Published with the kind permission of the National Fibromyalgia Research Foundation, USA.

 

CHIARI MALFORMATION IN FIBROMYALGIA PATIENTS


Abstracts from the National Fibromyalgia Research Foundation Conference in
Portland Oregon, September 26-27, 1999


CAN SPINAL CORD COMPRESSION CAUSE THE FIBROMYALGIA SYNDROME?


Dan S. Heffezą M.D., Daniel G. Malone˛ M.D., Sam R. Bannerą M.D., Alan
Shepardą M.D., Ruth E. Rossą Ph.D. and James W. Robertsoną B.S. (Sponsored by
Daniel Malone)ą Chicago Institute of Neurosurgery and Neuroresearch, Chicago,
IL 60614 and ˛University of Wisconsin at Madison, Madison, WI 53706


The symptoms of cervical myelopathy mimic those of fibromyalgia. We
established a prospective database to catalogue neurological findings in
patients with fibromyalgia to examine the possible etiological role of spinal
cord compression. The principal criterion for referral is cervical spinal
canal or foramen magnum stenosis on a screening MRI scan. Patients complete a
detailed questionnaire regarding current symptoms and past medical care and
are examined by a neurologist and a neurosurgeon. Measures of balance,
strength, coordination, and cognitive function are obtained using a battery
of standardized tests. To date, forty-five consecutive fibromyalgia patients
(87% female, median age 44 years, median duration of illness 6 years) have
been evaluated for myelopathy. Neurological symptoms included fatigue (93%),
fatigue upon exertion (98%), cognitive impairment (96%), diffuse pain (93%),
headache (89%), weakness (89%), impaired balance (80%), paresthesiae (82%),
clumsiness (71%), numbness (73%), dizziness (62%), and diplopia (71%).
Neurological signs included hyperreflexia (80%), spinothalamic sensory level
(79%), recruit of reflexes (44%), impaired tandem walk (32%), positive
Romberg sign (31%), clonus (28%), Hoffman sign (26%), dysdiadokokinesia
(28%), impaired position sense (19%) and dysmetria (19%). Only 3 patients had
a normal neurological examination. Detailed MRI imaging of the cervical spine
and foramen magnum revealed cervical stenosis (n=21), brainstem compression
due to tonsillar ectopia (n=12) or, both cervical stenosis and tonsillar
ectopia (n=11). We conclude that some patients with fibromyalgia have
cervical myelopathy on the basis of spinal cord or cervicomedullary
compression. We recommend a thorough neurological examination and a screening
MRI scan of the cervical spine and brain in all patients with fibromyalgia
who do not respond to conventional medical therapy.


PATIENTS WITH FIBROMYALGIA HAVE QUANTIFIABLE
NEUROLOGICAL DEFICITS


Pamela S. Johnsoną M.S./P.T., Helen Bourke-Taylorą O.T.R./L., Dan s. Heffezą
M.D., Daniel G. Malone˛ M.D., Ruth E. Rossą Ph.D. and James W. Robertsoną
B.S. (Sponsored by Daniel Malone) ąChicago Institute of Neurosurgery and
Neuroresearch, Chicago, IL 60614 and ˛University of Wisconsin at Madison,
Madison, WI 53706


An association has been proposed between fibromyalgia and cervical
myelopathy. As part of an investigation of a possible neurological etiology
of fibromyalgia, 42 consecutive patients with fibromyalgia were evaluated
using a series of standardized tests of neurological function. We assessed
upper extremity function including coordination and dexterity (Jebsen-Taylor
Hand Test and nine-hole peg test) and strength (Jamar dynamometer and pinch
gauge). Mobility and static and dynamic balances were assessed using the Berg
Balance Scale and timed measures of ambulation. The median age of patients
was 44 years. Patients carried the diagnosis of fibromyalgia for a median of
6 years. Eighty-seven percent of patients were female.
Data analysis indicated that 68% of patients had balance deficits as shown by
the Berg Balance Scale, including impaired functional reach (31%), tandem
stance (24%) and single limb stance (26%). The sample size allowed for the
analysis of the Jebsen-Taylor hand test of dexterity only for the 30
right-handed females; a statistically significant slowing as compared to
accepted standard of normal was identified in 6 of 7 subtests (p<.01).
Forty-six percent and 33% of patients fell below the 25th percentile on
nine-hole peg testing of the dominant and non-dominant hands, respectively.
Tests of grip strength and dexterity showed a lack of the normal dominance
pattern in 60% of patients.
We conclude that some patients with fibromyalgia have neurological
dysfunction that can be objectively quantified. This database will allow for
prospective objective analysis of the response of fibromyalgia to the
treatment of cervical myelopathy.


SURGICAL TREATMENT OF MYELOPATHY RELIEVES SOME
SYMPTOMS OF FIBROMYALGIA


Dan S. Heffezą M.D., Sam R. Bannerą M.D., Daniel G. Malone˛ M.D., Alan
Shepardą M.D. and Ruth E. Rossą Ph.D. (Sponsored by Daniel Malone) ąChicago
Institute of Neurosurgery and Neuroresearch, Chicago, IL 60614 and
˛University of Wisconsin at Madison, Madison, WI 53706


An association has been suggested between fibromyalgia and cervical
myelopathy. If so, treatment of myelopathy could relieve some symptoms of
fibromyalgia. Twenty-two fibromyalgia patients with cervical myelopathy (82%
female, mean duration of illness 6.1 years +/-4.49) were evaluated. Symptoms
included fatigue (100%), cognitive impairment (100%), exertion intolerance
(95%), diffuse pain (86%), headache (86%), clumsiness and instability (91%),
nonrestorative sleep (82%), nausea (64%), dizziness (59%) and numbness (59%).
Neurological signs included hyperreflexia (86%), recruitment of reflexes
(46%), impaired tandem walk (41%), positive Romberg sign (37%), spinothalamic
sensory level (32%), nystagmus (27%) and appendicular ataxia (23%). MRI
imaging revealed foramen magnum stenosis due to cerebellar tonsillar
herniation (n=8), cervical spinal stenosis (n=7) or both (n=7). Surgical
decompression of the foramen magnum (n=13), the cervical spinal canal (n=7)
or both (n=2) was performed. After a minimum 6-month follow-up, (range:
0.5-2.5 years), 81% of patients reported sustained improvement in the
distribution and intensity of pain. Cognitive dysfunction (75%), dizziness
(77%), numbness (77%), fatigue (70%), headache (68%), nausea (65%), capacity
for exertion (65%) and quality of sleep (50%) also improved. Improved
performance on neurological examinations was noted. We conclude that
fibromyalgia patients with myelopathy can experience an improvement in
symptoms of fibromyalgia following surgical decompression of the cervical
spine or foramen magnum. We have developed a database to identify any
etiological link between cervical myelopathy and fibromyalgia.


PAIN SYNDROMES AND C-SPINE/FORAMEN MAGNUM CORD COMPRESSION. TWO YEAR EXPERIENCE OF A UNIVERSITY-BASED RHEUMATOLOGIST


Daniel G. Malone, M.D., University of Wisconsin, Madison, WI
At the September 1997 seminar sponsored by the National Fibromyalgia Research
Association

I learned of the connection between fibromyalgia/CFIDS/chronic
pain, and cervical spine/foramen magnum compression abnormalities. Since that
time I have done thorough neurological examinations on nearly all patients
referred to me with chronic pain. Total patients seen with pain syndromes =
335, and those evaluated neurologically = 271. Of the 271, neurological
abnormalities resulted in 144 recommendations for C-spine MRI, done according
to a special protocol to assess true canal diameter at each cervical level.
Axial cuts were made through the foramen magnum in a plane parallel to the
foramen magnum to assess true cerebellar tonsillar ectopia. Eighty-eight such
MRIs were done. Almost all were interpreted by the UW radiology staff as
normal, as showing only minimal disc bulging, as showing DDD/DJD, or mild
thecal sac effacement. Only one was read as showing a Chiari malformation,
and 7 as showing frank stenosis of a moderate or severe degree. In contract,
79 of these MRIs were interpreted by the author and by Dr. Dan S. Heffez: 12
- normal, 16 - minimal abnormality, 18 - significant cerebellar tonsillar
ectopia, 21 - significant stenosis, and 24 - stenosis and ectopia.
Thirty-nine patients were seen and evaluated by Dr. Heffez. Twenty-three had
at least one operative procedure done, and three were not considered surgical
candidates. Of the remaining 3, surgery was recommended for 11, and follow-up
with possible surgery for 2.


CHIARI I MALFORMATION REDEFINED:

Clinical and Radiographic Findings for 364
Symptomatic Patients
Thomas H. Milhorat, M.D., Mike W. Chou, M.D., Elizabeth M. Trinidad, M.D.,
Roger W. Kula, M.D., Menachem Mandell, M.D., Chantelle Wolpert, M.B.A.,
P.A.-C., Marcy C. Speer, Ph.D.
Departments of Neurosurgery (THM, MWC, EMT), Neurology (RWK), and Radiology
(MM), State University of New York Health Science Center at Brooklyn,
Brooklyn, New York; The Long Island College Hospital (THM, MWC, EMT, RWK),
Brooklyn, New York; and the Department of Medicine (CW, MCS), Section of
Medical Genetics, Duke University Medical Center, Durham, North Carolina


OBJECTIVE: Chiari malformations are regarded as a pathological continuum of
hindbrain maldevelopments characterized by downward herniation of the
cerebellar tonsils. The Chiari I malformation (CMI) is defined as tonsillar
herniation of at least 3 to 5 mm below the foramen magnum. Increased
detection of CMI has emphasized the need for more information regarding the
clinical features of the disorder.
METHODS: We examined a prospective cohort of 364 symptomatic patients. All
patients underwent magnetic resonance imaging of the head and spine, and some
were evaluated using CINE-magnetic resonance imaging and other
neurodiagnostic tests. For 50 patients and 50 age- and gender-matcher control
subjects, the volume of the posterior cranial fossa was calculated by the
Cavalieri method. The families of 21 patients participated in a study of
familial aggregation.
RESULTS: There were 275 female and 89 male patients. The age of onset was
24.9 ± 15.8 years (mean ± standard deviation), and 89 patients (24%) cited
trauma as the precipitating event. Common associated problems included
syringomyelia (65), scoliosis (42%), and basilar invagination (12%).
Forty-three patients (12%) reported positive family histories of CMI or
syringomyelia. Pedigrees for 21 families showed patterns consistent with
autosomal dominant or recessive inheritance. The clinical syndrome of CMI was
found to consist of the following: 1) headaches, 2) pseudotumor-like
episodes, 3) a Meniere's disease-like syndrome, 4) lower cranial nerve signs,
and 5) spinal cord disturbances in the absence of syringomyelia. The most
consistent magnetic resonance imaging findings were obliteration of the
retrocerebellar cerebrospinal fluid spaces (364 patients), tonsillar
herniation of at least 5 mm (332 patients), and varying degrees of cranial
base dysplasia. Volumetric calculations for the posterior cranial fossa
revealed a significant reduction of total volume (mean, 13.4 ml) and a 40%
reduction of cerebrospinal fluid volume (mean, 10.8 ml), with normal brain
volume.
CONCLUSION: These data support accumulating evidence that CMI is a disorder
of the para-axial mesoderm that is characterized by underdevelopment of the
posterior cranial fossa and overcrowding of the normally developed hindbrain.
Tonsillar herniation of less than 5 mm does not exclude the diagnosis.
Clinical manifestations of CMI seem to be related to cerebrospinal fluid
disturbances (which are responsible for headaches, pseudotumor-like episodes,
endolymphatic hydrops, syringomyelia, and hydrocephalus) and direct
compression of nervous tissue. The demonstration of familial aggregation
suggests a genetic component of transmission. (Neurosurgery 44:1005-1017,
1999)


DISTRIBUTION OF SUBSTANCE P IN THE SPINAL CORD OF PATIENTS WITH SYRINGOMYELIA


Thomas H. Milhorat, M.D., Harrison T. M. Mu, M.D., Carole C. LaMotte, Ph.D.,
and Ade T. Milhorat, M.D.
Department of Neurosurgery, State University of New York Health Science
Center at Brooklyn and the Kings County Hospital Center, Brooklyn, New York;
Departments of Surgery and Anesthesiology, Yale University School of
Medicine, New York, New York


The distribution of substance P, a putative neurotransmitter and pain-related
peptide, was studied using the peroxidase-antiperoxidase immunohistochemical
method in the spinal cords obtained from autopsy of 0 patients with
syringomyelia and 10 age- and sec-matched, neurologically normal individuals.
Substance P immunoreactivity was present in axons and in terminal-like
processes in close apposition to neurons in the first, second, and third
laminae of the dorsal horn. Smaller amounts of peroxidase-positive staining
were found in the fifth lamina of the dorsal horn, the intermediolateral
nucleus, the intermediomedial nucleus, and the ventral horn. In nine of 10
patients with syringomyelia, there was a substantial increase in substance P
immunoreactivity in the first, second, third, and fifth laminae below the
level of the lesion. A marked reduction or absence of staining was present in
segments of the spinal cord occupied by the syrinx. Central cavities produced
bilateral abnormalities, whereas eccentric cavities produced changes that
were ipsilateral to the lesion. No alterations in staining were found in the
spinal cord of an asymptomatic patient with a small central syrinx. The
authors conclude that syringomyelia can be associated with abnormalities in
spinal cord levels of substance P, which may affect the modulation and
perception of pain.
Craniocervical Decompression, Cerebral Blood Flow and Neuropsychological
Dysfunction in FMS and CFS
Michael J. Rosner, M.D., F.A.C.S., F.C.C.M., Sharon E. Guin, C.R.N.P.,Alice
Johnson, R.N and Sheila D. Rosner, B.S.N., M.S.N.
The authors gratefully acknowledge Drs. J. M. Mountz and E. San Pedro of the
Division of Nuclear Medicine at the University of Alabama at Birmingham for
SPECT scan data.
Introduction: Because others have reported that rCBF measured by SPECT scan
was abnormal in patients with fibromyalgia syndrome, the hypothesis was
tested that rCBF as measured by SPECT scan would improve after
craniovertebral surgery in similar patients.
Methods: Sixteen patients underwent pre-operative rCBF SPECT scan who had
been offered decompressive craniovertebral surgery for Chiari syndrome,
congenital cervical stenosis, or both. Detailed neurologic history and
physical examination were recorded in a prospective standardized interview
and examination. Pre-operative grip strength was measured and the spinal cord
was measured in its AP and transverse diameters. After surgery, the
interviews, standardized exams, grip strength, the spinal cord measurements,
and the rCBF SPECT scan were repeated. A parallel group of patients later
were given a more detailed questionnaire pre- and post-operatively which
included a number of questions related to neuropsychological complaints.
These were graded by the patients for severity using a 0-3 scale where 0 = no
problem and 3 was severe, or a visual analog scale of 0-100 where 100
represented the worst imaginable degree of severity. A group of well controls
completed the same instrument.
Results: Generalized bi-hemispheric increases in rCBF SPECT measures of blood
flow occurred in a statistically reliable fashion. Generalized blood flow
increases averaged 3 to 4 percent for global cortical measures. The left
frontal lobe increased by approximately 10 percent and the right occipital
and parietal regions by 4 and 12 percent respectively (p<.05--.001).
The neurologic complaints and exams of this group generally improved with
reduction in hyperreflexia, Babinski responses, improvement in strength, etc.
Grip strength improved from 23.2 ± 5.9 Kg. to 30.3 ± 11.4 Kg. (p = .03). In
those patients undergoing cervical decompression (n = 8), spinal cord area
increased by as much as 40