CLINICAL EVIDENCE FOR CERVICAL MYELOPATHY DUE TO CHIARI MALFORMATION AND SPINAL STENOSIS IN A NON-RANDOMIZED GROUP OF PATIENTS WITH THE DIAGNOSIS OF FIBROMYALGIA.
Dan S. Heffez, Ruth E. Ross, Yvonne Shade-Zeldow, Konstantinos Kostas, Sagar Shah, Robert Gottschalk, Dean A. Elias, Alan Shepard, Sue E. Leurgans and Charity G. Moore

OBJECTIVE: While patients with fibromyalgia report symptoms consistent with cervical myelopathy, a detailed neurological evaluation is not routine. We sought to determine if patients with fibromyalgia manifest objective neurological signs of cervical myelopathy. METHODS: Two hundred and seventy patients, 18 years and older, who carried the diagnosis of fibromyalgia but who had no previously recognized neurological disease underwent detailed clinical neurological and neuroradiological evaluation for the prevalence of objective evidence of cervical myelopathy and radiological evidence of cerebellar tonsillar herniation (Chiari 1 malformation) or cervical spinal canal stenosis. RESULTS: Patients were primarily women (87%), of mean age 44 years, who had been symptomatic for 8 years (standard deviation, 6.3 years). The predominant complaints were neck/back pain (95%), fatigue (95%), exertional fatigue (96%), cognitive impairment (92%), instability of gait (85%), grip weakness (83%), paresthesiae (80%), dizziness (71%) and numbness (69%). Eighty-eight percent of patients reported worsening symptoms with neck extension. The neurological examination was consistent with cervical myelopathy: Upper thoracic spinothalamic sensory level (83%), hyperreflexia (64%), inversion of the radial periosteal reflex (57%), positive Romberg sign (28%), ankle clonus (25%), positive Hoffman sign (26%), impaired tandem walk (23%), dysmetria (15%) and dysdiadochokinesia (13%). MRI and contrast-enhanced CT imaging of the cervical spine revealed stenosis. The mean anteroposterior (AP) spinal canal diameter at C2/3, C3/4, C4/5, C5/6, C6/7 and C7/T1 was 13.5 mm, 11.8 mm, 11.5 mm, 10.4 mm, 11.3 mm and 14.5 mm respectively, (CT images). In 46% of patients, the AP spinal diameter at C5/6 measured 10 mm, or less, with the neck positioned in mild extension, i.e., clinically significant spinal canal stenosis. MRI of the brain revealed tonsillar ectopia >5mm in 20% of patients (mean=7.1±1.8 mm), i.e., Chiari 1 malformation. CONCLUSION: Our findings indicate that some patients who carry the diagnosis of fibromyalgia have both signs and symptoms consistent with cervical myelopathy, most likely resulting from spinal cord compression. We recommend detailed neurological evaluation of patients with fibromyalgia in order to exclude cervical myelopathy, a potentially treatable condition.
European Spine Journal, Vol. 13, Oct 2004, pp 516-523



FUNCTIONAL ABNORMALITIES OF THE CERVICAL CORD AND LOWER MEDULLA AND THEIR EFFECT ON PAIN: OBSERVATIONS IN CHRONIC PAIN PATIENTS WITH INCIDENTAL MILD CHIARI I MALFORMATION AND MODERATE TO SEVERE CERVICAL CORD COMPRESSION.
Mark Thimineur, MD, Madeline Kitaj, MD, Edward Kravitz, PhD,Tamara Kalizewski, PAC and Pardeep Sood, MD.

OBJECTIVE: Abnormalities of central sensory processing may play a role in the pathogenesis of chronic pain. The Chiari I malformation is a congenital hindbrain anomaly characterized by protrusion of the cerebellar tonsils into the upper cervical canal, with variable effects on the lower brain stem and cervical cord. The purpose of this study was to compare sensory function and pain among patients with chronic pain who had these disorders incidentally diagnosed, to assess the effect on pain in these patients in comparison with those without central nervous system disease. DESIGN: Retrospective study in which pain, mood, and sensory function in 32 patients with chronic pain who had mild Chiari I malformation were compared with that in 53 patients with chronic pain who had moderate to severe compression of the cervical spinal cord and 52 patients with chronic pain who had no apparent central nervous system disorder. Data had been collected previously as part of standard clinical assessments, including clinical neurological examinations, quantitative sensory testing, pain drawings and psychometric testing with the Symptom Checklist 90. PATIENTS: All subjects were patients of a hospital-based pain management practice who had been accepted for treatment over a 5-year period. RESULTS: Both the Chiari I and cervical compression groups had long tract signs evident on clinical neurological examination. Quantitative sensory testing indicated elevations in the trigeminal territory among patients with Chiari I malformation and on the neck, hands, and feet in both the Chiari I and cervical compression groups. The extent of pain and mood disturbance was greatest in the Chiari I group and least in the group with no central nervous system disorder. Complex regional pain syndrome, fibromyalgia, and temporal mandibular joint disorder were more common among the Chiari I malformation group than among the other groups. CONCLUSIONS: Quantitative sensory analysis indicates sensory dysfunction associated with Chiari I malformation and cervical cord compression. The pattern of sensory abnormality is consistent with medullary dysfunction among the patients with Chiari I malformation and cervical cord dysfunction among cord compression patients. There were differences in the types and extent of pain and the associated disorders of mood observed among the cohorts defined above. These differences may be partly due to the presence and location of central sensory dysfunction.
The Clinical Journal of Pain, 2002, 18:171-179

NEUROLOGICAL EXAMINATION IN INDIVIDUALS WITH FIBROMYALGIA (FM), AND RELATIONSHIP TO MRI FINDINGS.
Daniel J Clauw, Frank Petzke, Eduardo Paiva, Michael J. Rosner, Robert M Bennett

INTRODUCTION: Individuals with FM have classically been described as having a completely normal physical examination except for the finding of tenderness. However, most studies of FM have been performed by rheumatologists, who may not always perform a detailed neurological evaluation as part of the physical exam. The purpose of this study was to do such an examination in both FM pts. and controls, to determine whether FM pts. exhibited abnormalities in physical exam, as well as to determine whether abnormalities were related to MRI findings in the cervical region. METHODS: Before beginning the study, three physicians from two different study sites were trained in the performance of a thorough neurological evaluation by a neurosurgeon. The examination took approximately 40 minutes to complete, and included comprehensive sensory, motor, cranial nerve, spinal and reflex evaluations. The results of these examinations were then summarized by a single observer, blinded to subject group, as being normal, mildly abnormal or markedly abnormal. RESULTS: 39 FM subjects and 20 controls had this evaluation performed. The FM subjects demonstrated significantly more abnormalities on many portions of the neurological exam, especially in motor (53% abnl. vs. 0 of controls), reflexes (31% abnl. vs. 10% of controls), gait (33% abnl. vs. 15% of controls) and sensory (23% abnl. vs. 5% controls) components. There was no relationship between MRI findings and physical examination findings in either group. CONCLUSIONS: FM patients often have abnormal neurological examinations. Such findings could have several explanations including: 1) structural or compressive lesions, 2) the effects of pain on motor performance and balance, 3) hyperreflexia secondary to hypervigilance, or 4) hyperalgesia/allodynia. Further studies will be necessary to determine the precise mechanism(s), and the appropriate diagnostic paradigms for individuals with symptoms of FM and an abnormal neurological examination. DISCLOSURE: This study was supported by the National Fibromyalgia Research Association.
Abstract presented at:
American College of Rheumatology
Annual Meeting in Philadelphia
October-November 2000

IS CERVICAL MYELOPATHY OVERLOOKED IN PATIENTS WITH FIBROMYALGIA?
Dan Heffez, MD, et al

Fibromyalgia is a syndrome characterized by diffuse chronic pain. The American College of Rheumatology has established diagnostic criteria, which have been helpful in distinguishing fibromyalgia from other chronic pain states but have not advanced the understanding of its etiology. The worldwide prevalence of fibromyalgia is estimated to be 2%. An estimated six million Americans are affected. In addition to the widespread pain, patients complain of a variety of symptoms, including overwhelming fatigue exacerbated by exertion, headache, dizziness, cognitive difficulties, instability of gait, limb numbness and paresthesiae. Some physicians have come to view the syndrome as a somatization disorder because of these numerous and apparently unrelated complaints and because fibromyalgia fails to fit the biomedical cause-effect model. Many of the symptoms reported by fibromyalgia patients are identical to those reported by patients diagnosed with either Chiari 1 malformation or with cervical myelopathy due to spinal stenosis, (spondylotic cervical myelopathy), two well-defined neurological disorders. Therefore, we evaluated a cohort of patients who carried the diagnosis of fibromyalgia for objective evidence of cervical myelopathy. Two hundred and seventy (270) consecutive patients who carried the diagnosis of fibromyalgia were evaluated between September 1998 and May 2001. The sole requirement for referral was that the patient carry the diagnosis of fibromyalgia. On initial evaluation, patients completed a questionnaire detailing their symptoms, current medications and past medical consultations. A diagram depicting the distribution of the patient’s body pain and an analogue pain severity scale were completed. In order to insure that every patient was assessed in the same manner, they were evaluated by a neurologist and/or a neurosurgeon who, independent of each other, performed a neurological examination and recorded the findings on a standardized form. All data including the results of the examinations were entered into a relational database. Every patient underwent magnetic resonance imaging of the brain with special attention to the foramen magnum in order to exclude a Chiari 1 malformation. For the purpose of determining the position of the cerebellar tonsils the lower lip of the foramen magnum was defined as extending from the lowest cortical bone of the clivus anteriorly (basion) to the lowest cortical bone at the position posteriorly on themed sagittal MRI image. The position of the most caudal point of the tonsil(s) relative to the inferior lip of the foramen magnum was measured from the midsagittal MRI slice. MRI scan of the cervical spine was performed in order to identify any intrinsic spinal cord lesion capable of causing myelopathy. Every patient also underwent computed tomographic (CT) imaging of the cervical spine following the intravenous infusion of 150 ml of non-ionic contrast (300 mg of iodine/ml). The CT scan was performed with the patient’s neck in the neutral and then in the extended position. The gantry anangle was altered to obtain images perpendicular to the spine at each level. The mid-sagittal anterior-posterior (AP) dimension of the spinal canal was determined at the level of the intervertebral disc space on both neutral CT and MRI images and on CT images with the neck extended. MRI and CT images were individually scanned into a Pentium III personal computer using a Umax Power Look III scanner. One of two independent observers, unrelated to the medical evaluation or treatment of the patients, made measurements of the position of the cerebellar tonsils and the mid-sagittal AP spinal canal diameters using SigmaScan Pro software, version 5.0. Eighty-six percent of the patients were women. Ninety-seven percent were Caucasian. The mean age was 44 years (SD=11 years). The mean duration of symptoms was 8 years (std dev 67.3 yrs). Fifty-nine percent of patients reported antecedent craniospinal trauma within 3-6 months of the onset of symptoms. On average, the patients had consulted 10 different medical specialists during the course of their illness. Patients were taking a mean of 4.8 medications, (including but not limited to opiate and non-opiate analgesics, benzodiazepines, antidepressants, sedative hypnotics and muscle relaxants), for the relief of symptoms related to fibromyalgia. Forty-one percent of patients had at least a college education. Sixty-eight percent of patients had left their job as a direct result of their illness. The predominant complaints were neck/back pain (95%), fatigue (95%), exertional fatigue (96%), cognitive impairment (92%), instability of gait (85%), subjective grip weakness (83%), paresthesiae (80%), dizziness (71%) and numbness of the hands/feet (69%). Eighty-eight percent of patients reported worsening symptoms with neck extension. The findings of neurological examination were diagnostic of cervical myelopathy. An upper thoracic spinothalamic sensory level (T3-T6) was noted in 83% of patients: Typically, we detected hyperalgesia and allodynia to a cold or lightly applied pinprick stimulus below a dermatome level. Rarely, a suspended band of hypesthesia to cold or pinprick stimulus was detected between the third and seventh thoracic dermatomes. The second most common neurological finding, (noted in 64%of patients), was hyper-reflexia. Recruitment, (the pathological spread of reflexes beyond the muscle being tested), including inversion of the radial periosteal reflex was observed in 357% of patients. Other objective neurological findings included positive Romberg sign (29%), varying degrees of ankle clonus (25%), positive Hoffman signs (26%), impaired tandem walk (23%), dysmetria 915%) and disdiadochokinesia (13%). The patients were examined first with the neck in the neutral and subsequently in the flexed and then in the extended positions. Neck extension and neck flexion resulted in immediate accentuation of the abnormal pyramidal track findings in 88% and 73% of patients respectively, suggesting a mechanical etiology for the abnormal neurological findings. The MRI images of the brain did not show any consistent intrinsic disease of the brain, parenchyma. The only consistent finding was the caudal displacement of the cerebellar tonsils. The mean position of the cerebellar tonsils as measured on the mid sagittal MFI image was 1.1 mm (SD=4.4mm) below the rim of the foramen magnum. In 38% of patients the tonsillar herniation exceeded 3mm, (mean 5.6, SD=2.1 mm). In 20% of patients tonsillar ectopia exceeded 5 mm (mean=7.1, SD=1.8mm). MRI and contrast enhanced CT imaging of the cervical spine revealed a narrow canal. I.e. stenosis. The mean AP spinal canal diameter at C2/3, C3/4, C4/5, C5/6, C6/7 and C7T1 was 13.2, 11.7, 11.8, 10.7, 11.5 and 14.9 mm respectively, (CT images). In 23% of patients, the AP mid-sagittal spinal canal diameter was 10mm or less at the C5/6 intervertebral disc space measured 10mm or less in 46% of patients when the neck was positioned in extension, (CT images). MR imaging of the cervical spine did not reveal any consistent intrinsic spinal cord disease with the exception of signal hyper-intensity at the level of spondylotic spinal cord compression noted on the T2 sequence images in some patients. Symptoms of myelopathy are variable and can be quite vague, often leading to initial misdiagnosis. We have identified neurological findings diagnostic of cervical myelopathy in a selected cohort of 270 patients previously diagnosed with fibromyalgia. An upper thoracic sensory level has been described as a false localizing sign of cervical myelopathy. Recruitment of deep tendon reflexes is an upper motor neuron sign of pyramidal tract dysfunction. Inversion of the radial periosteal reflex is felt to be virtually diagnostic of myelopathy due to extrinsic compression of the spinal cord at the level of the fifth or sixth cervical vertebra. Neuroradiological findings were consistent with a treatable structural cause(s) for the myelopathy –i.e. spondylotic cervical stenosis and/or Chiari type 1 malformation. In 20% of our patients a radiological diagnosis of the Chiari 1 malformation could be made based on tonsillar ectopia in excess of 5mm. Meadows et al (J Neurosurgery 2000; 96:920-926), reviewed the brain and cervical spine MRI’s of 22,591 patients and could identify only 175 patients in whom tonsillar ectopia exceeded 5 mm, i.e. a prevalence of .77%. The CT scan of the cervical spine revealed cervical stenosis, which was accentuated by neck extension. Neck extension is known to reduce the AP spinal canal diameter. The stenosis in myelopathic patients has been described by Muhle et al, (Acta Radiologica 1999; 40:146-153), and Graham et al, (Clinical Radiology 2001; 50:35-39). The AP mid-saggital diameter at the C5/6 intervertebral disc space measured 10mm or less in 46% of patients with the neck placed in extension. A mid-sagittal diameter of 10mm is acknowledged as stenotic and consistent with symptomatic spinal cord compression, i.e. cervical myelopathy. Spondylotic cervical myelopathy and the Chiari 1 malformation are treatable conditions. Therefore, we recommend that a detailed neurologic examination should be incorporated into the evaluation of all patients with fibromyalgia. Evidence of cervical myelopathy would warrant neuroradiological examination of the brain and cervical spine and appropriate neurological referral.
Presented at:

  1. MYOPAIN
  2. 2001, Portland, OR – September 9-13, 2001
  3. Congress
  4. of Neurological Surgeons, San Diego, CA Oct 4, 2001 - Poster
  5. National
  6. Fibromyalgia Research Assn, Neurology & New Treatment Modalities in FM Symposium, Portland, OR Oct 2002

IS THERE AN ASSOCIATION BETWEEN CERVICAL MYELOPATHY AND FIBROMYALGIA?
Dan Heffez, MD, et al

INTRODUCTION: Cervical myelopathy and fibromyalgia have a number of symptoms in common. It has been suggested that fibromyalgia may be incorrectly diagnosed in some patients who actually have cervical myelopathy or perhaps that cervical spinal cord dysfunction is the underlying cause of the fibromyalgia syndrome. In order to examine the possible relationship between cervical myelopathy and fibromyalgia, we undertook a prospective nonrandomized, case control outcome study of operative versus non-operative treatment of cervical myelopathy in patients who had previously been diagnosed with fibromyalgia. METHODS: Patients carrying the diagnosis of fibromyalgia were referred for neurological evaluation in order to exclude the possibility of myelopathy. Patients underwent a highly structured evaluation, which included a neurological examination by a neurologist and a neurosurgeon, a psychological interview and detailed neuroradiological imaging of the brain and cervical spine. The radiological evaluation included MRI of the cervical spine, MRI of the brain with the axial cuts through the plane of the foramen magnum and dynamic contrast enhanced CT of the cervical spine. Patients also completed an HADS and a SF-36 outcome questionnaire. All data was gathered prospectively and entered into a relational database. Patients were followed up every 3 months using a uniform mail-in questionnaire regardless of treatment prescribed. Cervical myelopathy was diagnosed in the face of symptoms consistent with myelopathy and in the presence of neurological signs indicative of cervical spinal cord dysfunction. Both symptoms and signs of myelopathy were required for inclusion in the outcome study. Cervical stenosis was diagnosed if the anteroposterior (AP) mid-sagittal spinal canal diameter measured 10 mm or less at 1 or more levels as determined from MRI or dynamic CT imaging of the cervical spine. Chiari 1 malformation was diagnosed if tonsillar herniation equaled or exceeded 5 mm as measured on the mid-sagittal MRI image. Surgical candidates met the following criteria: 1) the neruorlogical examination was abnormal and localized to the cervical spine or cervicomedullary junction, 2) the neuroradiological findings were consistent with compression of the cervical spinal cord or caudal brain stem and 3) non-operative measures, if appropriate, had failed over 3-6 months. Non-operative treatment consisted of analgesics, use of a cervical collar and posture and body mechanics training. Non-operative therapy was offered as primary treatment if the patient was unwilling to undergo surgery or if the radiological findings suggested that external cervical immobilization might be effective in minimizing spinal cord compression. RESULTS: There were 64 patients in the surgical group and 44 patients in the non-surgical group. While the patients were not randomized to the treatment arms, the 2 groups were virtually identical with regards to sex ratio, mean age, mean duration of illness, history of craniospinal trauma, level of education and work history The prevalence of those symptoms commonly associated with both cervical myelopathy and fibromyalgia, including pain, headache, numbness, tingling, instability of gait, dizziness and grip weakness was identical in the 2 groups of patients. The prevalence of those symptoms commonly associated with fibromyalgia but not with cervical myelopathy, such as fatigue, cognitive difficulties, irritable bowel syndrome, insomnia and depression did not differ between the 2 groups. The findings on neurological examination did not differ between the 2 groups. The most prevalent findings were high thoracic spinothalamic sensory level to a cold or pinprick stimulus, hyper-reflexia, recruitment of reflexes, Hoffman sign, ankle clonus and absent gag reflex. In both surgical and non-surgical patients, the pyramidal tract findings became more pathological when the patient was examined with the neck positioned in flexion or extension. There was no difference between the 2 groups in their initial responses to the SF36 quality of life questionnaire, nor in their level of anxiety or depression (HADS questionnaire). The mid-sagittal AP spinal canal diameter in both the surgical and non-surgical patients was distinctly smaller than that reported in the literature for normal men and women using similar imaging techniques. In both the surgical and non-surgical groups, 23% of patients had a mid-sagittal spinal canal diameter 10 mm or less at the C5/6 disc space as measured on CT or MRI images. With the neck positioned in extension, 46% of surgical and non-surgical patients were found to have mid-sagittal AP spinal canal diameter 10 mm or less at the C5/6 disc space as measured on CT images. Forty percent of patients in the surgical group had 3 mm or more of tonsillar ectopia (mean 5.6 mm) while 27% of the patients in the non-surgical group had a similar finding (mean 4.0 mm) as measured in the traditional manner on the mid-sagittal T1 weighted MRI image. No single structural cause for myelopathy was identified and therefore no single procedure was performed. The surgical treatment of myelopathy included suboccipital decompression, anterior cervical discectomy and fusion or cervical laminectomy with or without instrumented fusion as indicated by the neuroradiological findings. While we diagnosed and treated myelopathy, we monitored all symptoms. At the six month follow-up, there was a statistically significant improvement in the surgical group as compared to the non-surgical group regarding patient reported dizziness, limb numbness, pain, impaired balance and grip weakness (p=0.04 – p=0.000, Chi squared analysis). Improvement was noted in a number of symptoms associated with fibromyalgia and not usually associated with cervical myelopathy such as irritable bowel syndrome (p=0.003) and impaired memory (p=0.0007), impaired concentration (p=0.03) and disorientation (p=0.002). Headache improved in 90% of the surgical group and 45% of the non-surgical group (p=0.06). Patients in the surgical group were more likely to report an improvement in fatigue, depression, insomnia, limb paresthesiae, clumsiness and cold intolerance than were patients in the non-surgical group but the differences were not statistically significant. There was an improvement in all 9 subscales of the SF36 in the surgical as compared with the non-surgical group, (p=0.037 – p<0.0001, Wilcoxon rank sum test and Fisher’s exact test). Surgical treatment of cervical myelopathy associated with spondylotic cervical stenosis and/or the Chiari 1 malformation may result in the improvement of a vast array of symptoms usually attributed to fibromyalgia, with an associated improvement in patient quality of life. Despite non-randomization, the surgical and non-surgical patients were virtually identical in all measured parameters at the time of initial evaluation. However, as the patients were not randomized, the observed difference in outcome cannot be definitively or exclusively attributed to surgery. Nevertheless, our outcomes implicate a potential association between cervical myelopathy and fibromyalgia in some patients.
Presented at:

  1. MYOPAIN
  2. 2001, Portland, OR – September 9-13, 2001
  3. Congress
  4. of Neurological Surgeons, San Diego, CA Oct 4, 2001 - Poster
  5. National
  6. Fibromyalgia Research Assn, Neurology & New Treatment Modalities in FM Symposium, Portland, OR Oct 2002

THE NEUROSURGICAL SUBSET OF FIBROMYALGIA:
PART I: CLINICAL FINDINGS WITH NEURALLY MEDIATED HYPOTENSION.
Michael J. Rosner, Royce K. Bailey, Jorge Flechas

Fibromyalgia is a syndrome characterized by low pain thresholds, but which may occasionally be a manifestation of surgically treatable disease. The hallmark of this subset of patients will be objective neurological abnormality. OBJECTIVE: To define neurological abnormalities in patients who may be candidates for surgical decompression, to document symptomatic outcome at one-year after surgery (part II) and to document radiological differences between control and this subset of FMS patients (part III). METHODS: 37 patients underwent protocol neurological and tilt table examinations that included a detailed neurological history/review of system and assessment of cranial nerve, motor, sensory, extrapyramidal and reflex evaluations. Each underwent a tilt table examination to objectively assess at least the cardiovascular component of the autonomic nervous system to restrict this subset to those with objectively defined organic disease. RESULTS: Patients had been symptomatic for > 10 years and on disability for an average of 4.9 years. Cranial nerve deficits: 14/37 demonstrated unilateral or bilateral Horner’s syndrome. 21/37 had CN V abnormalities including absent corneal reflexes. 7/17 demonstrated CN VII abnormalities (not including dry eyes, dry mouth, or hyperacusis) and 17/37 had abnormalities of hearing (not including tinnitus). 20/37 had CN IX, X, XI abnormalities, most commonly absent gag reflex and oropharyngeal sensory changes. Abnormalities of convergence, nystagmus, head tilt, skew deviation, esophoria and tongue weakness were common. Upper extremity motor deficits were identified in 36/37 and 35/37 in the lower extremities. Hyper-, hypotonia and atrophy were frequent. 32/37 had abnormalities to pin, touch, vibration or temperature sensation. 24/37 demonstrated hyperreflexia, and 17/37 demonstrated hyporeflexia. 34/37 had abnormalities of gait, 27/37 a positive Romberg’s sign and Babinski’s and Hoffman’s sign were present in 18 and 8 of the 37 respectively. CONCLUSION: Neurological deficits can be identified in those patients with fibromyalgia syndrome and a positive tilt table examination. These deficits localize to the brainstem and upper cervical spinal cord and when identified should lead to a detailed radiological evaluation of these regions.

INTRODUCTION: Fibromyalgia is a syndrome characterized by complaints of pain 2, and increasingly recognized as accompanied by neurological complaints of numbness, weakness, ataxia, dysphagia, tinnitus, visual changes and multiple dysautonomias. Particularly prominent amongst the latter are cardiovascular symptoms such as orthostasis, dizziness, palpitations, non-cardiac chest pain, Raynaud’s phenomena, and others. In order to establish a patient population with an objective characteristic which could not be mistaken for hysteria, malingering, depression or other psychosomatic manifestation, the study was limited to those with a positive tilt table test for neurally mediated hypotension (NMH), positional orthostatic tachycardia syndrome (POTS) or both. OBJECTIVE: To examine the occurrence of neurological deficit amongst individuals who had been previously diagnosed with FMS in the subset of tilt table proven dysautonomias. METHODS: Fifty-two patients with the diagnosis of fibromyalgia and referred for the possibility of surgical intervention underwent protocol neurological examinations (part 1), a protocol MR scan evaluation of the craniocervical region (part 2), questionnaires listing symptoms which the patient graded in severity (part 3), and dolorimetery (part 4). Cranial nerves and brainstem functions: Each nerve was assessed for normal versus impaired function. Additional observations included the presence or absence of Horner’s syndrome, convergence deficits and nystagmus. Hyperacusis, dry eyes, and dry mouth were not recorded as VII nerve abnormalities. Motor function was graded using the MRC scale of 0-5 for each major group of the upper and lower extremities. Data were summarized as showing normal or abnormal strength for the upper and lower extremities separately. Grip strength was measured as the average of each of three trials on a hand dynamometer. Sensory function included pinprick, light touch in each dermatome; temperature sensation (cool) and vibration for multiple points in each extremity. Data were recorded as normal or abnormal. Gait was assessed in terms of a standard walk, heel walking, tip-toe walking, and tandem gait. Data were summarized as normal or abnormal. Romberg’s test was recorded as “0” for normal, 1+ for a slight sway, 2+ for consistent sway, 3+ for severe unsteadiness which did not lead to a fall, and 4+ for those who fell or had to be caught. Reflexes were graded 0-4 with “2” being normal. Hyporeflexia and hyper-reflexia were summarized separately. Analysis was through Systat 7.0 and/or Microsoft Excel 2002.

RESULTS:
Cranial Nerve Abnormalities:

  1. Nystagmus
  2. 64%
    Usually fine or rotatory, more prominent in extension
  3. Convergence
  4. abnormal 43%
    Often asymmetric left eye usually worse
  5. Horner’s
  6. syndrome 31%
  7. Absent
  8. corneal reflex 37%
    Uni- or bilateral
  9. Other
  10. V abnormality 69%
    Often intraoral
  11. VII
  12. abnormality 20%
    Most apparent with testing of orbicularis oculi
  13. VIII
  14. abnormality 46%
  15. IX-X
  16. abnormality 60%
    Usually included uni- or bilaterally absent gag reflex
Motor Function:
  1. Upper extremities 86%
    Usually multiple groups
  2. Lower
  3. Extremities 94%
    Most often iliopsoas, usually bilateral
  4. Grip
  5. Strength
    • Right
    • 28.2 +/- 9.7 Kg (Median = 27.2)
    • Left
    • 27.1 +/- 9.5 Kg (Median = 24.5)
Sensory function: 88%
  • Multiple
  • modalities were usually abnormal

Deep tendon reflexes:

  • Normal
  • 15%
  • Hyper-reflexia
  • 52%
  • Hypo-reflexia
  • 19%
  • Both
  • hyper– and hypo- 14%
Pathological reflexes:
  • Babinski’s
  • response
    • Normal
    • 40%
    • Equivocal
      1. Unilateral
      2. 14%
      3. Bilateral
      4. 6%
    • Present

      1. Unilateral
      2. 15%
      3. Bilateral
      4. 25%
  • Hoffman
  • reflex
    • Absent
    • (normal) 76%
    • Uncertain
    • 6%
    • Unilateral
    • 16%
    • Bilateral
    • 2%
Gait:
  • 92%
  • Usually spasticity, ataxia, wide based
Romberg:
  • Normal
  • or “Trace” 35%
  • 2-3+
  • 55%
  • 4+
  • 10%
DISCUSSION:
In patients with the dysautonomias, NMH/POTS and who have been classified as fibromyalgia syndrome, neurological abnormalities are common and localize to the brainstem and upper cervical spinal cord. These abnormalities are frequently subtle, and require significant time and experience to define. However, they point directly to a coherent anatomical focus, which can explain many of the symptoms of “fibromyalgia” and encourage the search for correctible lesions. The brainstem not only is adequate explanation for symptoms such as dysphagia, dysphonia, difficulty with balance and other, but it also harbors the centers for cardiovascular control, gastrointestinal control, respirations and the reticular activating system (see part 3 & 4). The latter is particularly important with regard to sleep, arousal, attention and the subsequent incorporation of memory. The lower medulla also is the source of origin for descending nociceptive control fibers, which originate in the nucleus raphe magnus and paragigantocellularis 1. Fibromyalgia is not an explanation for neurological deficit, no matter how subtle it may be. Neurological abnormalities should invoke a search for treatable lesions: That search should focus on the upper spinal cord and brainstem based upon the observations presented here.

CONCLUSIONS:
In patients with the previous diagnosis of fibromyalgia who also had positive tilt table examinations for NMH/POTS, physical examination abnormalities were common. These abnormalities center about the lower brainstem and upper spinal cord. Fibromyalgia (and related syndromes) is not an adequate explanation for neurological abnormality.

Reference List

  1. Jessell
  2. TM, Kelly DD: Pain and analgesia, in Kandel ER, Schwartz JH, Jessell TM (eds): Principles of Neural Science, ed 3. Norwalk, Connecticut: Appleton & Lange, 1991, pp 385-3992.
  3. Wolfe
  4. F, Smythe HA, Yunus MB, et al: The American College of Rheumatology 1990 criteria for the classification of fibromyalgia. Arthritis Rheum. 33:160-172, 1990

    THE NEUROSURGICAL SUBSET OF FIBROMYALGIA: PART II: RADIOLOGICAL OBSERVATIONS—PATIENTS VS. CONTROLS.
    Michael J. Rosner, Jorge Flechas, Royce K. Bailey

    INTRODUCTION: Clinical observations suggest the upper spinal cord and brainstem may be involved in a subset of patients with FM like symptoms. OBJECTIVE: Test the hypothesis that there will be radiological differences related to the posterior fossa and/or cervical canal between control and the surgical subset of FMS patients. METHODS: 37 patients carrying the diagnosis of ‘fibromyalgia’ were selected based upon an abnormal tilt table examination defining cardiovascular/autonomic dysfunction (NMH/POTS) and abnor malities identified from the neurological examination. Protocol magnetic resonance (MR) scans of the craniocervical junction region were accomplished for each patient in sagittal, coronal and transaxial planes; cerebrospinal fluid (CSF) flow via phase contrast MR was assessed. Specific grading of tonsillar descent (midline and paramedian), obex height, tonsillar impaction within the foramen magnum, vertebral artery invagination of the brainstem, and AP diameter of the foramen magnum was carried out. The spinal cord and canal diameters were measured at each disk level with electronic calipers. These results were compared with those of asymptomatic control patients. Systat 7.0 and Excel were used for data analysis; all values X + SD.
    RESULTS:
      FM Subset    Controls               

    P Value

    Tonsil-Right  (mm)      2.7 + 3.1     0.46+2.4      0.006
    Tonsil-Left (mm)          2.6 + 2.8    0.50+2.4     

    0.008

    Tonsil-Midline (mm)   

     2.1 + 3.2      -1.7+3.7     <0.001
    Impaction in fore mag      2.2 + 1.0      1.1+0.96     0.005
    VA impaction brainstem  1 .2 + 0.6   0.24+0.56   <0.001 
    C3 Canal  (mm)        11.2 + 2.1    12.8+1.9        0.04
    C4 Canal  (mm)        11.0 + 2.7    12.9+1.8      0.03
    C5 Canal  (mm)        10.7 + 2.7    12.2+1.8        0.07
    CONCLUSION: FM-NMH/POTS patients with abnormal neurological examinations represent a population that is radiographically distinct from asymptomatic individuals. These differences relate to the brainstem and cervical spinal cord as suggested by neurological examinations. However, the overlap of standard deviations is such that simple review of MR scans will not discriminate between these groups independently of the neurological exam. INTRODUCTION: We have observed subtle radiographic changes on craniocervical MR scans of patients with neurological findings related to the brainstem and upper cervical spinal cord sufficient to explain clinical symptoms and physical findings. However, in most cases, these MR scans were reasonably read as “normal” by radiologists, leading the referring physician to seek other than structural etiologies for the patient’s complaints. This is an important error not to make, and we wish to establish a set of observations which might provide a core of information to help make an accurate radiological diagnosis in this group of patients. HYPOTHESES: The cerebellar tonsils will be lower in FMS patients when compared with controls. Similar hypotheses will be tested for differences between FMS patients and controls with regard to: Tonsillar impaction into the foramen magnum, vertebral artery impaction into the brainstem, and the AP diameter of the cervical canal at each level. Hypothesis statement and testing was of the form:

    H1: μ1 ≠ μ0
    H0: μ1 = μ0

    METHODS: Protocol MR craniocervical scans were obtained on 37 patients who carried the diagnosis of fibromyalgia syndrome (FMS) and who had also had a tilt table test positive for neurally mediated hypotension (NMH) and/or positional orthostatic tachycardia syndrome (POTS). Assessments were recorded on a data sheet which included:

    Distance above or below the lower limit of the foramen magnum (in mm) for the:

     

    ____       Midline Tonsil                                                      
    ____       Right paramedian tonsil                   

    ____       Left paramedian tonsil                                      
    ____       Obex

    Relation of the upper cervical cord and/or medulla to the vertebral arteries:

    Right

    ____       0 = “CSF” signal completely separates cord from vertebral arter
    ____       1 = Vertebral artery abuts cord without distortion
    ____       2 = Vertebral artery invaginates (< 33%) or with mass effect

    Left

    ____       3 = Vertebral invaginates > 33% but < 67%
    ____       4 = Vertebral invaginates > 68% of diameter
    ____       9 = Cannot determine

    Relationship of cerebellar tonsils within foramen magnum (transaxial views):

     

    0 = No tonsil in foramen magnum
    1 = Portion of one or both tonsils in foramen magnum
    2 = Tonsils mold to cord/medulla < 180°
    3 = Tonsils mold > 180° of cord/medulla
    4 = Only trace CSF signal within foramen magnum
    5 = Cannot determine

    Measurements (in mm) of the cervical cord at C2 through C7: These measurements were made perpendicularly to the cord and centered at each disc space to facilitate reproduction of these same measurements on subsequent scans. These results were compared to similar measurements obtained from 40 asymptomatic control subjects previously recruited for a separate study and supported by the National Fibromyalgia Research Association.

    Other observations included:


    1. Corona
    2. l views done in thin section (about 3 mm) through the foramen magnum and perpendicular to theplane of the foramen magnum to better define the relationship of the cerebellar tonsil to the lateral medulla.
    3. Lateral
    4. shift and/or distortion of the brainstem within the foramen magnum and posterior fossa.
    5. Absence
    6. or near absence of CSF spaces posterior to the cerebellum, compression of inferior cerebellar folia.
    7. Abnormalities
    8. of CSF flow though the posterior fossa and foramen magnum.

    RESULTS:

      FM Subset Controls P Value* 

    Reject H0

    Tonsil Relation to Foramen Magnum
    Tonsil-Right (mm)      3.6 ± 3.9    0.86 ± 2.9   0.010 YES
    Tonsil-Left (mm)  3.3 ± 3.7   0.50 ± 2.4    0.008 YES
    Tonsil-Midline (mm)   2.9 ± 3.9     -1.0 ± 4.2 0.005

    YES

    Impaction in FM  

    2.5 ± 1.0    

    1.2 ± 1.1    0.002 YES
    VA impaction brainstem
    Right Vertebral 1.1 ± 0.8  0.6 ± 0.9     0.170 NO
    Left Vertebral 1.2 ± 0.7 0.3 ± 0.6     0.0002 YES
    Canal Measurements
    C1 Canal  (mm)      15.1 ± 1.7 16.5 ± 1.5 0.045 YES
    C2 Canal  (mm)      11.7 ± 1.7 3.0 ± 1.5  0.001 YES
    C3 Canal  (mm)      11.1 ± 1.7    13.0 ± 1.5    0.001     YES
    C4 Canal  (mm)     10.8 ± 2.2    13.1 ± 1.8    0.002       YES
    C5 Canal  (mm)      10.6 ± 2.4    11.9 ± 1.6    0.068 ?
    C6 Canal  (mm)       11.5 ± 2.0    11.8 ± 1.2    0.597 NO
    C7 Canal  (mm)     13.5 ± 1.7    13.6 ± 1.0    0.768       NO

    *  All ‘p’ values ‘two-tailed’, separate variance

    Adequate control data did not exist to compare other aspects of the scans between the two groups. However, the scan of the FMS-NMH/POTS patient typically will show:

    1. Poor
    2. to absent CSF circulation posterior to the cerebellum
    3. Poor
    4. CSF flow posterior to the cervical cord
    5. A
    6. ‘meniscus’ sign below the cerebellar tonsils
    7. Distortion and
    8. subtle shift of the brainstem on transaxial views of the foramen magnum
    9. Compression
    10. of the cerebellar folia in the inferior cerebellum
    11. Absence
    12. of the subarachnoid space posterior to the cerebellar hemispheres
    13. Others

    DISCUSSION: The cerebellar tonsils in FMS-NMH/POTS patients descend further through the foramen magnum than do those of control subjects. The population difference is about 3 mm lower for the affected patients than in controls. The absolute descent is greatest for the right cerebellar tonsil, though the variability is great. The tonsils typically both descend into the foramen magnum and mold to some degree about the brainstem while the typical control patient shows only minimal presence of the tonsils in the foramen magnum. This will reduce the relative CSF space within the foramen magnum and increase the pressure gradient needed to move CSF through the foramen during systole and diastole. The mid and upper cervical segments fail to enlarge to a normal degree and remain significantly narrower than in control patients. The canal from C5 rostrally is about 1.5-2.0 mm smaller than control subjects. The narrower canal and tighter foramen magnum will increase the liability of the patient to hyperextension injuries. These are particularly prominent in “whip-lash” like events, during some surgeries, falls, and some forms of forceful forward bending. The subtle nature of these differences, though real, mean that accurate diagnosis must be based upon a very thorough and critical neurological exam anatomically related to the upper spinal cord and/or brainstem (part I). Merely using the MR scan, or worse, its radiological interpretation, as a screen for the presence of compression will usually be misleading.

    CONCLUSIONS:

    1. Patients
    2. with the diagnosis of fibromyalgia and NMH/POTS represent a population radiographically separate from the asymptomatic control population.
    3. These
    4. distinctions relate to the posterior fossa/brainstem and/or the cervical spine and cord:
      • The
      • cerebellar tonsils are lower
      • The
      • foramen magnum is more crowded
      • The
      • upper cervical segments (C1-C5) are narrower
    5. These
    6. relationships correspond anatomically to the neurological examination findings presented in part I.
    7. The
    8. findings are in the direction of developmental cervical stenosis and/or the hypoplastic posterior fossa, either of which can be associated with compression of the brainstem and or cervical spinal cord and which are amenable to surgical decompression when symptoms are severe enough.
    9. However
    10. , the overlap of means and the standard deviations of the two populations is such that it is not possible:
      • To
      • identify surgical candidates by screening MR scans of the neck and posterior fossa independently of the neurological history and examination.
      • The
      • radiologist and others will read most scans as being ”within normal limits.”

    THE NEUROSURGICAL SUBSET OF FIBROMYALGIA: PART III: FUNCTIONAL AND SYMPTOMATIC OUTCOME AT 12 MONTHS POST-OPERATIVELY.
    Michael J. Rosner, Jorge Flechas, Royce K. Bailey

    OBJECTIVE: Test the hypothesis that symptoms and function will be improved at 12 months after surgery when compared to the same measures obtained preoperatively. METHODS: 37 patients were selected based upon an abnormal tilt table examination defining cardiovascular/autonomic dysfunction, abnormalities identified from the neurological examination, and supported by radiographic changes at the foramen magnum and or cervical canal. Patients then underwent decompression of the involved region. They were followed longitudinally with repeat neurological examinations, questionnaires for grading of symptoms including incremental and visual analog scales of outcome and/or symptomatic improvement. Systat 7.0 and Excel were used for data analysis (all values X + SD). RESULTS: Ten measures of pain including joint pain, muscle pain, neck pain, headache, burning legs and others improved dramatically (p = 0.017-0.0000002). Dolorimetry is reported separately. Six cardiovascular symptoms including palpitations, SOB, dizziness, non-cardiac chest pain all improved (p= 0.016-0.000009). Excess fatigue decreased from a severe problem to a mild problem at 12 months (p=1.5E-9) along with three other measures of fatigue (p=0.00059-0.000007). Alimentary symptoms, cognitive complaints, sexual function, depression and others improved in a highly reliable fashion. The number of days/week patients felt “good” increased from 1.0 + 1.5 to 3.9 + 2.3 (p= 1.7E-11) at 12 months; only about 30% of patients were working pre-operatively compared with 80% at one year (p= 0.000015); this was paralleled by a large reduction in “days missed from work”. There were no surgical deaths, nor serious complications. No patient worsened neurologically. CONCLUSION: There is a subset of patients with radiological changes consistent with neurological abnormalities who can undergo posterior fossa and/or cervical decompression with important and long lasting improvement in quality of life as measured by their assessments of pain, multiple somatic symptoms, and the ability to function within the home and workplace. The risks of surgical decompression should be minimal. Fibromyalgia syndrome should not be used as a diagnosis for those with neurological findings. INTRODUCTION: The purpose of identifying posterior fossa and/or cervical cord compression is to provide direction in surgical decompression. Such decompression can be achieved with standard cervical laminectomy and/or forms of posterior fossa craniectomy. However, the array of symptoms with which those with the diagnosis of FMS complain is often considered ‘non-anatomical’ and probably psychosomatic in origin by most surgeons and they doubt that significant improvement in well being or function can be achieved for most of these individuals. OVERALL HYPOTHESIS: Symptoms will improve when compared with those of the preoperative state by 12 months after surgery. Hypothesis statement and testing was of the form:
    H1: μ1 ≠ μ0
    H0: μ1 = μ0

    METHODS: 52 patients with the established diagnosis of fibromyalgia and NMH/POTS were prospectively given questionnaires dealing with their symptoms and ability to perform activities of daily living, including their occupation. The questionnaires were administered preoperatively and at each post-operative visit, typically at 6 weeks, 6 months and 12 months (or longer) after surgery. The questionnaire asked the patient to grade symptoms as none, mild, moderate or severe. Other symptoms were graded on a visual analog scale of severity and converted to a 0-100 scale. Data were entered into Excel and/or Systat 7.0 for statistical analysis. Because of the sample size, most analyses were done with parametric statistics such as Student’s t-test for matched pairs. “Not a symptom” was coded as ‘0’; mild symptoms were coded ‘1’; moderate symptoms were coded ‘2’ and severe symptoms were coded as ‘3’. For double checks and entries splitting lines, answers were coded as 1.5, 2.5, etc.

    Results:  Symptoms (all values X + SC; [median])

    Pain Outcome
    Symptom

        Pre-Op

    12 mo Post

    P value

    Neck Pain

    2.5 + 0.9 [3.0]

    1.9 + 0.9 [2.0]

    P = 0.00076

    Headache

    2.5 + 0.8 [3.0]

    1.6 + 1.0 [2.0]

    P = 0.0000002

    Tender skin

    1.8 + 1.1 [2.0]

    0.8 + 0.8 [1.0]

    P = 0.0000017

    Burning legs

    1.4 + 1.2 [1.0]

    0.8 + 1.0 [0.0]

    P = 0.017

    Keeps awake

    2.1 + 1.1 [2.0]

    1.2 + 1.1 [1.0]

    P = 0.00014

    Joint Pain

    1.8 + 1.1 [2.0]

    1.0 + 1.0 [1.0]

    P = 0.00092

    Muscle Pain

    2.2 + 1.1 [3.0]

    1.3 + 1.0 [1.0]

    P = 0.0001

    Exertional Pain

    2.1 + 1.0 [2.0]

    1.3 + 1.2 [1.0]

    P = 0.00032

    Night Pain

    2.1 + 1.1 [2.0]

    1.2 + 1.1 [1.0]

    P = 0.017

    Back Pain

    2.2 + 1.0 [2.0]

    1.5 + 1.0 [2.0]

    P = 0.0014

    Cardiovascular Symptoms

        Pre-Op

    12 mo Post

    P value

    Palpitations

    1.0 + 1.1 [0.0]

    0.47 + 0.72 [0.0]

    P =0.0051

    SOB

    1.4 + 1.0 [1.0]

    0.67 + 0.82 [0.5]

    P =0.00077

    Flushing

    1.2 + 1.0 [1.0]

    0.69 + 0.93 [0.0]

    P =0.016

    Dizziness

    1.8 + 1.0 [2.0]

    0.90 + 0.80 [1.0]

    P =0.000092

    Cold Hands

    1.1 + 1.1 [1.0]

    0.50 + 0.80 [0.0]

    P =0.016

    Chest Pain

    1.0 + 1.0 [1.0]

    0.33 + 0.61 [0.0]

    P =0.00036

    Alimentary Symptoms

        Pre-Op

    12 mo Post

    P value

    Abdomin cramps

    0.9 + 1.0 [1.0]

       0.3 + 0.5 [0.0]

    P = 0.001

    GERD

    1.1 + 1.2 [1.0]

       0.5 + 0.9 [0.0]

    P = 0.015

    Dysphagia

    1.2 + 1.0 [1.0]

       0.6 + 0.8 [0.0]

    P = 0.0036

    Diarrhea

    1.1 + 1.1 [1.0]

        0.4 + 0.8 [0.0]

    P = 0.0004

    Constipation

    1.3 + 1.1 [1.0]

       1.0 + 1.1 [0.5]

    P = 0.07

    Nausea

    1.3 + 0.9 [1.0]

       0.8 + 0.9 [0.0]

    P = 0.050

    Fatigue Outcome

        Pre-Op

    12 mo Post

    P value

    Excess gatigue

    2.5 + 0.8 [3.0]

    1.4 + 1.2 [1.0]

    P = 1.5E –9

    Awaken tired

    2.4 + 0.9 [3.0]

    1.5 + 1.1 [1.0]

    P = 0.000010

    Poor sleep

    2.3 + 1.0 [3.0]

    1.3 + 1.0 [1.0]

    P = 0.0000073

    Insomnia

    .0 + 1.1 [2.0]

    1.2 + 1.0 [1.0]

    P = 0.00059

    Cognitive Outcome

        Pre-Op

    12 mo Post

    P value

    Concentration

    2.0 + 1.1 [2.0]

    1.3 + 0.9 [1.0]

    P = 0.00073

    Reasoning

    1.2 + 1.2 [1.0]

    0.4 + 0.6 [0.0]

    P = 0.00027

    Memory

    1.8 + 1.1 [2.0]

    1.4 + 0.9 [1.0]

    P =0.041

    Functional Outcome

        Pre-Op

    12 mo Post

    P value

    Days felt good

    1.0 + 1.5 [0.0]

    3.9 + 2.3 [4.0]

    P = 1.7E -11

    Working

    0.3 + 0.4 [0.0]

    0.8 + 0.4 [1.0]

    P = 0.000015

    Days missed work

    3.5 + 2.7 [3.0]

    1.8 + 2.1 [1.0]

    P = 0.0085

    Miscellaneous Symptoms

        Pre-Op

    12 mo Post

    P Value

    Anger

    1.2 + 1.0 [1.0]

    0.8 + 1.0 [0.0]

    P = 0.027

    Depression

    1.7 + 1.0 [2.0]

    1.1 + 1.2 [1.0]

    P = 0.0075

    Nervousness

    1.6 + 1.1 [2.0]

    1.0 + 1.1 [0.75]

    P = 0.00077

    Poor libido

    1.4 + 1.3 [1.0]

    0.9 + 1.1 [0.0]

    P = 0.032

    Poor orgasm

    1.2 + 1.2 [1.0]

    0.7 + 1.0 [0.0]

    P = 0.039

    Irritability

    1.7 + 1.0 [2.0]

    1.0 + 1.0 [1.0]

    P = 0.000196

    DISCUSSION: The cardinal symptom of fibromyalgia is pain. These data suggest that in the patient’s view of himself/herself, pain expressed in numerous fashions and manifestations improves throughout the year after surgery. Similarly, the cardiovascular symptoms related to NMH/POTS also improve in important and consistent ways. The relationship of the upper spinal cord and brainstem to cardiovascular symptoms is clear cut, and it is no surprise that gastrointestinal symptoms should also improve: The GI tract is integrated in its activity at the level of the lower brainstem. Improvement in fatigue and the ability to focus and concentrate probably relate to the reticular activating system, which is prominent at this (medullary) level. More importantly than any given ‘symptom’ is the concept that the upper cervical spinal cord/medulla is capable of causing dysfunction in multiple vegetative systems which lead to a plethora of somatic complaints. All too often, physicians dismiss these complaints as psychosomatic/ non-organic/non-an-atomic and refer the patient for psychiatric evaluation—a dead end for most. The brainstem and upper cord are the one area of the nervous system where all of these functions coincide, including pain control. The importance of this issue lies in the ability to improve functional outcome. By one year, about 78-80% of patients had resumed work. Pre-operatively, only about 30% were still employed and this was often tenuous. This figure is paralleled by improvements in feelings of well-being.

    CONCLUSIONS:

    1. Surgical
    2. decompression of the posterior fossa and or cervical spine significantly reduces somatic complaints.
    3. The
    4. reduction in pain, fatigue and multiple other complaints persists and actually improves through at least the first post-operative year. “Placebo” effect of surgery is highly unlikely.
    5. The
    6. reduction in complaints and symptoms leads to important increases in employment and ability to function both within and without the home environment.
    7. The
    8. improvement in patients’ symptoms includes a large improvement in autonomic dysfunction such as the array of GI complaints often called ‘Irritable Bowel Syndrome’, bladder function improves as well as the symptoms associated with NMH/POTS.
    9. Brainstem
    10. and upper cervical cord involvement should be suspected in the face of multiple dysautonomias with or without somatic complaints of numbness, weakness, ataxia, etc.

    National Fibromyalgia Research Association
    Neuro and New Treatment Modalities in FM
    Symposium—Portland, OR—Oct. 2002

    FIBROMYALGIA: PART IV: LONG TERM OUTCOME AS MEASURED BY DOLORIMETRY
    Flechas, J.D and Rosner, M.J.

    OBJECTIVE: Previous studies of patients with spinal cord injury, such as laceration, compression, and/or injury have described a pain syndrome, which is descriptive of fibromyalgia syndrome (FMS) pain. We hypothesized that dolorimeter (DOL) scores would go up as a function of time after surgery to decompress the spinal cord in patients with FMS. METHODS: All patients met the American College of Rheumatology classification criteria for FMS. Patients also had to demonstrate on MRI, spinal cord compression. Before surgery was considered, they had to show neurological dysfunction, such as numbness, limb weakness, neurally mediated hypotension etc. If their symptoms were well controlled by medication, surgery was not a consideration. At no time was surgery done for FMS pain. DOL was done at the time of initial office visit (IOV), prior to surgery (PTS), and at different times post op (PO) over the next year. Data was analyzed by student t test. Avg age 45. CONCLUSION: Spinal cord pain, which initially is mistaken as FMS pain, does statistically improve with decompression of the cord. The data supports that pain thresholds improve as a function of time.

    Results: Units are as follows:
    DOL (kg/cm2)+SEM, Time (weeks)+SEM

    Time DOL  N        Std Dev   P-Value
    IOV 43.7+2.7 62 21  
    PTS   43.2+3.2 63 21  
    6.4+.2 66.6+5.2 37 32 .00007
    13+.4 79.1+7.5 31 42 .002   

    INTRODUCTION: Previous studies of patients with spinal cord injury, such as laceration, compression, and or injury have described a pain syndrome, which is descriptive of fibromyalgia syndrome (FMS) pain1, 3. The patients in these studies described their pain as burning and/or stinging. They also had a feeling of pulling or pressure. Cramping, stabbing, and tingling/numbness were also seen. Exercise would make one half of them worse. Tension, overexertion and changes in weather were said to aggravate their pain. OBJECTIVE: Dolorimeter measurements have been used for years to assess how much pain fibromyalgia patients have. We hypothesized that dolorimeter (DOL) scores would increase as a function of time after surgery to decompress the spinal cord in patients with FMS. METHODS: All patients met the American College of Rheumatology classification criteria for FMS. Patients also had to demonstrate on MRI, spinal cord compression. No patients with spinal cord laceration, or injury were considered for this study. All patients were neurologically abnormal, with numbness, ataxia, dysphagia, dizziness, cranial nerve malfunction, limb weakness, etc. (see part I). All patients suffered from NMH/POTS and had abnormal tilt table examinations. The patients during this study were noted to have either a hypoplastic posterior fossa (roughly synonymous with Chiari malformation), and/or cervical spinal stenosis. The latter was usually developmental with degenerative change superimposed. If their symptoms were well controlled by medication, surgery was not a consideration. At no time was surgery done for FMS pain. DOL was done at the time of initial office visit (IOV), prior to surgery (PTS), and at different times post op (PO) over the next year. Data were entered into Systat 7.0 and analyzed by student’s t-test. Average patient age was 45.

    N

    Time (wks + SEM)

    Dolorimetry SD SEM P-Value
    62 Initial Visit   43.7

    +21

    +2.7

     

    63

    Pre-op

     

    43.2

    +21

    +3.2

     

    37

    6.4

    +0.2

    66.6

    +32

    +5/2

    .00007

    31

    13

    +0.4

    79.1

    +42

    +7.5

    .002

    25

    20.8

    +0.4

    65.9

    +32

    +6.5

    .006

    22

    27.4

    +0.5

    68.8

    +39

    +8.3

    .004

    18

    35.8

    +0.5

    74.9

    +45

    +10.6

    .027

    12

    52.1

    +1.5

    88.2

    +39

    +11.2

    .017


    Units: DOL (kg/cm2) + SD + SEM, Time (weeks) + SEM

    DISCUSSION: The pain experienced by patients in this series decreases over time (Part III). Dolorimetry is a measure of pain threshold as opposed to pain itself. These data demonstrate that the brainstem and upper spinal cord are capable of altering pain threshold when compressed and that decompression of these areas is capable of reversing toward normal those thresholds. The consistent improvement over the first year after surgery shows that this is a gradual and progressive process with improvement over time for most patients. It is also important to view the altered pain threshold with local pain produced by the pressure stimulus as a central event, and not necessarily due to altered tissue function or structure. This is most clearly demonstrated in those with overt spinal cord injury1, 3. The pain in this group of fibromyalgia patients is myelopathic, while the issue is largely semantic, one could argue that this group of patients does not have fibromyalgia. Anatomically, the most direct etiology for a reduction in pain threshold with the brainstem and/or upper spinal cord compression is interference with the outflow of descending nociceptive control fibers originating in the nucleus raphe magnus and paragigantocellularis of the medulla oblongata2.

    CONCLUSIONS:

    1. Pain
    2. threshold as measured by dolorimetry can be returned toward normal after spinal and/or brainstem decompression.
    3. Dolorimetry
    4. and the concept of “number of tender points” do not discriminate between cord compression and tissue dysfunction from other etiologies.

    Reference List

    1. Botterell
    2. , E. H., Callaghan, J.C. an Jousse, A.T.: Pain in Paraplegia: Clinical management and surgical treatment. Proc. R. Soc Med 47:281-288,1954.
    3. Jessell
    4. TM, Kelly DD: Pain and analgesia, in Kandel ER, Schwartz JH, Jessell TM (eds): Principles of Neural Science, ed 3. Norwalk, Connecticut: Appleton & Lange, 1991, pp 385-399.
    5. Nepomuceno
    6. , C., Fine, P. R., Richards, J. S., et al: Pain in patients with spinal cord injury. Arch Phys Med Rehabil 60:606-608, 1979.

    National Fibromyalgia Research Association, Neurology and New Treatment Modalities in FM, Symposium. Portland, OR - Oct. 2002

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