Modern medicine can explain and cure the symptoms of various diseases. However, many phenomena occurring in the human body remain poorly understood, especially in cases where the disorders affect the brain. One of these pathological features is considered Arnold Chiari syndrome.

The causes and mechanism of development of Arnold-Chiari syndrome

Arnold-Chiari Syndrome (SAC) includes a number of pathologies that affect the human brain. Often, the disturbances concern the cerebellum, which is responsible for the sense of balance.

The list also includes anomalies of the medulla oblongata leading to malfunctioning of the functionality of the centers responsible for respiration, the work of the heart, blood vessels and nervous system.

A typically developed cerebellum along with tonsils is located within the cranium just above the occipital foramen. The progressive anomaly of Arnold-Chiari forces the cerebellar tonsils to lower, squeezing a bridge.

Some statistics. According to medical research, this syndrome affects an average of 3-8 patients out of 100 thousand.

The specific causes affecting the manifestations of the Arnold-Chiari malformation are not considered to be thoroughly studied to date. Initially, doctors were sure that this was an exclusively congenital pathology. However, most experts came to the conclusion that there are much more patients with acquired pathology than with congenital ones.

Congenital causes of SAH:

  1. Atypical development of cranial bones. The latter do not have time to grow simultaneously with the brain and in the place where the cerebellum is located in the normally formed skull, people who are prone to the syndrome have an excessively small cranial fossa.
  2. Excessively large occipital foramen.

Note. The possibility of Arnold-Chiari syndrome in the fetus can occur in almost every pregnancy.

A special risk group includes expectant mothers exposed to such factors:

  • excessive use of drugs when bearing a child;
  • smoking, drinking during pregnancy;
  • frequently transmitted viral diseases while waiting for the baby.

All of these factors can affect the development of the unborn child is not the best way. In some cases, a woman may be shown abortion.

Acquired reasons:

  1. Birth injuries to the baby’s skull.
  2. Damage to the spinal cord of a baby with a fluid called cerebrospinal fluid. The result is a stretching of the central cerebral canal.

Types and severity of anomalies

The Arnold-Kari anomaly is divided into the following degrees:

  1. Cerebellar tonsils in the occipital region are lowered below the occipital foramen.
  2. Refers to congenital forms, as it progresses during embryonic development. Violation is tracked by instrumental methods. This degree of pathology can be marked by hydrocephalus.
  3. The medulla oblongata and cerebellum are lowered below the occipital area of ​​the meningocele.
  4. The cerebellum is underdeveloped.

NAO 3 and 4 are considered fatal.

Symptoms of the Syndrome

Grades 1 and 2 of SAH are recognized as more common than 3 and 4, since, in fact, these are different pathologies.

Symptoms of milformation of type 1 and 2:

  • persistent headache;
  • dizziness;
  • nausea;
  • loss of sensation in the hands;
  • pain in the neck;
  • difficulty swallowing;
  • a feeling of double vision;
  • blurry speech
  • impaired breathing, in newborns - noisy and wheezing.

Symptoms of stage 3-4:

  • a feeling of double vision;
  • blindness, loss of consciousness (often manifested when turning the head);
  • impaired coordination of the limbs, their tremor;
  • problems with urination.

Diagnosis of brain pathology

The diagnosis can be made in the first weeks of a child’s life based on the degree of malformation. It is also possible to track the pathology in an adult, which sometimes becomes a complete surprise, in view of the frequent absence of pronounced signs.

 

In the absence of patient complaints, the Arnold-Chiari anomaly is diagnosed according to the results of the planned examinations: ECHO-EG, EEG, REG. Such events help to identify increased intracranial pressure and abnormalities of the cranial bones. In complicated syndromes, the patient is prescribed an MRI or CTG of the brain. Additionally, spinal tomography may be needed.

Arnold - Chiari malformation treatment

Arnold-Chiari malformation is treated in two ways: surgical and non-surgical. Determining the specific course of therapy depends on the specific symptoms and severity of the SAH.

Non-surgical treatment

The mild symptoms of Arnold-Chiari pathology suggest regular preventive examinations of specialists. In the presence of mild pain in the nape of the neck, the patient can be prescribed painkillers or anti-inflammatory drugs, as well as medications that help relax muscles. Patients with a similar diagnosis are shown physiotherapy exercises aimed at improving muscle coordination and eliminating tremors. Sometimes you may need classes with a speech therapist.

Surgery

If non-surgical therapy does not bring relief, surgical intervention is recommended.

Neurosurgery helps to correct defects that cause brain compression. If successful, malformation ceases to progress and the condition stabilizes.

Treatment of Arnold-Chiari syndrome of the 1st degree is to eliminate a bone fragment in the posterior part of the fossa of the skull. Intervention lasts approximately an hour, and recovery takes no more than a week. The result of the operation is the expansion of space for the brain - it becomes more extensive, and the pressure inside the skull decreases. The 2nd degree of pathology is amenable to therapy by shunting and closing myelomeningocele.

Forecast and Life Span

Arnold-Chiari syndrome is complicated by the parallel accession of hydrocephalus, paralysis and paresis.

 

The prognosis of the disease, as well as the life expectancy of patients, largely depends on the characteristics of the course of the SAH and its severity. No less important is timely surgical intervention. People who have been diagnosed with grades 1 and 2 have an average life expectancy, since the pathology can occur without any symptoms. If 1 or 2 degrees are accompanied by neurological symptoms, it is important to carry out the operation as soon as possible. Otherwise, the resulting complications of the spinal cord and brain will make the pathology not amenable to therapy.

After the operation, many patients can live a normal life. Sometimes complications arise that can lead to additional health problems. With repeated manifestations of milformation, the patient can again plan the operation.

The Arnold-Chiari anomaly is not a very common disease, the prognosis of which largely depends on the severity of the disease. So, if the symptoms of the disease are insignificant, the patient has a great chance of recovery and the maintenance of a full future life.