Chiari Syndrome: a malformation of the nervous system with a low prevalence

  • The Austrian Arnold Chiari discovered this disease in 1891.
  • One in two thousand people suffer from this disease.
  • It is considered a rare disease
  • The sufferer loses quality of life
  • The disease is associated with the loss of the smile

Chiari syndrome typology

There are several different types of Chiari syndrome, although the most common are type I and type II.

In the case of type I Chiari syndrome, it is characterized by an abnormal descent of the cerebellar tonsils at least five millimeters below the foramen magnum. In short, the cerebellum would not have enough space in the cranial cavity and therefore tends to protrude into the spinal cord canal about five millimeters.

The cause of this problem is not known for certain, although it is believed that the tissues around the back of the skull are underdeveloped and the cavity is smaller than normal.

Some Chiari are asymptomatic and are discovered at the time of testing for other pathologies.

Signs and symptoms of Chiari syndrome

When the malformation does produce symptoms, they are most common in patients in their 30s and 40s. They are as follows:

  • Head and neck pain that worsens with coughing.
  • Hand and leg coordination problems
  • Sensation problems in the extremities, especially the upper extremities.

In some cases, Chiari syndrome is associated with other malformations, such as syringomyelia or hydrocephalus. In both pathologies there is an accumulation of cerebrospinal fluid, and sometimes there are vertebral anomalies with scoliosis, spinal fusion…

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Diagnosis and treatment of Chiari syndrome

To make the diagnosis of the disease, it is necessary to carry out a physical examination of the patient, as well as a cervical or cranial MRI which indicates whether syringomyelia or hydrocephalus is also present.

If this is not associated with any disease, the diagnosis will be much more complicated.

When it comes to treatment, it usually consists of the application of medication to control pain and educate patients in terms of standard of living. In cases where symptoms are so severe that they limit daily activity, surgery may be considered. In this case, a space is opened to make the fossa larger, and a larger connecting channel, so that the cerebrospinal fluid passes without problems and does not accumulate.

It should also be mentioned that there are other less frequent types of Chiari, each of them being a different disease, for example, type II is associated with spina bifida.