Arnold-Chiari malformation- type 1

December 31, 2014

Synonyms

Overview

Chiari malformation type 1 is a structural abnormality of the cerebellum, the part of the brain that controls balance. It involves the extension of the lower part of the cerebellum into the foramen magnum (the large hole at the base of the skull which allows passage of the spinal cord), without involving the brainstem. Normally, only the spinal cord passes through this opening. This malformation is the most common type of Chiari malformation and may not cause any symptoms. Depending on the symptoms present and severity, some individuals may not require treatment while others may require pain medications or surgery.

Symptoms

Upper motor neurone and cerebellar signs
Clumsiness
Awkward
Drunken like gait/walking difficulties
Bilateral problems with co-ordination of movement
Headache, neck pain, unsteady gait usually during childhood

Causes

Primary or congenital Chiari malformations are caused by structural defects in the brain and spinal cord that occur during fetal development. The underlying cause of the structural defects are not completely understood, but may involve genetic mutations or lack of proper vitamins or nutrients in the maternal diet.

Less frequently, Chiari malformation type 1 is acquired after birth. Causes of acquired Chiari malformation type 1 involve the excessive draining of spinal fluid from the lumbar or thoracic areas of the spine as a result of injury, exposure to harmful substances, or infection.

Prognosis

The prognosis for someone diagnosed with Chiari malformation type 1 is often unknown. Many people with this condition do not have any symptoms, and it is not possible to predict if symptoms will develop in the future. Other affected individuals may experience dizziness, muscle weakness, numbness, vision problems, headache, or problems with balance and coordination. In these individuals, it is not always known if symptoms will get worse over time. It is important for people with Chiari malformation type 1 to receive regular medical care so they can be monitored by a physician for any new symptoms.

There are some treatments that may help reduce or eliminate the symptoms of Chiari malformation type 1. Medications may ease certain symptoms, such as pain. Surgery is another treatment option, but it is typically reserved for people with severe symptoms. Many patients who have had surgery see a reduction in their symptoms and/or prolonged periods of relative stability.

Treatment

Some individuals with Chiari malformation type 1 are asymptomatic and do not require treatment. Individuals who have minimal symptoms, without syringomyelia, can typically be treated conservatively. Mild neck pain and headaches can usually be treated with pain medications, muscle relaxants, and the occasional use of a soft collar.

Individuals with more severe symptoms may be in need of surgery. Surgery is the only treatment available to correct functional disturbances or halt the progression of damage to the central nervous system. The goals of surgical treatment are decompression of the point where the skull meets the spine (the cervicomedullary junction) and restoration of normal flow of cerebrospinal fluid in the region of the foramen magnum (the hole in the bottom of the skull where the spinal cord passes to connect to the brain). Prognosis after surgery for the condition is generally good and typically depends on the extent of neurological deficits that were present before the surgery. Most individuals have a reduction of symptoms and/or prolonged periods of relative stability. More than one surgery may be needed to treat the condition.