Arnold-Chiari syndrome, also referred to as Chiari malformation, relates to the dysfunction of the brain. In this syndrome, the cerebellar tonsils are pushed downward through the foramen magnum of the skull. This condition can also result in non-communicating hydrocephalus, as the cerebrospinal fluid cannot flow out of the brain smoothly. Due to this blockage, people with Arnold-Chiari syndrome can suffer from headaches, experience difficulty while swallowing, apart from nausea, dizziness, fatigue, muscular weakness in the face and head, and problems with coordination. If the syndrome is particularly severe, it could result in paralysis too.
While Arnold-Chiari malformation is rare, improvements in diagnostic tools have led to more people being diagnosed with this condition. There are different degrees of the syndrome and the most commonly diagnosed are Type 1 and Type II malformations. In the former, the condition manifests itself as the brain and skull grow, with the symptoms appearing in late childhood or early adulthood. In Type II, the condition is present at birth.
The treatment depends on the type of malformation, symptoms and the severity of the condition. At times, no treatment is required; at other instances, monitoring, surgery and medications are the different treatment options.
Typically, this syndrome is categorised into three different types based on the amount of displacement of the brain, and whether the spine or brain have any development abnormalities. In several people, the syndrome is discovered during tests for some other unrelated health problems, as they do not experience any sign or symptom of the condition. Hence, such people do not require any treatment. In others, the symptoms are dependent on the severity of the displacement.
Typically, there are three types of Arnold-Chiari malformations. These are Type I, Type II and Type III. Type I and II are less serious compared to Type III, which albeit rare can have a devastating effect on the child.
The most common symptom associated with this type of malformation is a severe headache. The headaches usually come on all of a sudden once the person sneezes, coughs or strains. In addition, the person may also have other symptoms like:
Some people with Type I Chiari malformation may also experience the following symptoms:
This type is congenital and is more severe than Type I. Usually, the baby also will have a form of spina bifida along with the malformation. In such babies, the spine and the spinal canal do not close completely before birth. Symptoms of Type II include the following:
This is the most severe kind of Chiari malformation. In this type, usually a part of the cerebellum or brainstem pushes through the opening located at the anterior part of the skull into the spinal canal. This type has a high rate of mortality and if the babies survive, they would invariably suffer from severe neurological problems.
If a person exhibits any of the aforementioned symptoms, he/she should immediately consult a specialist, as these medical signs could be related to Arnold-Chiari syndrome.