Chiari malformation
Chiari malformation (pronounced kee-AR-ee mal-for-MAY-shun) is a condition in which brain tissue extends into the spinal canal. It occurs when part of the skull is abnormally small or misshaped, pressing on the brain and forcing it downward.
Etymology
The condition is named after Hans Chiari, an Austrian pathologist, who first described it in the late 19th century.
Types
Chiari malformation is categorized into four types, depending on the anatomy of the brain tissue that is displaced into the spinal canal, and whether developmental abnormalities of the brain or spine are present.
- Type I Chiari malformation is the most common and occurs during fetal development, causing the lower part of the cerebellum to be pushed downward into the spinal canal. Symptoms may not occur until late childhood or adulthood.
- Type II Chiari malformation, also known as Arnold-Chiari malformation, is usually present at birth and is associated with spina bifida.
- Type III Chiari malformation is the most serious form, involving the protrusion or herniation of the cerebellum and brain stem into the spinal canal.
- Type IV Chiari malformation involves an incomplete or underdeveloped cerebellum—a condition known as cerebellar hypoplasia.
Symptoms
Symptoms vary by type and patient, but may include headaches, especially after sudden coughing, sneezing, or straining; neck pain; unsteady gait; poor hand coordination; numbness and tingling of the hands and feet; dizziness; difficulty swallowing, sometimes accompanied by gagging, choking, and vomiting; speech problems, such as hoarseness.
Diagnosis
Diagnosis of Chiari malformation is often made through magnetic resonance imaging (MRI) or computed tomography (CT) scan.
Treatment
Treatment options depend on the type, severity, and associated symptoms. Regular monitoring, medications, and surgery are treatment options. In many cases, surgery can reduce or eliminate symptoms.
Related terms
- Syringomyelia: a disorder in which a cyst forms within the spinal cord. This cyst, called a syrinx, can expand and elongate over time, destroying the spinal cord.
- Hydrocephalus: a condition in which an accumulation of cerebrospinal fluid (CSF) occurs within the brain. This typically causes increased pressure inside the skull.
External links
- Medical encyclopedia article on Chiari malformation
- Wikipedia's article - Chiari malformation
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