The Spinal Series
This is part three of a series examining spinal issues which may mimic chronic fatigue syndrome (ME/CFS) and fibromyalgia. The first two in the series involved two very ill ME/CFS patients who have recovered or are recovering following surgery to correct craniocervical instability.
- Could Craniocervical Instability Be Causing ME/CFS, Fibromyalgia & POTS? The Spinal Series – Pt. I
- Jennifer Brea’s Amazing ME/CFS Recovering Story: the Spinal Series – Pt. II
Leader in the Field
Back in 1999, Peter Rowe was way, way ahead of the curve when he was the first to explicitly link ME/CFS to Ehlers Danlos Syndrome. Twenty years ago, he concluded: “These observations suggest that a careful search for hypermobility and connective tissue abnormalities should be part of the evaluation of patients with CFS and orthostatic intolerance syndromes.”
He was the second in the scientific literature to mention POTS and neurally mediated hypotension in connection with ME/CFS. In 2014, he was the first to report that reduced range of motion in the limbs and/or spine was commonly found in ME/CFS. In 2016, he demonstrated that seemingly innocuous movements such as leg lifts were able to produce unusual amounts of neuromuscular strain and symptoms in ME/CFS. Rowe speculated that these unusual findings could result from increased strain in and reduced blood flow to the spinal cord and its associated structures, as well as from mast cell activation. In 2017, he reported on chronic fatigue in Ehlers Danlos Syndrome (EDS). Last year, he was the first to link spinal stenosis with ME/CFS.
Is there a theme to all of this? Perhaps. Rowe has been digging into and finding connective tissue and autonomic nervous system abnormalities in ME/CFS, POTS and EDS for decades.
Peter Rowe’s Spinal Stenosis Chronic Fatigue Syndrome (ME/CFS) Group
J Transl Med. 2018 Feb 2;16(1):21. doi: 10.1186/s12967-018-1397-7. Improvement of severe myalgic encephalomyelitis/chronic fatigue syndrome symptoms following surgical treatment of cervical spinal stenosis. Rowe PC, Marden CL, Heinlein S, Edwards CC 2nd.
In his 2018 report, Peter Rowe presented three cases of severe chronic fatigue syndrome (ME/CFS) which resolved after surgery for spinal stenosis (narrowing of the canal the spinal cord goes through.) Each case, interestingly, had a different kind of onset.
The first person’s illness started with a gastrointestinal viral illness at age 12 which produced fatigue, unrefreshing sleep, post-exertional malaise (PEM), cognitive issues, headache, muscle and joint pain, sore throat, and tender glands. She later developed dizziness upon standing and was diagnosed with POTS at age 15. (She did not respond to POTS medications.) She dropped out of high school as a sophomore. Her Beighton score did not indicate Ehlers Danlos Syndrome (EDS) was present.
With her mother having undergone two procedures to correct congenital spinal stenosis, the condition clearly ran in her family. Nevertheless – possibly because of her ME/CFS label – spinal stenosis was not considered until she developed tinnitus. At age 21 – nine years after her first symptoms appeared – an MRI clearly showed she had a congenitally narrowed cervical spinal canal at C6-C7 with spondylotic (arthritic) stenosis.
Her improvement following cervical disc replacement at C6-C7 was remarkable. By six months, this young woman – who had been disabled for over five years – was working 12 hour shifts as a horse wrangler, saddling and feeding horses, leading rides and cleaning out horse stalls. A year after the surgery, she was at University full-time while working 20 hours a week. Five years later, she remains perfectly healthy.
Patient 2 had been generally active until age 29 when, without any evidence of an inciting event, she developed profound fatigue, unrefreshing sleep, PEM, cognitive problems, headaches, and muscle and joint pains, plus an array of neurological symptoms (burning in the legs while standing, numbness in the limbs, electric shock sensations in her arms, difficulty swallowing, and clumsy gait). To top it off, she developed anxiety and depression as well.
She, too, had a family as well as a personal history of connective tissue disorders. Her sister had scoliosis and Chiari I malformation, and she had had surgery (pectus excavatum repair) to correct a sunken breastbone and early onset varicose veins, which had been treated with vein ligation and striping at age 28. As with patient one, her Beighton score was low.
An MRI of the brain and spine at age 30 revealed she had mild degenerative disc desiccation extending from C3 through C7 that the neurologist did not believe was causing her symptoms. At age 31, she tested positive for POTS, but like patient 1, did not respond to treatments. Four years later, and seven years into her illness, she was in a wheelchair with severe PEM. This time, a finding of hyper-reflexia prompted another MRI, which indicated a greatly narrowed cervical canal (7mm) at C6-C7. Her gag reflex had also disappeared and she had reduced strength in her arms, but her Hoffman’s sign was negative.
A hybrid anterior cervical disc fusion and disc replacement resulted in slow but steady improvements. By 8 months, this formerly wheelchair-bound patient was able to walk for 35 minutes a day. By 20 months, she could “exercise on an elliptical machine and recumbent bicycle, perform daily house-keeping chores, run multiple errands in a day, and paint the interior rooms of her parents’ house.” She was also able to stop 3 of her 4 antidepressant/anti-anxiety medications.
In contrast to the other patients, this patient did not have a personal or family history of a connective tissue disorder. Her Beighton score, however, suggested she had Ehlers Danlos Syndrome (EDS). She was a healthy 31-year old until she developed severe fatigue following a trip overseas, and within four months had to stop working. Her symptoms were familiar to those with ME/CFS (unrefreshing sleep, post-exertional malaise, difficulty with concentration, headache, muscle and joint pains, nausea, lightheadedness, tremulousness, visual disturbances, and excessive thirst). As she got worse, anxiety and depression emerged.
She was quickly diagnosed with orthostatic intolerance (neurally mediated hypotension – severe drop in blood pressure after standing). Two years later, she developed POTS (high heart rate upon standing) as well and a positive Hoffman’s sign was present. As with the other patients in Rowe’s report, she responded poorly to POTS medications. As her symptoms progressed, she required a wheelchair for outside trips.
An MRI at age 35 indicated she had spondylosis with a canal diameter of 8.5 mm (at C5–C6). Three months after an anterior C5–C6 disc replacement, she was downhill skiing. At 7 months, she was able to exercise vigorously without provoking PEM. A year later, she reported minimal physical problems.
Another ME/CFS Patient Gets the Surgery
Esther didn’t think she had spinal stenosis, but after reading Dr. Rowe’s case report, she headed over to her orthopedist. She’d been seeing him since she woke up one morning a year and a half earlier with shoulder pain radiating down her arm and was unable to lift her arm above her head. Her orthopedist immediately ordered an MRI after reading Dr. Rowe’s report.
It wasn’t clear what her canal diameter was, but her stenosis – ranging from C4 to C7 – was much more extensive than in the three cases Dr. Rowe reported on. Her surgery occurred six weeks ago. It’s too early to tell how effective it will be, but she will keep us informed.
Dr. Rowe reported that he expected that cervical stenosis will be more common among those with more severe cases of ME/CFS.
- Check out a connection between the sympathetic nervous system and spinal problems and how treating one person’s severe scoliosis improved his energy – Chronic fatigue syndrome and your bad back
The only large study of spinal stenosis in ME/CFS or FM was done in fibromyalgia. In 2004, Heffetz’s found cervical spinal stenosis to be quite common in his neurological and neuroradiological exams of 270 people with FM.
It should be noted that this group was probably not reflective of fibromyalgia in general. The patients were referred by rheumatologists (66%) or contacted the doctor hoping to get in a study examining the neurological basis of FM. A higher percentage than normal (59%) had also sustained some sort of usually mild head trauma, and the rate of disability appeared to be quite high. They’d seen an average of 10 different medical specialists and were taking 5 prescription drugs. Almost 70% were no longer working. They were a very ill group.
Many symptoms (neck/back pain (95%), fatigue (95%), exertional fatigue (96%), cognitive impairment (92%), instability of gait (85%), grip weakness (83%), tingling (80%), dizziness (71%) and numbness (69%) suggested spinal cord involvement. The degree of exertional fatigue (PEM) exceeded that seen in some ME/CFS studies.
The neurological exam produced other signs (upper thoracic spinothalamic sensory level (83%), hyperreflexia (64%), of the radial periosteal reflex (57%), positive Romberg sign (28%), ankle clonus (25%), positive Hoffman’s sign (26%), impaired tandem walk (23%)) suggestive of spinal cord damage or myelopathy.
Anything less than 13 mm spinal canal diameter suggests spinal canal stenosis. With their neck in extension, the average spinal canal diameter of this group at C5/6 was 10 mm. Only four of the FM patients in the study had a spinal canal diameter of 13 mm or more.
Forty of the participants in the study who later underwent surgery reported a significant improvement in their FM symptoms including pain, fatigue, and cognition, reduced depression and anxiety and improved quality of life (SF-36) .
Cervical Spinal Stenosis
Cervical spinal stenosis (from the Greek stenos – narrow) occurs when a pinching in the spinal canal compresses the spinal cord in the neck – interrupting the flow of nervous system signals. (Spinal stenosis can also occur in the mid-back and lumbar regions.) Rowe reports that a spinal canal with a less than 13 mm diameter is a strong risk factor for spinal cord damage or “myelopathy”.
The risk of spinal stenosis increases with age and often occurs when arthritis of the spinal column and discs narrows the effective space of the canal. It can also be caused by a thickening of the ligaments in the back or by bulging discs, and it can be congenital (inherited). When spinal stenosis is inherited, symptoms usually start to show up in middle age.
Symptoms typically start gradually and slowly get worse. (Some people with spinal stenosis, however, experience no symptoms at all. In fact, one study of elderly patients with low back stenosis found that many also had cervical (neck) and/or thoracic (mid-back) stenosis but were asymptomatic.) When moderate to severe stenosis is causing symptoms, surgery appears to be the only option.
The College of Rheumatology reports spinal stenosis can cause symptoms such as pain, cramping, weakness or numbness in the lower back and legs, neck, shoulders or arms, and WebMD has a similar symptom list (stiffness, back pain, numbness, foot drop, sciatica, problems walking, loss of bladder or bowel control (lumbar stenosis). While neck pain is often present, some people do not report neck pain.
No one looking at these symptoms would consider that spinal stenosis might be able to cause ME/CFS and fibromyalgia. Other researchers, however, have asserted that spinal cord compression can cause far more symptoms, including autonomic nervous system symptoms such as racing heart, problems standing, cognitive issues, etc.
Heffetz reported that “muscular fatigue worsened by exertion is a well-described symptom of cervical myelopathy,” and that chronic fatigue was common (but by no means universal – 57%) in Chiari I malformation. Heffetz also found that FM patients with spinal cord issues were often misdiagnosed with psychogenic illnesses like depression and anxiety (59%).
Like Jeff and Jen with their craniocervical instability, the three patients in Rowe’s survey appeared to be different from typical spinal stenosis patients. All had POTS, extreme fatigue, post-exertional malaise (PEM) and cognitive problems – and all had been diagnosed with ME/CFS; i.e., they appear to be a rather unusual ME/CFS/POTS-like spinal stenosis subset.
Diagnosing Cervical Spinal Stenosis
Dr. Rowe recommends MRI studies in individuals with abnormal findings following a careful neurological examination. Those findings include pathologically brisk reflexes, a positive Hoffman’s sign, abnormal Romberg sign, or other abnormalities suggestive of a cervical myelopathy. A family history of the disease can also support getting an MRI. Dr. Rowe emphasized that he does not obtain cervical spine MRIs in all ME/CFS patients.
Showing hyperreflexia, clonus and a positive Hoffman’s sign
Both Rowe and Heffetz suggested that the bias associated with a chronic fatigue syndrome and fibromyalgia diagnosis may be causing some doctors to not dig deep enough when confronted with signs of spinal stenosis.
Because few doctors will think of spinal stenosis in connection with ME/CFS or FM, showing them Dr. Rowe’s case report and Dr. Heffetz’s fibromyalgia study (both of which are free (use PDF button on upper left to print)) should help. Both papers specifically note that spinal stenosis can cause the symptoms associated with ME/CFS and fibromyalgia.
Various medical websites cite a variety of tests (x-rays, MRI, CT scans) used to diagnose spinal stenosis. If spinal stenosis is being checked, though, other spinal issues sometimes associated with ME/CFS and FM such as craniocervical instability, Chiari malformation and tethered cord syndrome should be checked at the same time. CCI requires a top-of-the-line MRI. Testing with the head extended / flexed appears to be recommended for both spinal stenosis and CCI.
Jeff provides important advice for getting tested and diagnosed with CCI – including how to get your scans into the right hands. Most neurosurgeons aren’t trained to recognize craniocervical instability (CCI), and finding an imaging facility that does the right kind of scans can take time.
Dr. Rowe reported that it’s impossible to know how prevalent cervical spinal stenosis is in ME/CFS until “a study across a range of mild to severely affected patients is able to provide a precise estimate of its prevalence.” He stated that it appears to be present but is probably “uncommon in ME/CFS”. Several other of his ME/CFS patients – beyond those reported in the paper – have been identified with cervical spinal stenosis.
Dr. Rowe also noted the need to look for other spinal conditions, such as instability of the cervical spine and instability of the craniocervical junction. He reported that a small number of his adolescent patients have been diagnosed with craniocervical instability (CCI) – a known complication of joint hypermobility and Ehlers-Danlos syndrome. He noted that all of those diagnosed with CCI had described a sense of having a heavy head or a head that was not supported well. (One person’s head was so poorly supported that she was called “bobble head” by her classmates.)
His CCI/AAI patients also described increased symptoms when their necks were flexed, moved laterally or extended in positions of neck flexion, lateral rotation, or extension. Some also displayed autonomic problems and neck pain following relatively mild head trauma.
Connective Tissue Issues
Dr. Rowe, as noted earlier, has been a leader in uncovering the connective tissue issues in ME/CFS and POTS. With one of the case reports testing positive for hypermobility on the Beighton test, having pectus excavatum (sunken chest) and apparently severe varicose veins, I asked him what connective tissue issues he looked for when assessing his patients. (My sole connective tissue issue is a mild case of pectus carinatum.) After noting that the Beighton test does not cover hip and shoulder hypermobility, he stated that:
“We look for the entire range of abnormalities seen in association with hypermobility spectrum disorders, including skin hyperextensibility, easy eversion of the eyelids, Gorlin’s sign (touching the tongue to the tip of the nose), widened scars, unexpected stretch marks, piezogenic papules of the ankles, flat feet, dental crowding, and so on.”
Rowe noted that joint hypermobility can produce a number of anatomical problems that affect the nervous system, including ligament laxity at the base of the skull (cranio-cervical instability), early degenerative disc disease, cervical spine instability and kyphosis (hunchback/roundback), dynamic cervical compression, and Chiari malformation. Other possible complications include tethered cord syndrome, Tarlov Cyst Syndrome, intracranial hypertension and temporomandibular joint disease.
Rowe’s and Heffetz’s report indicates that not only is spinal stenosis (narrowed spinal canal) present in ME/CFS and FM, but that surgery to correct it can return some people, even some very disabled people, to health. The big question is how commonly these and other spinal conditions are actually causing or exacerbating these diseases.
Chiari malformation provides a cautionary note with regard to spinal issues. At one time considered a top possible diagnosis in FM, a large 2004 study (n=244) indicated it was no more common in FM than in healthy controls and was pretty rare to boot (2.8% of FM patients).
Dr. Heffetz’s study, because of the way it was structured, almost certainly overestimated the amount of spinal stenosis present in FM. Still, Heffetz found so much spinal stenosis (46%) in his 2004 study that even knocking that percentage down a considerable amount could still leave a substantial number of people with FM with this condition.
The 40 people in in Heffetz’s study who underwent surgery reported significant gains in significant improvement in their FM symptoms including pain, fatigue, and cognition, reduced depression and anxiety and improved quality of life (SF-36).
Unfortunately it appears impossible at this point to know if someone’s spinal stenosis is contributing to their ME/CFS/FM or not. The fact that spinal stenosis become more common as we age and does not always cause symptoms obviously complicates matters. Dr. Rowe believes that people with severe ME/CFS will be more likely to have cervical stenosis.
Dr. Rowe believes spinal stenosis (and presumably other spinal conditions such as craniocervical instability, tethered cord syndrome, Chiari malformation) are probably not common in ME/CFS, but because they can occur, and in selected cases can even be causing the disease, he strongly recommends a full neurological examination be done on everyone with ME/CFS and FM.
Health Rising’s ME/CFS and FM Spinal Series
- Could Craniocervical Instability Be Causing ME/CFS, Fibromyalgia & POTS? Pt I – The Spine Series
- Jennifer Brea’s Amazing ME/CFS Recovering Story
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