Arnold-Chiari Syndrome

Malformation (congenital anomaly), or Arnold-Chiari syndrome - is a pathology that occurs even at the stage of the future child's formation, that is, even during fetal development. This defect is an excessive squeezing of the brain due to a mismatch in the size or deformation of a certain cranial region. As a consequence, movement of the brain stem and cerebellar tonsils into the large occipital orifice is observed, where their infringement is detected.

[1], [2], [3], [4]

Epidemiology

Pathology can not be called too rare: the syndrome occurs in about 5 infants of 100 thousand people born.

[5], [6], [7]

Causes of the syndrome Arnold-Chiari

The reliable reasons for the anomaly have not yet been fully elucidated. Precisely, we can say that the disease is not associated with defects in chromosomes.

Moreover, many scientists do not recognize this syndrome as inherent, believing that the appearance of the disease is possible, including in adults.

Thus, we can distinguish two categories of probable causes of the Arnold-Chiari syndrome.

Causes of an innate nature:

• the cranium during intrauterine development undergoes changes - for example, a reduced posterior cranial fossa is formed, which becomes an obstacle to the normal location of the cerebellum. There may be other disorders in the growth and development of the bone apparatus, which does not match the parameters of the brain;
• in utero formed too large with the same occipital opening.

Reasons for the acquired nature:

• trauma of the cranium and brain of the child in the course of labor;
• cerebral spinal cord injury with dilatation of the central canal.

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

[8], [9], [10], [11]

Pathogenesis

In the etiology of the development of the syndrome, some common risk factors distinguish. Thus, the Arnold-Chiari syndrome in the fetus may appear due to such reasons:

• independent or uncontrolled use of medicines by a pregnant woman;
• use of alcoholic beverages by a pregnant woman, as well as the effect of nicotine;
• viral infections in a woman during pregnancy.

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

[12], [13]

Symptoms of the syndrome Arnold-Chiari

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

• dyspepsia, attacks of nausea;
• weakness in the hands, paresthesia;
• pain in the cervical spine;
• sensation of noise in the ears;
• shakiness while walking;
• diplopia;
• difficulty with swallowing, confusion in speaking.

The first signs of the II degree of the Arnold-Chiari syndrome appear immediately after the baby's birth, or in infancy. The Arnold-Chiari syndrome in the child is manifested by the following disorders:

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

The third degree of the disease is the most severe. Often it is possible to observe lethal cases as a result of a cerebral or spinal cord infarction. For the third degree of the Arnold-Chiari syndrome, the following characteristics are characteristic:

• when turning the head, the patient has a loss of vision or diplopia, sometimes dizziness and fainting;
• there is tremor, coordination disorders;
• the sensitivity of a part or half of the trunk is lost;
• the facial muscles, the muscles of the limbs and the body are weakened;
• there are difficulties with urination.

Depending on the degree and symptomatology prescribe different types of treatment of pathology.

Stages

The degrees, or types of the syndrome, differ among themselves in the structural features of the brain tissues, strangulated in the spinal canal, as well as the presence of impaired brain element formation and the depth of infringement.

• At type I, the infringement occurs relatively low (in the cervical region), the functions of the brain are within the normal range.
• In type II, the cerebellum is moved into the large occipital opening simultaneously with the presence of defects in the spinal cord and brain.
• In type III, the occipital hernia is detected with complete displacement of the posterior cerebral structures into the enlarged large occipital foramen. A similar degree of Arnold-Chiari syndrome has the worst prognosis and in most cases ends lethal.

[14], [15], [16]

Complications and consequences

1. Paralysis, which is a consequence of pressure on the spinal structures.
2. The accumulation of fluid in the brain (hydrocephalus).
3. Formation of cystic or cavity formation in the spine (syringomyelia).
4. An infarction of the brain or spinal cord.
5. Apnea, death.

[17], [18], [19], [20], [21], [22]

Diagnostics of the syndrome Arnold-Chiari

Analyzes for the diagnosis of Arnold-Chiari syndrome are usually of little informative. To clarify the diagnosis, it is possible to perform lumbar puncture with subsequent analysis of cerebrospinal fluid.

Instrumental diagnosis of the Arnold-Chiari syndrome consists in the appointment of magnetic resonance imaging, which is performed in neurological clinics and departments. The MRI method allows you to examine the cervical, thoracic part of the spine and skull.

A large proportion of diseases are detected by external examination: attention is paid to gait, sensitivity and other characteristic features.

[23], [24], [25], [26], [27]

Differential diagnosis

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

[28], [29], [30]

Treatment of the syndrome Arnold-Chiari

If the patient does not make any significant complaints other than intense painful sensations, he is prescribed medication with various combinations of non-steroidal anti-inflammatory drugs, nootropics and preparations of muscle relaxant action.

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

Meloksikam
Dosage of the drug	Assign inside the 1-2 tab. In a day.
Side effects	Dyspepsia, anemia, swelling.
Special instructions	It is not used to treat children under 15 years old.

Pyracetam
Dosage of the drug	Assign from 30 to 160 mg per kilogram of weight per day, approximately three times. The course of therapy - up to 2 months.
Side effects	Dyspepsia, anxiety, increased libido.
Special instructions	When insomnia, the evening dose of the drug is transferred to an after-dinner reception.

Ibuprofen
Dosage of the drug	Take 200 mg 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.

Midokalm
Dosage of the drug	Assign individually for 50-150 mg to 3 times a day.
Side effects	Sensation of weakness in muscles, lowering of blood pressure, dyspepsia, allergic manifestations.
Special instructions	Do not appoint children under 6 years.

B vitamins are also prescribed in high doses. These vitamins are actively involved in most biochemical processes, providing a normal function of the nervous system. For example, thiamine, which is found in neuronal membranes, significantly affects the repair processes of damaged neural conduction pathways. Pyridoxine provides the production of transport proteins in axial cylinders, and also serves as a quality antioxidant.

The prolonged intake of high doses of vitamins B1 and B12 is not accompanied by side effects. The intake of vitamin B6 in an amount of more than 500 mg per day can cause sensory polyneuropathy.

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

As an auxiliary technique, physiotherapy treatment proved to be very useful. Typically, neurologists recommend the following procedures for patients:

• cryotherapy - activates the body's regulatory systems, stimulates the immune and endocrine systems, anesthetizes;
• laser therapy - improves microcirculation and nutrition of tissues in a damaged area;
• magnetotherapy - helps to start the internal health reserves of the body.

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

Successfully applied to alleviate Arnold-Chiari syndrome and homeopathy. The main principle of homeopathic treatment is the use of negligible scanty dosages of herbal remedies that act as a counterbalance to the disease. Doses of homeopathic preparations are called "dilutions": they can be tithes or hundreds. For the manufacture of medicines, as a rule, plant extracts and, for the most part, alcohol are used.

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

Anevro	Take 8-10 pellets three times a day.	Anesthetizes, soothes, helps restore damaged nerve fibers.
Valeriana Heel	Take 15 drops three times a day.	Normalizes sleep, facilitates psychosomatic symptoms.
Nevesed	Take 8-10 pellets three times a day.	Eliminates irritability, soothes and smooths the manifestation of neurotic reactions.
Vertigoheel	Take 1 tablet or 10 drops three times a day.	Eliminates dizziness, facilitates the symptomatology of craniocerebral lesions.
Spiegelon	Take 1 tablet three times a day.	Anesthesia, relieves stress.

Homeopathic preparations are commercially available. Side effects during their admission are almost non-existent, however, taking medications without consulting a doctor is strongly discouraged.

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

The most common surgical procedure for Arnold-Chiari syndrome is suboccipital craniectomy - enlargement of the large occipital opening by the method of sawing the occipital bone with the removal of the arch of the cervical vertebra. As a result of surgery, direct pressure on the brain stem decreases and the circulation of the CSF stabilizes.

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

At the end of the operation, the wound is sutured, sometimes installing titanium stabilizer plates. The need for their installation is decided individually.

Typically, the standard operation lasts from 2 to 4 hours. The rehabilitation period is 1-2 weeks.

Alternative treatment

Alternative recipes for the treatment of Arnold-Chiari syndrome are primarily aimed at arresting the pain syndrome and relaxing the spasm-affected musculature. Such treatment can not replace traditional therapy, but it can effectively supplement it.

• Pour 200 ml of hot water 2 tbsp. L. Herbs or rhizomes of the althea, insist during the night. Used to make compresses several times a day, until the condition is relieved.
• Boil the chicken egg, clean it hot, cut it in half and put it to the sore spot. Take off when the egg completely cools.
• Apply compresses of pure natural honey.
• Brewed with boiling water (200 ml) 1 tbsp. L. Fern, keep on low heat for up to 20 minutes. Cool and take 50 ml before each meal.
• Brewed with boiling water (200 ml) 1 tbsp. L. Leaves raspberries, keep on low heat for up to 5 minutes. Cool and drink for 5 tbsp. L. Three times a day before meals.

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

• Take 1 tbsp. L. Dry raw anise, basil and parsley, pour 700 ml of boiling water, insist 2 h and filter. Take 200 ml in the morning, afternoon and evening before eating.
• Pour 700 ml of water into a mixture of sage, thyme and fennel (3 tbsp.). Insist up to 2 hours, filtered and taken on a glass three times a day before meals.
• Brew a mixture of lemon balm, basil and rosemary (2 tablespoons) in 750 ml of boiling water. Insist and filter, then take 200 ml three times a day before meals.

[31], [32], [33]

Kinesiological Exercises

Kinesiological gymnastics is a special complex of physical exercises that stabilize the human nervous system. Such exercises may well be used to alleviate the condition of patients with Arnold-Chiari syndrome of the 1st degree. As the research has shown, when performing kinesiological gymnastics only once in 7 days, you can achieve an improvement in the world perception and well-being, the removal of the effects of stress, the elimination of irritability, and so on.

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

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

• It is recommended to gradually accelerate the pace of gymnastic elements.
• Most exercises should preferably be done with the eyes closed (to increase the sensitivity of certain areas of the brain).
• Exercises with the connection of the upper extremities are recommended to be combined with synchronous movements of the eyes.
• During breathing movements, you should try to connect the visualization.

As practice has shown, the employment of kinesiology, in addition to the development of nerve conduction, brings a lot of pleasure to patients.

Prevention

Because of the lack of necessary information about the etiology of the Arnold-Chiari syndrome, it is rather difficult to determine the 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 the possible consequences of smoking and drinking alcohol.

For the birth of a healthy child, the expectant mother should adhere to the following recommendations:

• fully and nutritionally balanced;
• refrain from smoking and drinking alcohol;
• Do not engage in self-medication and strictly follow the advice and prescriptions of the doctor.

Such rules must be observed not only with the pregnancy already taken place, but also when planning it.

[34], [35], [36], [37], [38], [39]

Forecast

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

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

If you ignore the Arnold-Chiari syndrome, the disorder will increase, gradually disabling the spine, which sooner or later ends in paralysis.

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