May - June 1999
Spine, skull surgery
may help many with CFIDS
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 (see the following).
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.
Cold, numbness and tingling in the extremities.
- Tinnitis (ringing, buzzing or watery sounds in the ears).
- 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.
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.
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
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.
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
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.
Dr. Michael Rosner of Charlotte, N.C., found the possible connection 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
"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
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.
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 the hands," 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, 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.
people get better (from those kinds of injury) on their own; some don't get better and they may need surgery."
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 web site for more information.)
Of greatest interest 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.
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.
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 envolope to P.O. Box 500, Salem,
Web sites of
The following web sites offer
more information about Chiari malformation and the surgical techniques used to correct it.