What is Chiari Malformation?
Also known as Arnold Chiari Malformation, Chiari malformation affects the part of the brain called the cerebellum. There are several different forms of Chiari Malformation but most cases are congenital, meaning they are present from birth.
Although Chiari Malformation can be present at birth, there may not be any symptoms until adulthood. For this reason, Chiari Malformation is often not diagnosed until adulthood.
There is a higher incidence of diagnosis in women than in men, the reasons for which are unknown.
What are the Symptoms?
The most common symptom of Chiari malformation is a headache, which begins at the back of the head (neck) and radiates upward. The pain is often made worse or can be brought on by coughing, sneezing, or straining. These activities are known as valsalva manoeuvres.
Visual problems such as nystagmus (involuntary eye movements), double, or blurred vision may occur. Balance difficulties, vertigo, and dizziness also may be present. Some people may have cranial nerve compression. This can result in apnea (cessation of breathing), gagging, swallowing difficulties, facial numbness, or syncope (temporary loss of consciousness).
Symptoms may present as muscle weakness, particularly in the upper extremities, coordination problems, and gait abnormalities. Imaging of the spine may reveal a fluid collection inside of the spinal cord, known as a syrinx. Some individuals may have hydrocephalus, a build-up of fluid in the ventricles of the brain.