical intervention allows creating an artificial smile, facilitating the process of social communication with others. Such microsurgery is technically complex, as it consists of transplanting a graft from the thigh muscles to the face and can cause a number of complications.

In addition to creating a smile, multidisciplinary surgical therapy is aimed at correcting ophthalmological deviations, jaw and limb deformities. Before the operation, the patient undergoes a comprehensive diagnostic examination to assess the severity of the disorders and select the optimal treatment method.

Prevention

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

1. Planning pregnancy and improving the living environment.

• The optimal reproductive age is considered to be 20-35 years. Later or earlier conception significantly increases the risk of having a baby with chromosomal and congenital anomalies.
• If there is a high risk of hereditary diseases, one should refrain from having children. This is recommended for marriages between blood relatives and between heterozygous carriers of a pathological gene.
• Improvement of the living environment is necessary to prevent gene mutations caused by external factors. This is especially relevant for the prevention of somatic genetic pathologies (malformations, immunodeficiency states, malignant neoplasms).

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

• Healthy lifestyle and timely treatment of any diseases. Refusal of bad habits and minimization of nervous tension, stress. Maintaining a normal sleep and rest regime.
• Physical activity to maintain normal functioning of nerves, blood vessels and the entire body.
• Healthy nutrition, rich in vitamins and minerals for the proper functioning of all organs and systems.

In cases of high risk of having a child with abnormalities, termination of pregnancy is indicated. Recommendations for abortion are based on prenatal diagnostics. This is not the easiest method of prevention, but it prevents most severe and fatal genetic defects.

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Forecast

Moebius syndrome is a non-progressive malformation of the cranial nerve nuclei. The prognosis of the disease depends entirely on its symptoms, i.e. the severity of congenital defects. With a comprehensive approach to treatment, doctors minimize pathological symptoms, correct facial nerve paralysis, limb defects, cleft palate 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.