Chiari Malformation: Signs and Symptoms

What are the signs and symptoms of Chiari malformation (CMI)?

In children who have CMI, the most common symptom is headache, with or without neck pain (which occurs in 28% to 63% of patients). Typically the neck pain is severe and short-lived, and often related to coughing or straining. Less frequently, the pain is dull and persistent.

In nonverbal children, pain may manifest as persistent crying or irritability, sometimes with hyperextension of the neck or entire spine. There are many other potential symptoms, which sometimes makes diagnosis challenging. Examples include poor balance, eye movement abnormalities, swallowing problems and facial numbness. Spinal cord compression, which may result from fluid within the spinal cord, may cause pain, weakness or sensation changes. One in 5 patients may have dysfunction of nerves of the face or head. This can cause sleep apnea, difficulty speaking, hoarseness, recurrent aspiration and tongue atrophy.

In studies of children with CMI, numbness or sensation loss has been reported in 26% to 50% of patients. Partial loss of strength is present in 12% to 19% of patients, and it is considerably more common in patients who have a fluid collection within the spinal cord.