General Practice 2022

SíArnold-Chiari syndrome: what é, types, symptoms and treatment

Table of contents:

SíArnold-Chiari syndrome: what é, types, symptoms and treatment
SíArnold-Chiari syndrome: what é, types, symptoms and treatment

Arnold-Chiari syndrome is a rare genetic malformation in which the central nervous system is compromised and can result in balance difficulties, loss of motor coordination and visual problems.

This malformation is more common in women and normally occurs during the development of the fetus, in which, for an unknown reason, the cerebellum, which is the part of the brain responsible for balance, develops improperly. According to the development of the cerebellum, Arnold-Chiari syndrome can be classified into four types:

  • Chiari I: It is the most frequent and most observed type in children and occurs when the cerebellum extends to a hole at the base of the skull, called the foramen magnum, where normally it should pass only the spinal cord;
  • Chiari II: It happens when, in addition to the cerebellum, the brainstem also extends to the foramen magnum. This type of malformation is more common to be seen in children with spina bifida, which corresponds to a failure in the development of the spinal cord and the structures that protect it. Learn more about spina bifida;
  • Chiari III: It happens when the cerebellum and brainstem not only extend to the foramen magnum but also reach the spinal cord, this malformation being the most serious, despite to be rare;
  • Chiari IV: This type is also rare and incompatible with life and occurs when there is no development or incomplete development of the cerebellum.

The diagnosis is made based on imaging tests, such as magnetic resonance imaging or computed tomography, and on neurological examinations, in which the doctor performs tests to assess the person's motor and sensory capacity, in addition to balance.

Main symptoms

Some children who are born with this malformation may not show symptoms or present when they reach adolescence or adulthood, being more common after the age of 30. Symptoms vary according to the degree of involvement of the nervous system, and can be:

  • Neck pain;
  • Muscle weakness;
  • Balance difficulty;
  • Change of coordination;
  • Loss of sensation and numbness;
  • Visual change;
  • Dizziness;
  • Increased heart rate.

This malformation is more common during fetal development, but it can occur, more rarely, in adult life due to situations that can decrease the amount of cerebrospinal fluid, such as infections, blows to the head or exposure to toxic substances.

The diagnosis by a neurologist based on the symptoms reported by the person, neurological exams, which allow the evaluation of reflexes, balance and coordination, and analysis of computed tomography or magnetic resonance imaging.

How the treatment is done

Treatment is done according to symptoms and their severity and aims to relieve symptoms and stop the progression of the disease. If there are no symptoms, there is usually no need for treatment. In some cases, however, the use of medication to relieve pain, such as Ibuprofen, for example, may be recommended by the neurologist.

When the symptoms appear and are more severe, interfering with the person's quality of life, a surgical procedure may be indicated by the neurologist, which is performed under general anesthesia, with the objective of decompressing the spinal cord and allow circulation of cerebrospinal fluid.In addition, physiotherapy or occupational therapy may be recommended by the neurologist to improve motor coordination, speech and coordination.

Popular topic