Complex chiari syndrome. Chiari malformation type II

Arnold-Chiari is a congenital or anomaly that occurs during the formation of a child inside the womb. The anomaly occurs due to compression of the brain, which causes deformation of the cranial regions. The consequences are as follows: the cerebellum and brain stem are greatly displaced and descend into occipital part, performance is impaired.

The main causes of the anomaly

All procedures should be carried out strictly according to the doctor’s recommendation. A real specialist will not put accurate diagnosis without conducting a proper examination.

Effective treatment of the syndrome

Currently, two types of treatment are used: surgical, when it comes to, and conservative.

Conservative treatment is used when the disease does not cause the patient severe discomfort and does not affect its development. The doctor recommends more frequent physical education, exercises for muscle coordination. Some drugs are also prescribed: pain reliever, muscle relaxant, anti-inflammatory agent. Additionally, a complex is prescribed, especially group B, since they are responsible for biochemical processes in the body and normalize the work of the central nervous system.

Of course, such appointments will not help to completely get rid of the disease, but will allow you to do without surgical intervention as long as possible.

If the malformation of the disease is progressive, then urgent surgical intervention will be required. Either surgery or bypass surgery is performed. The operation solves two main reasons:

Correct defects that contribute to compression of the skull and brain.
Brings the movement of cerebrospinal fluid back to normal.

Such an operation is quite common, its duration is no more than two hours. The patient recovers completely within a couple of weeks. Thanks to the operation, intracranial pressure normalizes, and the space in the spinal cord and brain increases, the disease recedes.

Preventive measures

You always need to take care of your health, and if this is a period when a woman bears under her heart, then the responsibility doubles. There are some preventive measures to prevent the disease:

include more fruits and vegetables in your diet
drink fresh juices, eat dairy products and meats rich in protein
take prenatal vitamins
give up bad habits, if any
take only those medications that are acceptable during pregnancy and only as prescribed by a doctor
carry out all necessary examinations

Arnold-Chiari malformation is a collective concept that refers to a group of congenital defects of the cerebellum, medulla oblongata and pons (mainly cerebellum) and upper sections spinal cord. In a narrow sense, this disease appears to be a prolapse posterior sections brain to the foramen magnum - the place where the brain passes into the spinal cord.

Anatomically, the posterior and lower sections of the brain are localized in the posterior part of the skull, where the cerebellum, medulla oblongata and pons are located. Below is the foramen magnum - a large hole. Due to genetic and congenital defects, these structures are displaced downwards, towards the foramen magnum. Due to such dislocation, the brain structures are damaged and neurological disorders occur.

Due to compression lower sections brain, blood flow and lymphatic drainage are disrupted. This can lead to brain swelling or hydrocephalus.

The incidence of the pathology is 4 people per 1000 population. The timeliness of diagnosis depends on the type of disease. For example, one of the forms can be diagnosed immediately after the birth of a baby, while another type of anomaly is diagnosed accidentally during routine examinations using magnetic resonance imaging. Average age Patient 2 is from 25 to 40 years old.

In more than 80% of cases, the disease is combined with syringomyelia, a pathology of the spinal cord in which hollow cysts form in it.

Pathology can be congenital or acquired. Congenital is more common and manifests itself on early years child's life. The acquired variant is formed due to slowly growing cranial bones.

Will they be accepted into the army with an anomaly: Arnold-Chiari disease is not on the list of contraindications for military service.

Whether they give disability: the issue of issuing a disability depends on the degree of displacement of the tonsils of the cerebellum. So, if they are not lowered lower than 10 mm, disability is not given, since the disease is asymptomatic. If the lower parts of the brain are lowered below, the issue of issuing a disability depends on the severity of the clinical picture.

Prevention of the disease is nonspecific, since there is no single cause of the development of the disease. Pregnant women are advised to avoid stress, injury and adhere to proper nutrition during gestation. What pregnant women should not do: smoke, drink alcoholic drinks and drugs.

Life expectancy depends on the intensity of the clinical picture. Thus, types 3 and 4 anomalies are not compatible with life.

For what reasons does pathology occur?

Researchers have not yet determined the exact cause. Some researchers argue that the defect is a consequence of a small posterior cranial fossa, which is why the posterior parts of the brain simply have “nowhere to go”, so they are displaced downward. Other scientists say that Arnold-Chiari malformation develops due to an overly large brain, which, with its volume and mass, pushes the lower structures into the foramen magnum.

A birth defect may be latent. A downward displacement can be provoked, for example, by cerebral hydrocele, which increases pressure inside the skull and pushes the cerebellum and brainstem downward. Injuries to the skull and brain also increase the likelihood of developing a defect or create conditions for the brain stem to shift to the foramen magnum.

Clinical picture

The disease and its symptoms are based on three pathophysiological mechanisms:

Compression of the stem structures of the lower parts of the brain and upper parts of the spinal cord.
Compression of the cerebellum.
Impaired circulation of cerebrospinal fluid in the area of the foramen magnum.

The first pathophysiological mechanism leads to disruption of the structure of the spinal cord and the functional ability of the nuclei of the lower parts of the brain. The structure and function of the nuclei of the respiratory and cardiovascular centers are disrupted.

Compression of the cerebellum leads to impaired coordination and the following disorders:

Ataxia is a violation of coordination in the movements of different muscles.
Dysmetria is a violation of motor acts due to the fact that spatial perception is disrupted.
Nystagmus is oscillatory rhythmic eye movements with high frequency.

The third mechanism - a violation of the utilization and waste of cerebrospinal fluid - leads to increased pressure inside the skull and develops hypertension syndrome, manifested by the following symptoms:

Bursting and aching headache, worsening with changes in head position. Cephalgia is localized mainly in the back of the head and upper neck. The pain also increases with urination, defecation, coughing and sneezing.
Autonomic symptoms: loss of appetite, increased sweating, constipation or diarrhea, nausea, dizziness, shortness of breath, palpitations, short-term losses consciousness, sleep disturbance.
Mental disorders: emotional lability, irritability, chronic fatigue, nightmares.

Types

Congenital anomalies are divided into four types. The type of defect is determined by the degree of displacement of stem structures and a combination of other malformations of the central nervous system.

1 type

Type 1 disease in adults is characterized by the fact that parts of the cerebellum descend below the level of the foramen magnum in the back of the head. It is more common than other types. A characteristic symptom of the first type is the accumulation of cerebrospinal fluid in the spinal cord.

The first type of anomaly is often combined with the formation of cysts in the tissues of the spinal cord (syringomyelia). The big picture anomalies:

bursting headache in the neck and back of the head, which gets worse when coughing or overeating;
vomiting, which does not depend on food intake, as it is of central origin (irritation of the vomiting center in the brain stem);
stiffness of the neck muscles;
speech disorder;
ataxia and nystagmus.

When the lower structures of the brain are displaced below the level of the foramen magnum, the clinic is supplemented and has the following symptoms:

Visual acuity decreases. Patients often complain of double vision.
Systemic vertigo, in which the patient feels that objects are rotating around him.
Noise in ears.
Sudden short-term cessation of breathing, from which the person immediately wakes up and takes a deep breath.
Short-term loss of consciousness.
Vertigo when abruptly assuming a vertical position from a horizontal position.

If the anomaly is accompanied by the formation of cavity structures in the spinal cord, the clinical picture is supplemented by a violation of sensitivity, paresthesia, weakening of muscle strength and disruption of the pelvic organs.

2 type and 3 type

An anomaly of type 2 in the clinical picture is very similar to an anomaly of type 3, so they are often combined into one variant of the course. Arnold-Chiari anomaly of the 2nd degree is usually diagnosed after the first minutes of a baby's life and has the following clinical picture:

Noisy breathing accompanied by whistling.
Periodic complete stops of breathing.
Violation of the innervation of the larynx. Paresis of the muscles of the larynx occurs and the act of swallowing is disturbed: food does not enter the esophagus, but into the nasal cavity. This manifests itself from the first days of life during feeding, when milk comes back through the nose.
Chiari anomaly in children is accompanied by nystagmus and increased tone of the skeletal muscles, mainly the tone of the muscles of the upper extremities increases.

An anomaly of the third type, due to gross defects in the development of brain structures and their downward displacement, is incompatible with life. Type 4 anomaly is hypoplasia (underdevelopment) of the cerebellum. This diagnosis is also not compatible with life.

Diagnostics

To diagnose pathology, instrumental methods for diagnosing the central nervous system are usually used. The complex uses electroencephalography, rheoencephalography and echocardiography. However, they reveal only nonspecific signs of brain dysfunction; these methods do not produce specific symptoms. The radiography used also visualizes the anomaly in insufficient volume.

Most informative method Diagnosis of a birth defect – . The second valuable diagnostic method is multislice computed tomography. The study requires immobilization, so small children are put into artificial medicated sleep, in which they remain during the entire procedure.

MRI signs of anomaly: on layer-by-layer images of the brain, displacement of the brain stem is visualized. Because of its good visualization, MRI is considered the gold standard in diagnosing Arnold-Chiari malformation.

Treatment

Surgery is one of the treatment options for the disease. Neurosurgeons use the following methods of elimination:

Cutting the filum terminale

This method has advantages and disadvantages. Advantages of cutting the final thread:

treats the cause of the disease;
reduces the risk of sudden death;
no after surgery postoperative complications and death;
the removal procedure lasts no more than one hour;
eliminates the clinical picture, increasing the patient’s quality of life;
reduces intracranial pressure;
improves local blood circulation.

Disadvantages of the method:

after 3-4 days, pain is present in the surgical area.

Craniectomy

The second operation is craniectomy. Advantages:

eliminates the risk of sudden death;
eliminates the clinical picture and improves a person’s quality of life.

Flaws:

the cause of the disease is not eliminated;
after the intervention, the risk of death remains – from 1 to 10%;
there is a risk of postoperative intracerebral hemorrhage.

How to treat Arnold-Chiari malformation:

The asymptomatic course does not require either conservative or surgical intervention.
In case of mild clinical picture, it is indicated symptomatic therapy. For example, for headaches, painkillers are prescribed. However, conservative treatment of Chiari malformation is ineffective if the clinical picture is severe.
If neurological deficits are observed or the disease reduces the quality of life, neurosurgery is recommended.

Forecast

The prognosis directly depends on the type of anomaly. For example, with an anomaly of the first type, the clinical picture may not appear at all, and a person with a displacement of the brain stem will die a natural death not from the disease. How long do people live with anomalies of the third and fourth types: patients die a few months after birth.

Under certain conditions, babies are born with congenital diseases. Arnold-Chiari malformation is a brain disease that is associated with dysfunction of the cerebellum, medulla oblongata, there are several options, it has specific symptoms, which accompany a person throughout his life or develop over time. Arnold-Chiari syndrome in a fetus or adult affects the functioning of the vasomotor, respiratory center.

Arnold-Chiari Syndrome

low position cerebellar tonsils - a congenital condition, the structures of the brain of the head descend into the foramen magnum. The process usually involves the medulla oblongata and cerebellum. Arnold Chiari disease is detected in some people completely by accident, for example, during an examination due to another pathology. At the first stage, the course of the disease is mild and often unnoticeable. In this situation, Arnold-Chiari malformation does not pose a threat to the health of the body.

The pathology can be practically asymptomatic, but often this disease is combined with syringomyelia (the name of the disease gray matter spinal cord). Lack of treatment can provoke hydrocephalus (accumulation of fluid in the skull), cerebral infarction, etc. dangerous pathologies, cases of disability were noted. The defect can be identified immediately after birth or after 20-30 years. Chiari malformation can be one of 4 types.

1 type

Arnold Chiari malformation of the 1st degree - the structures of the posterior part are often infringed due to exit through the foramen magnum. This position leads to the accumulation of cerebrospinal fluid. Chiari syndrome of the first degree is a displacement of the cerebellar tonsils, they are located below the foramen magnum. Arnold-Chiari malformation is often found in adolescence.

Arnold-Chiari syndrome stage 2

Anomaly of the second degree has more pronounced structural changes. The cerebellum, which is located in the foramen magnum, is involved in the process. Screening ultrasounds of the fetus can show some defects in the structure of the spine and spinal cord. In most cases, the prognosis for the baby’s life is favorable, but clinical signs of pathology will be present. This indicates the need dynamic observation for the patient.

Type 3

In this case, almost all formations of the cranial fossa are located below the foramen magnum (pons, 4th ventricle, medulla oblongata, cerebellum). Often they are found in a cervico-occipital cerebral hernia (when there is a defect in the spinal canal, in which the vertebral arches are not closed, the contents of the dural sac, which includes all the membranes and the spinal cord). With this type of anomaly, the foramen magnum has an increased diameter.

4 type

This is the most severe version of the disease. At this option In Arnold Chiari syndrome, cerebellar hypoplasia and underdevelopment are observed. Often combined with congenital cysts posterior cranial fossa, congenital cysts and hydrocephalus. When type 4 is diagnosed, the prognosis is unfavorable; in most cases, the disease ends in the death of the patient.

Lifespan

Chiari Arnold syndrome comes as a surprise to some when diagnosed with another pathology. The question immediately arises about life expectancy with this pathology. The answer depends on the severity of the pathology, the type of anomaly, and the timeliness of treatment. proper treatment, surgical intervention. For example, the first type of Arnold-Chiari syndrome is often asymptomatic; people have an average life expectancy. Forecasts for other types are as follows:

In the presence of neurological symptoms In people with type 1-2, it is recommended to undergo surgery faster. Possible complications which tighten the spinal cord and brain cannot be cured in the future.
The third type, as a rule, causes the death of a child at birth.
As in the previous case, it ends in the death of the newborn.

Causes

The exact causes of Arnold-Chiari malformation have not been established at the moment. Doctors identify the following factors that may increase the risk of developing pathology:

the brain has increased in size;
the cranial fossa has decreased;
smoking, alcohol during pregnancy, which caused fetal intoxication.

Arnold-Kari anomaly can be a consequence of improper planning and management of pregnancy. Alcohol intoxication, excess medication, viral diseases, smoking is the most hazardous factors risks that can cause a variety of defects in the fetus. According to another version, the anomaly becomes a consequence of a violation of the development of the skull. The progression of the pathology can cause craniocerebral injuries, hydrocephalus.

Symptoms

The most common type of Arnold-Chiari syndrome is type 1. Symptoms usually appear during puberty or in an adult after 30 years of age. The following symptoms may be observed:

after physical activity headaches appear as a result of sneezing and coughing;
violations of fine motor skills of the hands;
imbalance resulting in abnormal gait;
numbness of the hands, arms;
temperature sensitivity deviations;
pain in the neck, neck.

Diagnosis of types 2 and 3 indicates congenital symptoms of the same nature. In some cases, there is a severe course of the pathology, the patient feels such symptoms under such conditions:

nausea;
headache;
vomit;
speech difficulties;
increased tone muscles of the floor of the neck line;
hearing loss;
noise in ears;
loss of vision;
different sizes of pupils;
sleep disorders;
systemic dizziness;
violation of pain sensitivity;
skin thickening;
the appearance of burns on the hands;
joint enlargement;
ataxia;
violation of the processes of swallowing.

arnold chiari anomaly type 1 what not to do

The brain is responsible for the activities of the entire organism, and therefore any violation of its structure inevitably leads to dire consequences. This conclusion is clearly seen in the example of such a serious disease as "Chiari malformation" or Arnold-Chiari anomaly, the degrees and treatment of which will be discussed in this article.

Characteristics of the disease

The Arnold-Chiari anomaly is congenital disorder the structure of the rhomboid part of the brain, leading to displacement of brain structures and disrupting the flow of cerebrospinal fluid (CSF). By definition, it can be understood that this anomaly is extremely severe. It is divided into 4 types, and some of them are not compatible with life.

The “diamond-shaped brain” includes the medulla oblongata, the cerebellum, and the “bridge” located between its lobes. These sections are located at the base of the skull, at the point of its connection with the spinal canal, and therefore the spinal cord. Arnold-Chiari malformation develops when, for some reason, the size of the bone receptacle no longer accommodates the rhombencephalon. and it is forced to shift into the foramen magnum up to I-II cervical vertebra. This displacement leads to disruption of the free flow of cerebrospinal fluid, and therefore to the accumulation of cerebrospinal fluid in the brain. This process leads to severe consequences for the body.

Causes of the disease

Medicine still does not have an exact answer about the causes of Arnold-Chiari malformation. Scientists suggest that with this disease there are two factors at once: birth defects nervous and skeletal system. This disease is congenital, and depending on the type, the anomaly can manifest itself both from the first minutes of life and at a fairly mature age.

Types of disease

Type I anomaly is the most common and has congenital causes. As a rule, the disease first manifests itself at the age of 30-40 years and with early diagnosis treatable.

Type II anomaly develops from injuries that the baby received at birth and often leads to the development of hydrocephalus. This disease, although difficult, is treatable.

Anomalies of types III and IV are quite rare and are characterized by a significant decrease in the size of the cerebellum. The disease is noticeable from the first minutes of birth and is usually incompatible with life.

Symptoms of the disease

Arnold-Chiari malformation type 1 makes itself felt with the following symptoms:

frequent headaches, sneezing and coughing after any physical activity;
pain and weakness in the occipital region and neck;
frequent dizziness and fainting;
problems with fine motor skills;
violation of movement coordination;
attacks of falling without loss of consciousness;
numbness of the hands and weakening of the arm muscles;
violation of temperature sensitivity;
swallowing dysfunction;
decreased visual acuity up to blindness;
convulsions.

As for the symptoms of other types of disease, they are the same, but more pronounced.

Treatment of the disease

In the case when the disease does not make itself felt, the person does not receive any treatment, but must regularly undergo examination (MRI) and consult with a neurosurgeon. For pain in the neck, doctors prescribe painkillers (Nimesulide or Mydocalm). Loss of coordination frequent fainting and decreased vision is also treated with medications, and if therapy is ineffective, the patient undergoes surgery. Swallowing dysfunction in a patient with Chiari malformation requires urgent surgery to relieve compression of the spinal cord.

Disease prognosis

Having studied the reasons why Arnold-Chiari malformation develops, the extent and treatment of such dangerous disease, it is worth saying that in the case of type I or II disease, doctors give a favorable prognosis for treatment, up to the complete elimination of all manifestations of the disease. As for anomalies of III and IV types, in these cases even surgery does not always allow saving the patient's life. Good health to you!

Did you like the article? Share with your friends on social networks!

This anomaly is classified as congenital and represents a discrepancy in the size of the brain structures and the posterior cranial fossa, which leads to dislocation of the cerebellum, which emerges from the large foramen of the base of the brain and is infringed. This pathology is named after the names of two scientists who described its symptoms at different times.

In essence, this anomaly is a heterotopic location of the medulla oblongata and cerebellum in an excessively expanded spinal canal. Given congenital disease leads to the development of various neurological symptoms, which are perceived by neurologists as symptoms multiple sclerosis, syringomyelia, or growth of a tumor located in the posterior cranial fossa. This anomaly in most cases (about 80%) is adjacent to syringomyelia, which is characterized by the appearance of spinal cysts.
There are four types of this pathology, but the first two types are more common, since with types 3 and 4 life is impossible.

Arnold Chiari anomaly type 1 includes a displacement of the brain structures of the posterior cranial fossa below the foramen magnum: the cerebellar tonsils, one or two, descend into the spinal canal, often due to the displacement of the medulla oblongata.

Pathogenesis on present stage development of medicine is unknown. It is assumed that they play a role hereditary factors, traumatic factors during childbirth ( birth injury head) and increased intracranial pressure, causing cerebrospinal fluid to strike the walls of the central canal of the spinal cord.
With this disease, three syndromes can occur:

Cerebellobulbar
Syringomyelitic
Pyramid

Active symptoms begin to appear at 30-40 years of age. Soreness and pain occur in the cervical spine, which intensifies with sneezing and coughing. Over time, muscle strength decreases, the sensitivity of the skin of the hands, both pain and temperature, is impaired. Spasticity of the legs and arms increases, fainting and dizziness appear, visual acuity decreases, and in advanced cases, attacks of apnea periodically occur.

In the case of Arnold Chiari malformation type 1, the diagnostic standard with a high degree of certainty is an MRI of the brain.

In treatment latent form disease, the main role is played by monitoring the dynamics and examining the patient every year. For minor symptoms - pain in the cervical spine, dizziness - conservative treatment with non-steroidal anti-inflammatory drugs is prescribed, and dehydration therapy is also periodically carried out using diuretics. If such therapy is ineffective, surgical intervention is prescribed, the purpose of which is to decompress brain structures. After treatment, symptoms almost always disappear, and motor function and sensitivity can be partially restored.

Left ventricular hypertrophy is a fairly serious disease that begins gradually, and many people do not even know about it. The reason for its occurrence in 25-30% is hypertension. The heart works in tension, the walls.

The thoracic spine is made up of 12 small bones called vertebrae, located from the middle of the back to the base of the neck. When these bones are improperly compressed or damaged.

In our article we will talk about possible reasons, symptoms and treatment methods for a disease such as inflammation trigeminal nerve, or trigeminal neuralgia.

Tachycardia is not always a sign of heart disease and disorders in it. There are many factors that can cause heart palpitations.

Arnold-Chiari malformation

Arnold-Chiari Syndrome is an abnormality of brain development in which the part of the skull containing the cerebellum is too small or deformed, resulting in compression of the brain. The most Bottom part cerebellum or tonsils are displaced into top part spinal canal. The pediatric form - Arnold-Chiari syndrome type III - is always associated with myelomeningocele (herniation of the spinal cord and meninges). The adult form, Chiari syndrome type I, develops because the back of the skull is not large enough.

Causes

When the cerebellum is pressed into the upper part of the spinal canal, it can interfere with the normal flow of cerebrospinal fluid, which protects the brain and spinal cord. Impaired circulation of cerebrospinal fluid can lead to blockage of signals transmitted from the brain to underlying organs or to the accumulation of cerebrospinal fluid in the brain and spinal cord. Pressure of the cerebellum on the spinal cord or lower brain stem can cause syringomyelia.

Diagnostics

The diagnosis is established based on the results of MRI. If necessary, we will carry out computed tomography with three-dimensional reconstruction of the occipital bone and cervical vertebrae.

Symptoms:

With Arnold-Chiari anomaly, we usually observe two to three or more of the following symptoms:

Headache associated with an increase intracranial pressure(stronger in the morning) or with increased tone of the neck muscles ( pain points under the back of the head);

In more severe cases, the following is likely:

In severe cases, the development of conditions that threaten a heart attack of the brain and spinal cord is likely.

Treatment

Treatment for Arnold-Chiari anomaly depends on the severity and condition of the patient. If there are no symptoms, your doctor may only prescribe observation with regular examinations as treatment.

If primary symptoms headaches or other types of pain, your doctor may recommend pain medications. Some patients find relief from taking anti-inflammatory foods. This may prevent or delay surgery.

Articles from the forum on the topic “ Arnold-Chiari malformation »

Arnold-Chiari syndrome is a developmental abnormality of the brain in which the part of the skull containing the cerebellum is too small or misshapen, causing compression of the brain. The lowest part of the cerebellum or tonsils are displaced into the upper part of the spinal canal. The pediatric form - Arnold-Chiari syndrome type III - is always associated with myelomeningocele (herniation of the spinal cord and meninges). The adult form, Chiari syndrome type I, develops because the back of the skull is not large enough.
Symptoms:
With Arnold-Chiari malformation, we usually observe two or three or more of the following symptoms:

Dizziness and/or unsteadiness (may worsen when turning the head);
Noise (ringing, hum, whistle, hiss, etc.) in one or both ears (may increase when turning the head);
Headache associated with an increase in intracranial pressure (stronger in the morning) or with an increase in the tone of the neck muscles (pain points under the back of the head);
Nystagmus (involuntary twitching of the eyeballs).
In more severe cases, the following is possible:

Transient blindness, double vision, or other visual disturbances (may appear when turning the head);
Tremor of arms, legs, movement coordination disorders;
Decreased sensitivity of a part of the face, part of the torso, one or more limbs;
Weakness of the muscles of the face, part of the body, one or more limbs;
Involuntary or difficult urination;
Loss of consciousness (may be caused by turning the head).
In severe cases, it is possible to develop conditions that threaten a heart attack of the brain and spinal cord.
The diagnosis is established based on the results of MRI.

Arnold-Chiari malformation type I is a prolapse of the structures of the posterior cranial fossa (cerebellum) in spinal canal below the plane of the foramen magnum.
The posterior cranial fossa is the part of the internal base of the skull formed by occipital bone, pyramids temporal bones and body sphenoid bone; contains the brain stem and cerebellum.

Arnold-Chiari anomaly is manifested by the descent of the cerebellar tonsils through the foramen magnum into the spinal canal. If the clinical picture of the disease includes ataxia (unsteadiness, instability when walking), damage to the motor pathways and syringomyelia, then a consultation with a neurosurgeon is necessary to decide on surgical treatment. In milder cases, symptomatic conservative therapy is performed.

Sources:

Arnold-Chiari malformation is classified as a defect of the cranio-vertebral zone. It is formed in the posterior cranial fossa (PCF), with an insufficient volume of which, the posterior parts of the brain and cerebellum are shifted towards the foramen magnum, and the flow of cerebrospinal fluid is also disturbed.

The back of the skull forms the so-called posterior cranial fossa, in which the hemispheres and the cerebellar vermis, the pons, the medulla oblongata, which passes into the spinal cord after passing through the foramen magnum, are located. The large occipital foramen is limited by the bone base of the skull and is not able to change its diameter; any displacement of the brain structures is fraught with a discrepancy between their size and the diameter of the hole, wedging and infringement nerve tissue, the consequences of which can be fatal.

The vital nerve centers responsible for the activity of the cardiovascular system and respiration are concentrated in the medulla oblongata, so not only a neurological deficit will be a manifestation of the disease. In severe cases, there is depression of vital functions, and the patient may die. The displacement of the hemispheres of the cerebellum leads to a stop in the circulation of the cerebrospinal fluid with hydrocephalus, which further exacerbates the existing disorders.

Arnold-Chiari malformation can be congenital, developing in the fetus and combined with other developmental abnormalities, and its clinical manifestations do not always appear immediately. In some cases, a significant period of time passes before the manifestation of the pathology, or a situation arises that provokes the manifestation of a previously asymptomatic anomaly; in other patients, it may even turn out to be an accidental finding on MRI. Often the pathology is acquired in nature and occurs under the influence of external causes, while the brain and skull at birth have a normal structure.

Causes and mechanism of development of posterior fossa defect (PCD)

There is no consensus on the etiology of Chiari malformation. Scientists put forward various theories, each of which is fully justified and has the right to exist.

Previously, the anomaly was considered exclusively a congenital defect, but the observations of specialists showed that only a small part of patients had defects during prenatal development, the rest acquired them already in the process of life.

The causes of the acquired pathology of the cranio-vertebral junction are the uneven growth rate of the nervous tissue of the brain and bone base skull, when the brain increases much faster than the bone container in which it is located. The resulting discrepancy between volumes is the basis of Arnold-Chiari disease.

The congenital form of the pathology is combined with bone dysplasia, which leads to underdevelopment of the skull bones, as well as impaired formation ligamentous apparatus, and any external influence, trauma can dramatically aggravate the manifestations of pathology. The combination of a defect of the cranial fossa with other disorders of uterine development and congenital syndromes is considered characteristic.

Neurologists have formulated two main mechanisms for the formation of pathology:

Reducing the size of the PCF with normal volumes of brain regions (probably due to disturbances during the prenatal period).
An increase in the volume of the brain itself while maintaining the correct parameters of the cranial fossa and foramen magnum, when the brain pushes its caudal sections in the direction of the foramen magnum.

Since the anomaly can be congenital, among the causes are those that can change normal course pregnancy and intrauterine development:

Abuse of medications, drinking alcohol and smoking during pregnancy, especially during pregnancy early stages when the organs and systems of the embryo are just being formed;
Viral lesions in pregnant women, among which infections with a teratogenic effect - rubella, cytomegalovirus, etc. - are especially dangerous.

Arnold-Chiari malformation also occurs for a number of acquired reasons when the brain and bones of the skull are initially correctly developed. The following can lead to its appearance after birth:

Birth injuries, both spontaneous and during obstetric care;
Traumatic brain injuries and hydrodynamic impact of the cerebrospinal fluid on the walls of the spinal cord canal in the event of a violation of the cerebrospinal fluid dynamics (this happens in adults);
Hydrocephalus.

Hydrocephalus can be a provoking factor, since an increase in the volume of contents in the skull, even due to fluid, inevitably entails an increase in pressure and a displacement of the brain in the caudal (posterior) direction. On the other hand, it is a manifestation of the anomaly itself, when cerebellar prolapse causes a blockade of the cerebrospinal fluid pathways and an increase in the pressure of the cerebrospinal fluid circulating through the cavities of the brain.

Types and degrees of Arnold-Chiari malformation

Depending on the presence of certain changes in the brain and bone base of the skull, it is customary to distinguish several types of Arnold-Chiari anomaly:

Chiari malformation type 1, when there is a downward displacement of the cerebellar tonsils, is usually detected in adults and adolescents, and is often combined with impaired cerebrospinal fluid dynamics and accumulation of cerebrospinal fluid in the central canal of the spinal cord (hydromyelia). Possible compression of the brain stem.

Arnold-Chiari malformation type 1 is the most commonly diagnosed and has a fairly favorable prognosis

Arnold-Chiari malformation type 2 - manifests itself already in newborns, as there is a displacement of a much larger volume of the brain than with type 1: the cerebellar tonsils and its vermis, the medulla oblongata with the fourth ventricle, and possibly the midbrain formations. Usually, with stage 2 defects, there is a disruption of the flow of cerebrospinal fluid with hydromyelia. The disease is often combined with the presence of a congenital hernia of the spinal cord and vertebral anomalies.
Type 3 of the disease is characterized by protrusion of the pia mater with the substance of the brain in the occipital region, which also includes the cerebellum and medulla oblongata.

Arnold-Chiari malformation type 3 in the picture

Arnold-Chiari malformation type 4 is manifested by underdevelopment of the cerebellum when the latter is reduced, and therefore does not descend more distally to the canal in the bone. The pathology makes the newborn unviable and usually ends in death.

As for the degrees of severity:

Arnold-Chiari malformation of the 1st degree can be considered one of the mildest variants of the pathology, since there are practically no defects of the brain itself, and the clinic may be completely absent, appearing only under unfavorable conditions - trauma, neuroinfection, etc.

Malformations of the second and third degrees, in turn, are often combined with various malformations of nervous tissue - hypoplasia of some parts of the brain and subcortical nodes, displacement of gray matter, cysts of the cerebrospinal fluid tract, underdevelopment of the brain convolutions.

Manifestations of Arnold-Chiari syndrome

The symptoms of Arnold-Chiari syndrome are determined by its type and the nature of the displacement of the PCF structures. It is often asymptomatic and discovered incidentally during brain examinations. In adults, the appearance of symptoms can be triggered by a head injury; in children, some forms of the disease are noticeable already in the first hours and days of life.

Type I anomaly is diagnosed most often and can manifest itself in adolescence or adulthood with the following syndromes:

Hypertensive;
Cerebellar;
Bulbar;
Syringomyelic;
Phenomena of damage to cranial nerves.

Hypertensive syndrome is caused by an increase in intracranial pressure due to blockade of the outflow of cerebrospinal fluid by displaced parts of the brain. It manifests itself:

Headaches in the back of the head, especially when sneezing or coughing;
Nausea and vomiting, after which the patient does not feel relief;
Neck muscle tension.

Signs of involvement of the cerebellum (cerebellar syndrome) are considered disorders of speech, motor function, balance, nystagmus. Patients complain of unsteadiness of gait, instability of body position in space, difficulty in fine motor skills and clarity of movements.

Damage to the brain stem is dangerous due to the location of the nuclei of the cranial nerves and vital nerve centers. Stem symptoms include:

dizziness; double vision and decreased vision;
difficulty swallowing;
hearing loss, tinnitus;
fainting, hypotension, sleep apnea.

Adult carriers of Arnold-Chiari malformation indicate an increase in dizziness and tinnitus, as well as paroxysms of loss of consciousness when turning and tilting the head. Due to compression of the trunks of the cranial nerves, atrophy of half of the tongue and a violation of the movement of the larynx with a disorder in the act of swallowing, breathing and voice formation appear.

With the formation of cavities and cerebrospinal fluid cysts against the background of obstructed flow of cerebrospinal fluid in patients with type I malformation, signs of syringomyelic syndrome appear - sensory disorder, numbness of the skin, muscle wasting, dysfunction of the pelvic organs, decrease and disappearance of abdominal reflexes, peripheral neuropathies and changes in the joints.

Sensitivity disorders are accompanied by a disturbance in the perception of one’s own body, when the patient, having closed his eyes, cannot say in what position his arms or legs are. Sensitivity to pain and temperature also decreases.

According to the observations of neurologists, the diameter and localization of a spinal cord cyst do not necessarily affect the severity and prevalence of sensory and motor sphere, muscle wasting.

With type 2 and 3 syndrome, the course of the pathology is much more severe; symptoms appear in the child immediately after birth. Breathing disorders are characteristic - stridor ( noisy breathing), attacks of its stopping, as well as bilateral paresis of the larynx, which provokes swallowing disorders when liquid food enters the nasal passages.

The second type of anomaly in babies in the first months of life is accompanied by nystagmus, increased muscle tone in the arms, and bluish skin, which are especially noticeable when feeding a baby. Motor disorders are variable, their manifestations change, tetraplegia is possible - paralysis of both the upper and lower extremities.

Arnold-Chiari malformation of the third and fourth variants is severe, This congenital pathology, which is not compatible with normal life activities, therefore, the prognosis for such a diagnosis cannot be considered favorable.

Arnold-Chiari malformation can lead to complications caused by blockade of cerebrospinal fluid flow, damage to the cranial nerve nuclei, and infringement of stem structures. The most common are:

Hypertension-hydrocephalic syndrome - an increase in intracranial pressure due to blockage of the cerebrospinal fluid outflow pathways, possible in both children and adults;
Breathing disorders, apnea;
Infectious and inflammatory processes - bronchopneumonia, urinary infections, which are associated with the patient's supine position, impaired swallowing and breathing, and the function of the pelvic organs.

At severe course pathology can cause coma, cardiac and respiratory arrest, which leads to death in a matter of minutes. Resuscitation measures allow you to provide vital functions, but bring the brain back to life and eliminate irreversible consequences Compression of its departments, unfortunately, is practically impossible.

Diagnosis and treatment of Arnold-Chiari malformation

Based on the characteristics of the symptoms and on the basis of an examination by a neurologist, it is impossible to make a diagnosis of Chiari malformation. Encephalography, studies of the vessels of the head will also not give any information regarding the causes of neurological disorders, but they can show the presence of increased pressure in the skull. X-ray, CT, MSCT will indicate the presence of defects in the bones of the skull, which are characteristic of this pathology, but the state of soft tissue structures, nervous tissue cannot be established.

Accurate diagnosis of the anomaly has become possible thanks to the use of MRI, through which the doctor can determine both bone defects and developmental options for the brain itself, its vessels, the level of location of the departments relative to the cranial bones, their size, the volume of the posterior cranial fossa and the width of the foramen magnum. MRI can be considered the only accurate and most reliable method for detecting pathology.

MRI requires immobilization of the patient, who must lie quietly on the machine table for some time. Children with this may have significant difficulties, so the study is carried out in a state of medical sleep. To search for combined defects of the spinal cord and spine, these sections of the spinal column are also examined.

When the diagnosis is established, the patient is referred to a neurosurgeon or neurologist to determine the treatment plan, indications for surgery, and its type.

Arnold-Chiari malformation, which is asymptomatic, does not require treatment. Moreover, the carrier of the pathology himself may not realize that something is wrong in the body. When clinical signs of the disease appear, conservative or surgical treatment is indicated.

If manifestations are limited to headaches, it is prescribed drug therapy , including anti-inflammatory drugs (Nise, ibuprofen, diclofenac), analgesics (ketorol) and drugs that relieve muscle spasm(mydocalm).

In the presence of neurological disorders, signs of compression of parts of the brain, nerve trunks, if there is no effect from drug treatment for 3 months, the patient needs surgical correction.

The operation is necessary to eliminate compression of the nerve tissue and normalize the circulation of cerebrospinal fluid. The most popular operation for Chiari disease is craniovertebral decompression, which is aimed at increasing the size of the PCF.

During decompression, the surgeon removes sections of the occipital bone, resects the cerebellar tonsils, and, if necessary, excises the posterior sections of the first cervical vertebrae. To prevent prolapse rear parts brain, special synthetic patches are applied to the dura mater into the resulting hole.

example of removal of parts of the occipital bone and cervical vertebrae

Decompression of the PCF is considered both traumatic and risky. The statistics are that complications occur in at least every tenth patient, while without surgery the mortality rate is much lower. Due to high risk For surgical treatment, neurosurgeons resort to it only in the case of really serious indications - clinical signs of compression of areas of the brain.

Another option for surgical treatment is shunting, which ensures the outflow of cerebrospinal fluid from the cranial cavity into the thoracic or abdominal cavity. By implanting special tubes, there is an overflow of cerebrospinal fluid and a decrease in intracranial pressure.

At severe forms pathology, hospitalization, measures to prevent infectious complications and correct neurological disorders are indicated. The increase in cerebral edema due to wedging of its sections into the occipital foramen requires treatment in intensive care, including the fight against edema (magnesia, furosemide, diacarb), establishing artificial ventilation lungs in case of breathing problems, etc.

Life expectancy and prognosis for Arnold-Chiari anomalies depend on the type of pathology. In type I, the prognosis can be considered favorable; in some cases, the clinic does not occur at all or is provoked only by strong traumatic factors. When asymptomatic, carriers of the anomaly live as long as all other people.

For anomalies of the second and first types with clinical manifestations, the prognosis is somewhat worse, since a neurological deficit manifests itself, which is difficult to eliminate even with active treatment, so timely surgery is of great importance for such patients. The sooner the patient receives surgical assistance, the less pronounced neurological changes await him.

Malformations of the third and fourth types are the most severe forms of pathology. The prognosis is unfavorable, since many brain structures are involved, combined defects of other organs often occur, severe violations brain stem functions incompatible with life.

Video: presentation and other information on Arnold-Chiari malformation

Step 1: pay for the consultation using the form → Step 2: after payment, ask your question in the form below ↓ Step 3: You can additionally thank the specialist with another payment for an arbitrary amount

Arnold Chiari malformation is a developmental disorder that consists of a disproportion between the size of the cranial fossa and the structural elements of the brain located in it. In this case, the cerebellar tonsils descend below the anatomical level and can be infringed.

Symptoms of Arnold Chiari anomaly are manifested in the form of frequent dizziness, and sometimes end in a brain stroke. Signs of an anomaly may be absent for a long time, and then abruptly declare themselves, for example, after viral infection, head impact or other provoking factors. Moreover, this can happen at any stage of life.

Description of the disease

The essence of the pathology is reduced to incorrect localization of the medulla oblongata and cerebellum, resulting in craniospinal syndromes, which doctors often regard as an atypical variant of syringomyelia, multiple sclerosis, spinal tumors. In most patients, the anomaly in the development of rhombencephalon is combined with other disorders in the spinal cord - cysts that provoke the rapid destruction of the spinal structures.

The disease was named after the pathologist Arnold Julius (Germany), who described the abnormal deviation in the late 18th century, and the Austrian physician Hans Chiari, who studied the disease during the same period of time. The prevalence of the disorder varies from 3 to 8 cases for every 100,000 people. Mostly Arnold Chiari malformation occurs in grades 1 and 2, and adults with types 3 and 4 of the anomaly live very short lives.

Arnold Chiari malformation type 1 involves the descent of elements of the posterior cranial fossa into the spinal canal. Chiari disease type 2 is characterized by changes in the location of the medulla oblongata and fourth ventricle, and hydrops is often present. Much less common is the third degree of pathology, which is characterized by pronounced displacements of all elements of the cranial fossa. The fourth type is cerebellar dysplasia without downward shift.

Causes of the disease

According to a number of authors, Chiari disease is an underdevelopment of the cerebellum, combined with various abnormalities in parts of the brain. Arnold Chiari malformation grade 1 is the most common form. This disorder is a unilateral or bilateral descent of the cerebellar tonsils into the spinal canal. This can occur as a result of the medulla oblongata moving downwards, often the pathology is accompanied various violations craniovertebral border.

Clinical manifestations can occur only in the 3rd–4th decade of life. It should be noted that the asymptomatic course of ectopia of the cerebellar tonsils does not require treatment and often appears accidentally on MRI. To date, the etiology of the disease, as well as the pathogenesis, are poorly understood. A certain role is assigned to the genetic factor.

There are three links in the development mechanism:

genetically determined congenital osteoneuropathy;
trauma to the stingray during childbirth;
high pressure of cerebrospinal fluid on the walls of the spinal canal.

The disease may be caused by hereditary disorders

Manifestations

Based on the frequency of occurrence, the following symptoms are distinguished:

headaches – in a third of patients;
pain in the limbs – 11%;
weakness in the arms and legs (in one or two limbs) – more than half of the patients;
feeling of numbness in the limb – half of the patients;
decrease or loss of temperature and pain sensitivity – 40%;
unsteadiness of gait – 40%;
involuntary eye vibrations – a third of patients;
double vision – 13%;
swallowing disorders – 8%;
vomiting – 5%;
pronunciation disorders – 4%;
dizziness, deafness, numbness in facial area– in 3% of patients;
syncope (fainting) – 2%.

Pain in the head and neck area is a common symptom of pathology.

Chiari disease of the second degree (diagnosed in children) combines dislocation of the cerebellum, brainstem and fourth ventricle. An integral symptom is the presence of a meningomyelocele in the lumbar region (herniation of the spinal canal with protrusion of the substance of the spinal cord). Neurological symptoms develop against the background of an abnormal structure of the occipital bone and cervical region spinal column. In all cases, hydrocephalus is present, often a narrowing of the cerebral aqueduct. Neurological signs appear from birth.

Surgery for meningomyelocele is performed in the first days after birth. Subsequent surgical expansion of the posterior cranial fossa makes it possible to achieve good results. Many patients require bypass surgery, especially with stenosis of the aqueduct of Sylvius. With an anomaly of the third degree, a cranial hernia in the lower occiput or in the upper cervical region is combined with developmental disorders of the brain stem, cranial base and upper vertebrae of the neck. The formation involves the cerebellum and in 50% of cases the occipital lobe.

This pathology is very rare, has an unfavorable prognosis and sharply reduces life expectancy even after surgery. It is impossible to say exactly how long a person will live after timely intervention, but most likely not for long, as this pathology is considered incompatible with life. The fourth degree of the disease is an isolated hypoplasia of the cerebellum and today does not belong to the Arnold-Chiari symptom complex.

Clinical manifestations in the first type progress slowly over several years and are accompanied by the inclusion of the upper cervical spinal cord and distal medulla oblongata with disruption of the cerebellum and caudal group of cranial nerves. Thus, in persons with Arnold-Chiari anomaly, three neurological syndromes are distinguished:

Bulbar syndrome is accompanied by dysfunction of the trigeminal, facial, vestibulocochlear, hypoglossal, and vagal nerves. In this case, there are disturbances in swallowing and speech, beating downwards spontaneous nystagmus, dizziness, breathing disorders, paresis soft palate on the one hand, hoarseness of voice, ataxia, discoordination of movements, incomplete paralysis of the lower extremities.
Syringomyelitic syndrome is manifested by atrophy of the muscles of the tongue, impaired swallowing, lack of sensitivity in the facial region, hoarseness, nystagmus, weakness in the arms and legs, spastic muscle tone etc.
Pyramidal syndrome is characterized by slight spastic paresis of all limbs with hypotonicity of the arms and legs. Tendon reflexes on the extremities are increased, abdominal reflexes are not elicited or decreased.

The operation is performed for severe forms of the disorder

Pain in the back of the head and neck may intensify when coughing or sneezing. The temperature in the hands decreases and pain sensitivity, as well as muscle strength. Fainting and dizziness often occur, and patients' vision deteriorates. When the form is running, apnea (short-term cessation of breathing), rapid uncontrolled eye movements, deterioration of the pharyngeal reflex appear.

Interesting clinical sign in such people - provoking symptoms (syncope, paresthesia, pain, etc.) by straining, laughing, coughing, Valsalva maneuver (forced exhalation with the nose and mouth closed). When increasing focal symptoms(stem, cerebellar, spinal) and hydrocephalus, the question arises of surgical expansion of the posterior cranial fossa (suboccipital decompression).

Diagnostics

The diagnosis of type 1 anomaly is not accompanied by spinal cord damage and is made mainly in adults using CT and MRI. According to post-mortem autopsy, in children with a herniated spinal canal, Chiari disease of the second type is detected in most cases (96-100%). Using ultrasound, you can determine disturbances in the circulation of cerebrospinal fluid. Normally, cerebrospinal fluid circulates easily in the subarachnoid space.

Downward displacement of the tonsils impedes circulation. Due to impaired fluid dynamics, hydrocephalus occurs.

The lateral X-ray and MRI of the skull shows the expansion of the canal of the spinal column at the level of C1 and C2. Angiography of the carotid arteries shows circumflexion of the tonsil cerebellar artery. X-rays show such concomitant changes in the craniovertebral region as underdevelopment of the atlas, odontoid process of the epistrophy, shortening of the atlantooccipital distance.

With syringomyelia, the lateral x-ray shows underdevelopment of the posterior arch of the atlas, underdevelopment of the second cervical vertebra, deformity of the foramen magnum, hypoplasia of the lateral parts of the atlas, and expansion of the spinal canal at the C1-C2 level. Additionally, an MRI and an invasive X-ray examination should be performed.

MRI is the most preferred diagnostic method

The manifestation of symptoms of the disease in adults and the elderly often becomes the reason for the detection of tumors of the posterior cranial fossa or craniospinal region. In some cases, the correct diagnosis is helped by the patient's external manifestations: low hairline, shortened neck, etc., as well as the presence of craniospinal signs of bone changes on X-rays, CT and MRI.

Today, the “gold standard” for diagnosing the disorder is MRI of the brain and cervicothoracic region. It is possible to perform intrauterine ultrasound diagnostics. Possible ECHO signs of a violation include internal dropsy, lemon-shaped head and banana-shaped cerebellum. At the same time, some experts do not consider such manifestations to be specific.

To clarify the diagnosis, different scanning planes are used, thanks to which several informative symptoms in the fetus can be detected regarding the disease. Obtaining an image during pregnancy is quite easy. In view of this, ultrasound remains one of the main scanning options to exclude pathology in the fetus in the second and third trimesters.

Identification of signs of disease in the fetus can be an indication to exclude malformations of the spinal column, but even absolute absence Ultrasound data indicate spina bifida in 95% of cases.

In asymptomatic cases it is indicated constant surveillance with regular ultrasound and radiographic examination. If the only sign of an abnormality is minor pain, the patient is prescribed conservative treatment. It includes a variety of options using non-steroidal anti-inflammatory drugs and muscle relaxants. The most common NSAIDs include Ibuprofen and Diclofenac.

You cannot prescribe painkillers yourself, as they have a number of contraindications (for example, peptic ulcer). If there is any contraindication, the doctor will select an alternative treatment option. Dehydration therapy is prescribed from time to time. If there is no effect from such treatment within two to three months, surgery is performed (expansion of the foramen magnum, removal of the vertebral arch, etc.). In this case, a strictly individual approach is required to avoid both unnecessary intervention and delays in surgery.

Treatment tactics for each patient require an individual approach

In some patients, surgical exploration is the way to make a definitive diagnosis. The purpose of the intervention is to eliminate compression of the nerve structures and normalize cerebrospinal fluid dynamics. This treatment results in significant improvement in two to three patients. The expansion of the cranial fossa helps to eliminate headaches and restore tactility and mobility.

A favorable prognostic sign is the location of the cerebellum above the C1 vertebra and the presence of only cerebellar symptoms. Relapses may occur within three years after the intervention. Such patients, by decision of the medical and social commission, are assigned a disability.

Medical institutions where you can contact General description

Arnold-Chiari anomaly type I (Q07.0) is a congenital anomaly of the development of the brain, characterized by a displacement of the formations of the posterior cranial fossa into the region of the spinal canal below the level of the foramen magnum.

Prevalence: average 3.3–8.2 per 100 thousand people. The onset of clinical symptoms is observed in adulthood or adolescence. In 50–75% of cases there is a combination with syringomyelia, in 10% with hydrocephalus.

Sometimes this pathology has no clinical symptoms, it becomes an x-ray finding.

Clinical picture

The main symptoms of Arnold-Chiari malformation type I are as follows:

pain in the back of the head, may intensify during coughing, straining (35–50%),
neck pain (15%),
weakness, numbness in the hands (60%),
instability when walking (40%),
double vision, speech impairment, difficulty swallowing - 5–25%,
noise in the head (15%).

An objective examination of the patient can reveal cerebral syndrome, platybasia (50%), nystagmus, diplopia, dysphagia, dysarthria, hypesthesia in the neck, arms, spastic paresis in the arms, revival of deep reflexes, ataxia.

Diagnosis of Arnold-Chiari malformation

Magnetic resonance imaging of the brain, cervical spinal cord.

Consultation with a neuro-ophthalmologist (congestive optic discs).

Differential diagnosis:

Other anomalies of brain development, craniovertebral junction.
hereditary ataxia.
Demyelinating diseases.

Treatment of Arnold-Chiari malformation

Treatment is symptomatic. Prescribed only after confirmation of the diagnosis by a medical specialist. The increase in clinical symptoms is an indication for suboccipital decompression, bypass surgery.

Essential drugs

There are contraindications. Specialist consultation is required.

Ketoprofen (non-steroidal analgesic). Dosage regimen: orally 50 mg situationally, no more than 2 times a week.
Xefocam (non-steroidal anti-inflammatory drug). Dosage regimen: for the relief of acute pain syndrome, the recommended oral dose is 8-16 mg / day. in 2-3 doses. The maximum daily dose is 16 mg. The tablets are taken before meals with a glass of water.
Piroxicam (painkiller, anti-inflammatory drug). Dosage regimen: orally, with meals, 10-20 mg/day. for 1 or 2 doses. If necessary, you can temporarily increase the dose to 30 mg / day, once or in divisible doses, or reduce to 10 mg / day.
Tizanidine (a muscle relaxant central action). Dosage regimen: the initial oral dose is 6 mg (1 capsule). If necessary, the daily dose can be gradually increased by 6 mg (1 capsule) at intervals of 3-7 days. For most patients, the optimal dose is 12 mg per day (2 capsules); V rare cases The daily dose may need to be increased to 24 mg.
Mydocalm (central acting muscle relaxant). Dosage regimen: orally, after meals, without chewing, with a small amount of water, starting with 50 mg 2-3 times a day, gradually increasing the dose to 150 mg 2-3 times a day.

Incidence (per 100,000 people)

What to do if you suspect a disease

Symptoms

Jasmine 2018-05-09 01:38:45

Anastasia 2018-04-10 00:03:51

Dilrabo 2018-03-11 08:24:29

Kolya 2018-03-09 17:00:05

Ernar 2018-03-03 23:29:33

petmat 2018-02-11 19:44:37

Natalia 2018-02-05 21:39:32

Galina 2018-01-05 11:40:40

Natalya Grigorievna 2017-12-16 01:08:53

Christina 2017-12-03 11:13:07

Galina 2017-11-21 08:21:24

Irina 2017-10-23 22:35:30

Alexey 2017-10-16 23:26:59

OLGA 2017-10-05 21:26:17

Tatyana 2017-08-30 00:05:16

Marat 2017-08-14 17:12:44

Julia 2017-07-30 16:30:41

Zarina 2017-07-26 14:21:30

Irina 2017-07-17 18:09:03

Irina 2017-06-08 02:55:33

Svetlana 2017-05-31 04:14:32

Alya 2017-05-26 19:15:55

Anastasia 2017-05-25 14:06:15

Ivan 2017-05-14 16:26:56

Nikolay 2017-04-27 07:57:55

TATYANA 2017-04-16 22:53:45

Irada 2017-04-06 00:29:21

Tatyana 2017-03-31 18:34:28

Ekaterina 2017-03-31 02:35:05

Evgeniya 2017-03-22 20:31:51

Alexander 2017-03-17 02:16:51

Elena 2017-03-16 16:54:43

Elena 2017-03-08 00:19:52

Arsen 2017-02-28 00:50:24

Alexander 2017-02-17 00:54:31

Inna 2017-02-11 20:28:07

Evgeniya 2017-02-04 19:49:25

Olga 2017-02-02 22:21:41

Elena 2017-01-24 21:27:06

Ekaterina 2017-01-19 23:10:06

Tatyana 2017-01-06 17:35:03

Irina 2016-12-24 19:30:51

Hamid 2016-12-22 16:32:02

Valery 2016-12-11 18:06:18

Maria 2016-12-01 16:28:39

Marina 2016-11-28 21:52:12

Anna 2016-11-28 13:55:47

Olga 2016-11-10 03:08:16

SVETLANA 2016-10-23 15:46:39

Marina 2016-10-16 22:07:35

E. Lyudmila 2016-10-14 21:01:43

Yuri 2016-10-14 01:33:14

Natalia 2016-09-23 13:06:28

Julia 2016-09-22 11:44:19

Layla 2016-09-15 21:38:59

Julia 2016-08-30 23:23:56

Maral 2016-08-14 01:37:42

Svetlana 2016-08-11 17:37:43

Olga 2016-07-22 21:58:48

Nadezhda 2016-07-18 01:00:49

Dmitry 2016-06-24 20:51:57

Nadezhda 2016-05-21 21:05:13

Zulikhan 2016-05-11 17:27:33

Valery 2016-05-01 17:44:52

Ruzilya 2016-03-28 23:51:47

Victoria 2015-05-16 22:53:51

Gulnara 2015-02-08 06:23:00

How to poison rats at home using folk remedies

Folk remedies to increase blood pressure at home

Hello! I am diagnosed with Arnold Chiari stage 1, since February 2014 I began to be bothered by symptoms of numbness on the right side, headaches, muscle weakness, and tinnitus. Systems…

Arnold-Chiari malformation is a congenital pathology of the development of the rhomboid brain, manifested by a discrepancy between the sizes of the posterior cranial fossa and the brain structures located in this area, which leads to the descent of the brain stem and cerebellar tonsils into the foramen magnum and their infringement at this level.

In most cases, the defect is combined with hydrocephalus and abnormalities of the spinal cord. The causes may be congenital dysplasia (impairment) of the wide occipital foramen, the dimensions of which become significantly larger than normal.

It was first described by N. Chiari in 1896. This condition is characterized by caudal displacement of the medulla oblongata, pons and cerebellar vermis, when all these structures end up in the cervical part of the spine.

The frequency of this disease ranges from 3.3 to 8.2 observations per 100,000 population.

True frequency various types Arnold-Chiari syndrome, and the frequency of this defect in general, have not been established. One of the reasons for the lack of such data is different approaches to the classification of this defect. According to International classification diseases, Arnold-Chiari syndrome has a separate code (Q07.0), but is defined in it as “... pathological condition, in which there is an increase in intracranial pressure as a result of an intracranial tumor, occlusive forms of hydrocephalus, an inflammatory process, which in some cases leads to herniation of the cerebellum and medulla oblongata into the foramen magnum.” In the ultrasound prenatal literature, it has still not been possible to find descriptions of cases of prenatal diagnosis of Arnold-Chiari syndrome that fully correspond to these characteristics.

The morphological features of various types of Harold-Chiari defect determine the possibilities of prenatal detection and prognosis for life.

The causes of Arnold-Chiari syndrome have not been fully established. Chromosomal abnormalities with this pathology, as a rule, it is not possible to detect.

Pathogenesis(what happens?) during Arnold-Chiari malformation:

To date, the pathogenesis of the pathology has not been definitively established. In all likelihood, these pathogenetic factors three:

the first is hereditarily caused congenital osteoneuropathies,

the second is traumatic damage to the sphenoethmoidal and sphenooccipital part of the clivus due to birth trauma,

the third is a hydrodynamic impact of the cerebrospinal fluid into the walls of the central canal of the spinal cord.

Anatomical features of Chiari malformation

The cerebellum is located in the posterior cranial fossa. (WCH)

The tonsils are the lower part of the cerebellum. Normally they are located above the foramen magnum. In Chiari malformation, the cerebellar tonsils are located below the foramen magnum, in the spinal canal.

The foramen magnum is a kind of border between the skull and the spine, between the brain and the spinal cord. Above the foramen magnum is the posterior cranial fossa, below is the spinal canal.

At the level of the foramen magnum, the lower part of the brain stem (medulla oblongata) passes into the spinal cord. Normally, cerebrospinal fluid (CSF) circulates freely in the subarachnoid spaces of the brain and spinal cord. At the level of the foramen magnum, the subarachnoid spaces of the brain and spinal cord are connected, which ensures the free outflow of cerebrospinal fluid from the brain.

In Chiari malformation, low-lying cerebellar tonsils impede the free circulation of cerebrospinal fluid between the brain and spinal cord. The tonsils block the foramen magnum, like a cork plugs the neck of a bottle. As a result, the outflow of cerebrospinal fluid is disrupted and hydrocephalus develops.

Symptoms of Arnold-Chiari Malformation:

Chiari identified four types of anomalies with a detailed presentation of them. Doctors still use this classification today.

1. Arnold-Chiari malformation type I is a descent of the PCF structures into the spinal canal below the plane of the foramen magnum.

2. With Arnold-Chiari malformation type II, caudal dislocation of the lower parts of the vermis, medulla oblongata and fourth ventricle occurs, and hydrocephalus often develops.

3. Arnold-Chiari malformation type III is rare and is characterized by gross caudal displacement of all structures of the posterior cranial fossa.

4. Arnold-Chiari malformation type IV - hypoplasia of the cerebellum without its downward displacement.

Anomalies of types III and IV are usually incompatible with life.

In approximately 80% of patients, Arnold-Chiari malformation is combined with a spinal cord pathology - syringomyelia, which is characterized by the formation of cysts in the spinal cord, causing progressive myelopathy. These cysts are formed when the structures of the posterior cranial fossa descend and the cervical spinal cord is compressed.

A typical clinical picture of the Arnold-Chiari anomaly is characterized by the following symptoms:

Pain in the cervical-occipital region increases with coughing, sneezing,

Reduced pain and temperature sensitivity in the upper extremities,

Decreased muscle strength in the upper limbs,

Spasticity of the upper and lower extremities,

Fainting, dizziness,

Decreased visual acuity,

In more advanced cases, they join: episodes of apnea (short respiratory arrest), weakening of the pharyngeal reflex, involuntary rapid eye movements.

Possible consequences, complications:

1. Against the background of increasing signs intracranial hypertension(sometimes without it) there are progressive dysfunctions of the cerebellum and compression of the cervical spinal cord, cranial nerve palsies.

2. Sometimes the Arnold-Chiari anomaly is combined with bone defects - occipitalization of the atlas and basilar impression (funnel-shaped depression of the clivus and craniospinal joint).

3. Spinal abnormalities, foot deformities.

Diagnosis of Arnold-Chiari malformation :

Sometimes the Chiari anomaly does not manifest itself in any way and is detected by chance during diagnostic procedures.

IN currently the method of choice in the diagnosis of this pathology is MRI of the brain of the cervical and thoracic spinal cord (to exclude syringomyelia).

Treatment of Arnold-Chiari Malformation :

If the only symptom of the disease is a slight intensity pain syndrome, conservative therapy is used for treatment, which includes various schemes with the use of non-steroidal anti-inflammatory drugs and muscle relaxants.

If there is no effect from conservative therapy within 2-3 months or if the patient has a neurological deficit (numbness, weakness in the limbs, etc.), an operation is indicated.

The purpose of the operation is– laminectomy, decompressive craniectomy of the posterior cranial fossa and dural plasty. With such an operation, the volume of the posterior cranial fossa and the expansion of the foramen magnum increase, as a result of which the compression of the nerve structures and the normalization of the flow of the cerebrospinal fluid stop. In cases of concomitant hydrocephalus, shunt surgery is performed.

In Israel, patients are offered gentle and quality treatment, which after treatment allows patients to conduct full life. Surgical treatment of Arnold-Chiari syndrome is performed using an endoscope, while the traumatic effect of surgical treatment is minimized. A method of minimally invasive surgical treatment performed in Israeli clinics makes it possible for patients with Arnold-Chiari anomaly to lead a full-fledged lifestyle in the future, even without drug support.

Symptomatology of Arnold-Chiari syndrome

Bone and joint congenital anomalies are clinically less obvious on their own and much more so due to their severe complications in the central and peripheral regions. nervous system. Neurological manifestations are tolerated most severely by patients and determine the unfavorable course of this syndrome. In general, the onset of the disease is slow and uncharacteristic.

Symptomatology, initially very blurred and even absent for a long time, is often detected as a result of the intervention of a number of resolving factors, such as traumatic brain injury or infections of the nasopharynx.

The first manifestation that attracts attention to the presence congenital anomaly immediately after childbirth, is the presence of myelomeningocele ( spina bifida). Others are noted later clinical phenomena, indicating the presence of osteoarticular and neurological abnormalities, namely:

spina bifida,
lateral head tilt,
deviation of the eyeballs,
headache and intermittent or passing pain in the neck (especially in older children and adults), appearing with head movements;
nausea, vomiting.

In many patients, due to blockade of the circulation of cerebrospinal fluid between the 4th ventricle and the cisterns of the skull base, ivolutive disease develops already during the first months of life. internal hydrocephalus, causing the appearance of many and varied neurological phenomena. Gradually, the child's skull increases in size and difficulty with feeding appears, as well as respiratory problems, and the meningocele (when it exists) may ulcerate.

Manifestations of intracranial hypertension:

severe headaches,
papillary stasis or atrophy optic nerve(late),
accompanied by progressive impairment of visual function, up to complete blindness.

Cerebellar manifestations:

dizziness;
ataxia when walking and in an orthostatic position;
dysarthria;
swallowing disorder,
intentional trembling,
nystagmus.

Manifestations in the peripheral nervous system:

paresthesia, anesthesia, paresis or spastic paralysis,
enhanced bone-tendon reflexes,
the presence of the Babinski reflex.

Manifestations in the area of cranial nerves:

unilateral or, less commonly, bilateral facial paralysis;
paralysis of the oculomotor nerves, most often expressed by internal strabismus or diplopia.

Diagnosis of Arnold-Chiari syndrome.

Lumbar puncture and biochemical and bacteriological tests cerebrospinal fluid in most cases does not provide significant data. In addition, the use of lumbar puncture can worsen the patient's condition and even cause death due to a sudden decrease in pressure and complete penetration of the cerebellar tonsils and medulla oblongata into the spinal canal.

A routine X-ray examination reveals the following aspects: small posterior cranial fossa; expansion of the foramen magnum and spinal canal; hydrocephalus (large skull with dehiscence); fingerprints on the bone plate of the skull; flattening of the sella turcica; cervical, dorsal and lumbar spina bifida.

Additional X-ray examinations (gas myeloencephalography) are contraindicated in children under 2 years of age. However, in older children and adults, they determine indirect and direct signs of hydrocephalus, displacement of the medulla oblongata and cerebellar tonsils, as well as compression of the spinal cord in the cervical region.

Pathological examination. From an anatomical and topographical point of view, Arnold–Chiari syndrome exists in four well-individualized types, namely:

The first type, in which there is stretching and prolapse of the cerebellar tonsils without displacement of the medulla oblongata. This type occurs most often in older children and adults. WITH clinical point vision, it can remain asymptomatic throughout life and its detection can occur completely by accident.
The second type, in which the lower part of the cerebellum and medulla oblongata is displaced through the foramen magnum into the spinal canal. This type is more common in infants and is clinically manifested by hydrocephalus and often the presence of myelomeningocele.
The third type, with complete penetration of the cerebellum into the myelomeningocele of the cervical vertebrae.
The fourth type, in which hypoplasia of the cerebellum is noted, caused by its total hernia; the cerebellar vermis cannot be distinguished, and the tonsils and the cerebellar patch are barely noticeable. This form is extremely rare.

In addition to the anomaly of the occipital bone and vertebrae affecting the cerebellum and medulla oblongata, there are other cranial and vertebral anomalies, namely the soldering of the first vertebra to the occipital bone, upward movement of the spine into the cranial region due to hypoplasia of the occipital bone; fusion of two or three vertebrae (most often the 2nd and 3rd cervical vertebrae), as occurs in Klippel-Feil syndrome; cervical, dorsal or lumbar spina bifida.

Course and prognosis Arnold-Chiari syndrome . The course of the disease is slow. The appearance of the syndrome in a newborn is incompatible with its survival and its course quickly leads to death.

In the case of a slower course, Arnold-Chiari syndrome is complicated by the appearance of chronic arachnoiditis and parenchymal lesions of the axon; this becomes especially significant when a circulatory disorder occurs in the nervous tissue. Typically, in such cases, neuropsychiatric disorders are noted late and manifest themselves in the form of paraplegia, tetraplegia and delayed mental development.

Treatment Arnold-Chiari syndrome . The only effective treatment is surgery. Indications for surgery are not made on the basis of radiological determination of bone abnormalities, but only when the latter are accompanied by severe neurological manifestations. Surgical intervention consists of occipital craniotomy combined with high laminectomy; the hard shell is dissected and left open. Fibrous adhesions, often extensive, existing around the foramen magnum are intersected and peeled off.

In the presence of severe hydrocephalus, ventricular derivation is performed using a Holter or Pudenz valve (according to the classical method of surgical intervention for hydrocephalus).

Although in some cases, the immediate results are favorable, nevertheless, all surgical interventions with Arnold-Chiari syndrome are associated with a high postoperative risk, due to disorders in the medulla oblongata, which can occur in the immediate postoperative period and often cause death.