Patients with Chiari and Syringomyelia Frequently Misdiagnosed

Allison Gandey

October 10, 2008

October 10, 2008 — The American Association of Neurological Surgeons (AANS) reports that Chiari and syringomyelia are often misdiagnosed. In a news release, the association reminds clinicians about these conditions, which are challenging to treat and diagnose.

Operative exposure of a Chiari type 1 malformation [Source: American Association of Neurological Surgeons]

Chiari and syringomyelia are frequently mistaken for fibromyalgia or chronic fatigue syndrome. In some cases, patients are told their chronic headaches and muscle weaknesses are psychosomatic.

"Chiari that goes undiagnosed for a long time can lead to real psychological problems in some patients, making the condition even more problematic to treat," AANS spokesperson Ghassan Bejjani, MD, from the University of Pittsburgh Medical Center, in Pennsylvania, said in a news release.

Patients with Chiari type 1 and syringomyelia often present with intense, burning-type nerve pain and headaches, among other symptoms. The prevalence of Chiari in the general population is estimated at slightly less than 1 in 1000.

In many cases, symptoms can be addressed individually through nonsurgical treatments. Some patients can cope with the symptoms without any major problems, whereas others are severely affected by the condition, Dr. Bejjani explained.

Chiari is considered a congenital condition, although acquired forms have been diagnosed. Symptoms vary greatly, depending on the type of Chiari and the individual case, but can include:


  • Severe head and neck pain.

  • Occipital headache experienced at the base of the skull made worse by coughing, sneezing, or straining.

  • Loss of pain and temperature sensation of the upper torso and arms.

  • Loss of muscle strength in the hands and arms.

  • Collapsing because of muscle weakness.

  • Spasticity.

  • Dizziness.

  • Balance problems.

  • Double or blurred vision.

  • Hypersensitivity to bright lights.

Chiari malformations are closely related to syringomyelia.

In an interview with Medscape Neurology & Neurosurgery, Dr. Bejjani pointed out that in cases where surgery is required, the failure rate can be as high as 10% to 30%. "This can be very challenging," he said, "and clinicians really have to weigh the benefits and risks."

Dr. Bejjani added: "It is really key that patients with either of these conditions work closely with their neurosurgeons and other healthcare providers on pain-management strategies because, while surgery may help, it does not always provide complete resolution of symptoms."

The researchers have disclosed no relevant financial relationships.


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