Arnold-Chiari syndrome.

Malformation (congenital anomaly), or Arnold-Chiari syndrome is a pathology that occurs at the stage of formation of the future child, that is, during the period of intrauterine development of the fetus. This defect is excessive compression of the brain due to the discrepancy in size or deformation of a certain cranial section. As a consequence, there is a displacement of the brain stem and cerebellar tonsils inside the foramen magnum, where their infringement is detected.

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Epidemiology

The pathology cannot be called too rare: the syndrome occurs in approximately 5 babies out of 100 thousand born.

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Causes Arnold-Chiari syndrome.

The exact causes of the anomaly have not yet been fully clarified. It is certain that the disease is not associated with chromosomal defects.

Moreover, many scientists do not recognize this syndrome as congenital, believing that the disease can occur in adults as well.

Thus, two categories of probable causes of Arnold-Chiari syndrome can be distinguished.

Congenital causes:

• the cranium undergoes changes during intrauterine development - for example, a reduced posterior cranial fossa is formed, which becomes an obstacle to the normal positioning of the cerebellum. There may also be other disturbances in the growth and development of the skeletal apparatus, which does not correspond in parameters to the brain;
• In utero, an excessively large occipital foramen magnum is formed.

Causes of acquired nature:

• trauma to the child's skull and brain during labor;
• cerebrospinal fluid damage to the spinal cord with stretching of the central canal.

In addition, the development of Arnold-Chiari syndrome is possible as a result of exposure to other factors or defects.

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Pathogenesis

In the etiology of the development of the syndrome, some generally accepted risk factors are distinguished. Thus, Arnold-Chiari syndrome in the fetus may appear due to the following reasons:

• independent or uncontrolled use of medications by a pregnant woman;
• consumption of alcoholic beverages by a pregnant woman, as well as exposure to nicotine;
• viral infections in women during pregnancy.

However, the exact step-by-step pathogenesis of the anomaly has not yet been established, which often complicates possible prevention of the disease.

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Symptoms Arnold-Chiari syndrome.

Arnold-Chiari syndrome in adults most often has type I anomaly. Symptoms characteristic of stage I of the disease appear simultaneously with permanent pain in the head:

• dyspepsia, nausea attacks;
• weakness in the arms, paresthesia;
• pain in the cervical spine;
• sensation of noise in the ears;
• unsteadiness when walking;
• diplopia;
• difficulty swallowing, slurred speech.

The first signs of stage II Arnold-Chiari syndrome appear immediately after the baby is born, or in infancy. Arnold-Chiari syndrome in a child is manifested by the following disorders:

• swallowing disorders;
• respiratory disorders, weakness of the child's cry, noisy breathing movements with a characteristic whistle.

The third stage of the disease is the most severe. Often, fatal cases can be observed as a result of a brain or spinal cord infarction. The third stage of Arnold-Chiari syndrome is characterized by the following signs:

• when turning the head, the patient experiences loss of vision or diplopia, sometimes dizziness and fainting;
• tremors and coordination disorders are present;
• loss of sensitivity in part or half of the body;
• the facial muscles, limbs and body muscles weaken;
• difficulties with urination appear.

Depending on the degree and symptoms, various types of treatment for the pathology are prescribed.

Stages

The degrees, or types, of the syndrome differ from each other in the structural features of the brain tissues trapped in the spinal canal, as well as the presence of disturbances in the formation of brain elements and the depth of the entrapment.

• In type I, the infringement occurs relatively low (in the cervical region), and brain functions are within normal limits.
• In type II, there is a displacement of the cerebellum into the foramen magnum, simultaneously with the presence of defects in the spinal cord and brain.
• Type III is characterized by an occipital hernia with complete displacement of the posterior brain structures into the enlarged foramen magnum. This degree of Arnold-Chiari syndrome has the worst prognosis and is fatal in most cases.

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Complications and consequences

1. Paralysis that results from pressure on the spinal structures.
2. Accumulation of fluid in the brain (hydrocephalus).
3. Formation of a cystic or cystic formation in the spine (syringomyelia).
4. Infarction of the brain or spinal cord.
5. Apnea, fatal outcome.

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Diagnostics Arnold-Chiari syndrome.

Tests for diagnosing Arnold-Chiari syndrome are usually uninformative. To clarify the diagnosis, a lumbar puncture with subsequent analysis of the cerebrospinal fluid may be performed.

Instrumental diagnostics of Arnold-Chiari syndrome consists of prescribing magnetic resonance imaging, which is performed in neurological clinics and departments. The MRI method allows examining the cervical, thoracic spine and cranium.

A large proportion of diseases are detected during an external examination: attention is paid to gait, the presence of sensitivity and other characteristic signs.

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Differential diagnosis

Differential diagnosis of Arnold-Chiari syndrome is carried out with secondary displacement of the cerebellar tonsils due to increased intracranial pressure caused by a tumor, extensive hematoma, etc.

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Treatment Arnold-Chiari syndrome.

If the patient does not express significant complaints other than intense pain, he is prescribed drug treatment with various combinations of non-steroidal anti-inflammatory drugs, nootropics and muscle relaxant drugs.

Medicines that are used to relieve the condition of a patient with Arnold-Chiari syndrome:

Meloxicam
Dosage of the drug	Prescribed orally 1-2 tablets per day.
Side effects	Dyspepsia, anemia, edema.
Special instructions	Not used to treat children under 15 years of age.

Piracetam
Dosage of the drug	Prescribed from 30 to 160 mg per kilogram of weight per day, approximately in three doses. The course of therapy is up to 2 months.
Side effects	Dyspepsia, anxiety, increased libido.
Special instructions	In case of insomnia, the evening dose of the drug is transferred to the afternoon dose.

Ibuprofen
Dosage of the drug	Take 200 mg up to 4 times a day.
Side effects	Abdominal pain, dyspepsia, tachycardia, allergic reactions, sweating.
Special instructions	Do not use to treat children under 6 years of age.

Mydocalm
Dosage of the drug	Prescribed individually at 50-150 mg up to 3 times a day.
Side effects	Feeling of muscle weakness, decreased blood pressure, dyspepsia, allergic reactions.
Special instructions	Not prescribed to children under 6 years of age.

Additionally, high doses of B vitamins are prescribed. These vitamins actively participate in most biochemical processes, ensuring normal functioning of the nervous system. For example, thiamine, which is found in neuron membranes, significantly affects the recovery processes of damaged nerve conduction pathways. Pyridoxine ensures the production of transport proteins in the axial cylinders, and also serves as a high-quality antioxidant.

Long-term intake of excessive doses of vitamins B1 and B12 is not accompanied by side effects. Taking vitamin B6 in an amount of more than 500 mg per day can cause the development of sensory polyneuropathy.

The most common vitamin remedy used for Arnold-Chiari syndrome is Milgamma, a drug that contains 100 mg of thiamine and pyridoxine, and 1000 mcg of cyanocobalamin. The course of treatment begins with 10 injections of the drug, then moves on to oral administration.

Physiotherapy has proven itself as an auxiliary method. Neurologists usually recommend the following procedures to patients:

• cryotherapy – activates the body’s regulatory systems, stimulates the immune and endocrine systems, relieves pain;
• laser therapy – improves microcirculation and tissue nutrition in the damaged area;
• magnetic therapy – helps to activate the body’s internal healing reserves.

Physiotherapy successfully complements drug treatment, which allows for more sustainable positive results.

Homeopathy is also successfully used to alleviate Arnold-Chiari syndrome. The main principle of homeopathic treatment is the use of extremely small doses of herbal remedies that act against the disease. Doses of homeopathic preparations are called "dilutions": they can be tithe or centesimal. As a rule, herbal extracts and, mostly, alcohol are used to make medicines.

Homeopathic remedies are used according to generally accepted rules: half an hour before meals, or half an hour after meals. Granules or liquid should be held in the mouth for resorption.

Aneurysm	Take 8-10 granules three times a day.	Relieves pain, soothes, promotes restoration of damaged nerve fibers.
Valeriana Heel	Take 15 drops three times a day.	Normalizes sleep, alleviates psychosomatic symptoms.
Neurosed	Take 8-10 granules three times a day.	Eliminates irritability, calms and smooths out the manifestation of neurotic reactions.
Vertigoheel	Take 1 tablet or 10 drops three times a day.	Eliminates dizziness, alleviates symptoms of traumatic brain injury.
Spigelon	Take 1 tablet three times a day.	Relieves pain and tension.

Homeopathic medicines are freely available for sale. There are virtually no side effects when taking them, but taking medications without consulting a doctor is strongly discouraged.

If drug therapy does not improve the dynamics of Arnold-Chiari syndrome, and symptoms such as paresthesia, muscle weakness, impaired visual function or consciousness remain, the doctor prescribes planned or urgent surgical treatment.

The most common surgical procedure for Arnold-Chiari syndrome is suboccipital craniectomy, which is the widening of the foramen magnum by sawing off an element of the occipital bone and removing the cervical vertebral arch. As a result of the operation, the direct pressure on the brainstem is reduced and the circulation of cerebrospinal fluid is stabilized.

After bone resection, the surgeon performs plastic surgery of the dura mater, simultaneously increasing the posterior cranial fossa. Plastic surgery is performed using the patient's own tissues - for example, the aponeurosis or part of the periosteum. In some cases, artificial tissue substitutes are used.

At the end of the operation, the wound is sutured, sometimes with the installation of titanium stabilizer plates. The need for their installation is decided on an individual basis.

As a rule, a standard operation lasts from 2 to 4 hours. The rehabilitation period is 1-2 weeks.

Folk remedies

Folk recipes for the treatment of Arnold-Chiari syndrome are primarily aimed at relieving pain and relaxing the muscles affected by spasm. Such treatment cannot replace traditional therapy, but it can effectively complement it.

• Pour 200 ml of hot water over 2 tbsp of marshmallow herb or rhizome, leave overnight. Use for compresses several times a day until the condition is relieved.
• Boil a chicken egg, peel it while hot, cut it in half and apply it to the sore spot. Remove when the egg has completely cooled.
• Apply compresses of pure natural honey.
• Brew 1 tbsp of fern with boiling water (200 ml), keep on low heat for up to 20 minutes. Cool and take 50 ml before each meal.
• Brew 1 tbsp of raspberry leaves with boiling water (200 ml), keep on low heat for up to 5 minutes. Cool and drink 5 tbsp three times a day before meals.

Herbal treatment helps to significantly improve the condition of a patient with Arnold-Chiari syndrome. In addition to pain, herbs normalize the functioning of the nervous system, improve mood and sleep.

• Take 1 tbsp. of dry raw materials of anise, basil and parsley, pour 700 ml of boiling water, leave for 2 hours and filter. Take 200 ml in the morning, afternoon and evening before meals.
• Pour 700 ml of water over an equal mixture of sage, thyme and fennel (3 tbsp). Infuse for up to 2 hours, filter and take a glass three times a day before meals.
• Brew a mixture of lemon balm, basil and rosemary (2 tablespoons each) in 750 ml of boiling water. Infuse and filter, then take 200 ml three times a day before meals.

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Kinesiological exercises

Kinesiological gymnastics is a special set of physical exercises that stabilize the human nervous system. Such exercises can be used to alleviate the condition of patients with Arnold-Chiari syndrome of the first degree. As studies have shown, by performing kinesiological gymnastics just once every 7 days, you can achieve an improvement in your perception of the world and well-being, relieve the effects of stress, eliminate irritability, etc.

In addition, the classes help to establish the synchronous work of the brain hemispheres, improve the ability to concentrate and remember information.

The course of exercises lasts one and a half to two months, 20 minutes a day.

• It is recommended to gradually speed up the tempo of gymnastic elements.
• It is advisable to do most of the exercises with your eyes closed (to increase the sensitivity of certain areas of the brain).
• Exercises involving the upper limbs are recommended to be combined with synchronous eye movements.
• During breathing movements, you should try to include visualization.

As practice has shown, kinesiology classes, in addition to developing nerve conduction, bring a lot of pleasure to patients.

Prevention

Due to the lack of necessary information about the etiology of Arnold-Chiari syndrome, it is quite difficult to determine specific prevention of the disease. All that can be done is to warn future parents about the need to lead a healthy lifestyle, as well as about the possible consequences of smoking and drinking alcohol.

To give birth to a healthy child, the expectant mother should adhere to the following recommendations:

• eat a complete and balanced diet;
• give up smoking and drinking alcohol;
• Do not self-medicate and strictly follow the advice and instructions of your doctor.

These rules must be followed not only if you are already pregnant, but also if you are planning to become pregnant.

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Forecast

Patients with the first or second degree of the disease can lead a normal life, in the absence of pronounced clinical symptoms. If problems of a neurological nature are observed, then such a patient is prescribed urgent surgical intervention (which, however, does not always allow for the restoration of some neurological functions).

The third degree of pathology in the overwhelming majority of cases ends in the death of the patient.

If Arnold-Chiari syndrome is ignored, the disorders will increase, gradually disabling parts of the spine, which sooner or later ends in paralysis.