Written by David Hoh, editor of The CFIDS Chronicle

Spine, skull surgery may help many with CFIDS

Chiari malformation or squeezing of spinal cord
may be common in CFIDS, fibromyalgia

For some patients with CFIDS and fibromyalgia, the crux of their problems may be all in the backs of their heads. New research is focusing attention on neurological conditions in which the brain stem or upper portion of the spinal cord is compressed. All the signals that go from the brain to the body and vice versa must pass through this narrow passageway, just about a half an inch in diameter. When this nerve passageway is squeezed, a person can experience the same assortment of symptoms that are familiar to persons with CFIDS and fibromyalgia.

The best known of these conditions is the Chiari malformation, in which the cerebellar "tonsils" (a portion of the cerebellum, shaped like the tonsils in the neck) extend several millimeters through the opening in the base of the skull (the foramen magnum) that allows the spinal cord to attach to the brain. This puts pressure on the brain stem and spinal cord. In a less well recognized but perhaps much more common condition known as cervical stenosis, the spinal canal appears normal but is actually too narrow for the spinal cord. Sometimes a condition called syringomyelia develops, in which a cyst grows in the spinal canal, putting greater pressure on the spinal cord.

Symptoms from these conditions often don't develop until adulthood, when the compression may grow more severe or may be triggered by an injury such as whiplash, surgery that involves hyperextending the neck, or prolonged coughing. Thus, onset of symptoms may be gradual or sudden. And symptoms may vary widely according to the individual.

Sound familiar? Difficult diagnosis, vague symptoms— but there is an enormous benefit to patients when this diagnosis can be made. There is an accepted and generally effective treatment.

Treatment for these conditions is surgery to expand the space available for the brain stem and spinal cord. This is done by removing bone from the skull and/or the cervical (top seven) vertebrae. Neurosurgeons who perform the surgery report that most patients experience significant and broad improvement of symptoms, beginning almost immediately following surgery and progressing with time as the spinal cord recovers from the compression. While it is not at all clear whether surgery can relieve all the symptoms associated with CFIDS and fibromyalgia, some fibromyalgia patients who have undergone the decompression surgery have reported that their tender points completely disappeared.

"We're very hopeful that this will be the first real, viable treatment for many people," said Rae Gleason, director of the National Fibromyalgia Research Association (NFRA) in Salem, Oregon. The NFRA is funding a $150,000 study to determine the percentage of fibromyalgia patients who have a Chiari malformation or spinal cord compression.

"The treatment is not 100%," Gleason said. "Each person gets back a different kind of quality. Of the people I've talked to, the most dramatic improvement has been that headaches are gone. Number two, fatigue is greatly decreased, and flareups seem to be limited. For some people, the irritable bowel syndrome is basically gone. So the relief comes in different ways."

At this point, the optimism needs to be tempered with good science. "This is not yet something we can tell people to run out and do. I think we will find a high percentage (for whom the surgery will be appropriate), but it will not be the answer for everyone," Gleason said.

Neurosurgeon Dr. Michael Rosner of Charlotte, N.C., found the possible connections between CFIDS/fibromyalgia and spinal cord compression in the process of diagnosing and treating a physician who was disabled by CFIDS. He agreed that it's premature for patients to start seeking diagnosis and treatment from their local neurosurgeon because awareness of the possible connection between the condition (absent the actual herniation of the cerebellar tonsils) and CFIDS/fibromyalgia is still low. Research is just beginning to be published on this topic.

"We're looking at this as a subset of patients," Dr. Rosner said. "Fibromyalgia and chronic fatigue syndrome may be many diseases, but clearly there is a big chunk of them who may be surgical (candidates)."

The Type I Chiari malformation (Type II is related to spina bifida and hydrocephalus and is found in infants) was first identified in 1891 and was considered to be rare before the development of MRI scans. Even with MRI scans, however, the diagnosis is frequently missed because of the way radiologists usually scan the neck. They're looking for herniation of the cerebellar tonsils, Dr. Rosner explained, but a spinal canal or foramen magnum that is congenitally narrow, not misshapen, would be reported as normal. Rosner said MRI scans typically do not account for the curvature of the spine and therefore make the diameter of the spinal canal appear larger than it really is. However, even MRIs that are done according to a protocol designed to find compression of the spinal cord may provide vague results. Therefore, Dr. Rosner explained that a judgement on whether to perform surgery is usually made with a combination of MRI scan and neurological testing.

Dr. Rosner believes that neurally mediated hypotension (NMH), which is associated with CFIDS, may well prove to be a "good objective marker" for cervical stenosis.

"The real diagnostic clue is anything that signals neurological impairment— abnormal reflexes, tingling in both arms or both legs, shooting pain, urinary frequency, inability to stand on one foot, ataxia (coordination problems), dropping things out of hand," Rosner said.

Fatigue and pain alone are not enough to suggest this condition, he said. In fact, before he became aware of CFIDS through that disabled physician, he would have dismissed someone who complained of being tired all the time. Now, he interviews the patient to find out the range of symptoms and what set them off.

"When you hyperextend the neck backward," Dr. Rosner explained, "the spinal canal narrows. This happens in the case of whiplash in an automobile accident, extended dental work in which the head is bent back, and coughing severely for an extended period of time, even something like painting a ceiling." Interestingly, the surgery in breast implantation requires the head to be positioned backward while the patient is unconscious and unaware of any pain in the neck. At the same time, Rosner said, blood pressure and oxygen delivery to the spine and brain stem is lower.

"Most people get better (from these kinds of injury) on their own; some don't get better and they may need surgery."

In a paper expected to be published in May 1999 in the journal Neurology, Dr. Thomas Milhorat of the State University of New York in Brooklyn reports his experience with Chiari and related spinal compression. Of 364 Chiari patients he surveyed, nearly 60% had a prior diagnosis of fibromyalgia, 12% of chronic fatigue syndrome, 31% migraine or sinus headache, 9% multiple sclerosis and 63% psychiatric or malingering (some had more than one prior diagnosis). In another study, Dr. Rosner reported that 20% of the fibromyalgia patients he examined had cervical compression.

The University of Missouri is beginning a broad-based study of Chiari. It will attempt to characterize the wide variety of symptoms, analyze the MRI features, and define the short- and long-term outcomes following treatment. Pre-operative evaluations will be tracked and compared with outcomes, which will be measured at one month, three months, one year, five years and 10 years. It appears patients in this study will be limited to those whose MRI scans reveal the classic herniated cerebellar tonsils. (See website fo more information.)

Of greatest interests to patients with CFIDS and/or fibromyalgia, however, is a study funded by the NFRA to determine the percentage of people diagnosed with fibromyalgia who also have the Chiari malformation or spinal cord compression. This study will involve 105 newly diagnosed fibromyalgia patients, 30 of whom will be matched by age and sex with 30 healthy controls. Patients will be selected at three sites— Oregon Health Sciences University, Dr. Robert Bennett; The University of Texas, Dr. I. Jon Russell; and Georgetown University, Dr. Dan Clauw. Each will be given an extensive neurological examination. MRI scans, done according to Dr. Rosner's specifications, will all be read "blind" by a radiology clinic in Charlotte.

Preliminary results should be presented and discussed in September at an NFRA research meeting. Dr. Rosner, who will chair that meeting, will present data from his own clinical experience, as well.

Other research linking Chiari and cervical stenosis to fibromyalgia is being submitted to the American College of Rheumatology for possible presentation at its upcoming meetings.

The National Fibromyalgia Research Association raises funds for fibromyalgia research. For information, send a self-addressed, stamped envelope to P.O. Box 500, Salem, OR 97308.

The symptoms of Chiari or spinal cord compression may include:

• Headache in the back of the head that may radiate behind the eyes and into the neck and shoulders.
• Disordered eye movements, vision changes.
• Dizziness, autonomic symptoms (orthostatic intolerance, NMH).
• Muscle weakness.
• Unsteady gait.
• Cold, numbness and tingling in the extremities.
• Chronic fatigue.
• Tinnitis (ringing, buzzing or watery sounds in the ears).
• Sleep apnea.
• Speech impairment.
• Hearing loss.
• Gastrointestinal problems, irritable bowel syndrome, frequent urination.
• Lack of gag reflex, difficulty swallowing.
• Symptoms are exacerbated by exertion, and especially by leaning the head backward or coughing.

Web sites of interest:

The Chiari Information Exchange

A chapter from an on-line neurosurgery textbook by John Oro, MD, University of Missouri, Columbia

New study at University of Missouri, with summary of Chiari I malformation

One patient's experience with Chiari surgery

The Chiari Malformation Page by Neil Feldstein, MD, Columbia-Presbyterian Medical Center, New York

World Arnold-Chiari Malformation Accociation

American Syringomyelia Alliance Project

National Fibromyalgia Research Association - comments by Dr. Michael Rosner