Cervical spine stenosis as a cause of severe ME/CFS and orthostatic intolerance symptoms

[Tom Kindlon]

Poster presentation, IACFS/ME 2016 conference

Cervical spine stenosis as a cause of severe ME/CFS and orthostatic intolerance symptoms

Peter C. Rowe, M.D*, Colleen L. Marden, Scott Heinlein, PT, Charles Edwards II, M.D.

Background:

Comparatively little has been published on the clinical features and management of severe forms of ME/CFS.

Objectives:

To describe the presenting symptoms and neurological examination findings in three young adult women whose disabling ME/CFS symptoms and orthostatic intolerance improved after the recognition and surgical management of cervical spine stenosis (CSS).

Methods:

This retrospective case series includes three consecutive individuals who (1) met the Fukuda and criteria for CFS, (2) had evidence of refractory orthostatic intolerance, (3) were unable to work or attend school, and (4) were minimally responsive to medical and psychiatric management. To investigate pathological reflex findings, all underwent MRI evaluations. CSS was considered present if the AP cervical spinal canal diameter (SCD) was less than 10 mm at any level. Overall function was assessed before and after cervical disc replacement surgery using (1) a clinician-assigned Karnofsky score (range 0 to 100) and (2) the SF-36 physical function (PF) subscale score (range 10-30). Higher scores indicate better function on both measures.

Results:

Age at onset of symptoms was 12, 29, and 29 years. The onset of ME/CFS was acute in all three. Neurological exam findings included > 3+ (brisk) deep tendon reflexes (DTR) in 2/3, positive Hoffman sign in 2/3, tremor in 2/3, and absent gag reflex in 1/3. Diagnosis was delayed for 6-9 years after the onset of symptoms. Brain MRIs were normal. The youngest patient had congenital CSS with a single level disc protrusion at C5-6 that caused further ventral cord compression and a SCD of 7 mm. Her mother also has cervical stenosis. A second patient had two disc protrusions at C5-6 and C6-7 with SCD of 7 and 9 mm, and myelomalacia (this patient has a sibling with Chiari I malformation). The third had acquired CSS due to a single level disc bulge at C5-6 (SCD = 8.5 mm).

Improvements were evident within 2 months of single-level cervical disc replacement surgery (one patient also had fusion at an adjacent level). After 16-40 months of follow-up, all reported improved fatigue, cognitive dysfunction, PEM, lightheadedness, and anxiety. The pre- to post-op SF-36 PF scores improved from 13 to 30, 18 to 30, and 16 to 26, respectively, and the Karnofsky scores improved from 40 to 90, 40 to 90, and 50 to 100, respectively. Standing tests conducted at variable intervals from pre- to post-op showed a reduction in the maximal heart rate (HR) change during 5 minutes of standing from 64 to 22 bpm, 42 to 29 bpm, and 34 to 27 bpm, respectively.

Conclusion:

This case series draws attention to the potential for CSS to contribute to ME/CFS and orthostatic symptoms, extending work by Heffez in fibromyalgia (Eur Spine J 2004;13:516). Further work is needed to define indications for surgery. However, the improvements in HR and function following surgery emphasize the importance of detecting and treating CSS, especially in the subset of those with ME/CFS whose severe symptoms are refractory to other interventions.

Peter C. Rowe, M.D.
Professor of Pediatrics
Johns Hopkins University School of Medicine/200 N. Wolfe Street/Room 2077
Baltimore, MD 21287
prowe@jhmi.edu

Dr. Rowe is supported by the Sunshine Natural Wellbeing Foundation Professorship in Chronic Fatigue and Related Disorders. No author has a conflict of interest.

https://twitter.com/i/web/status/797125285095280640

 

[BurnA]

So what theory does this study fit into ?

 

[Cheesus]

So what theory does this study fit into ?

There are some osteopathic interventions, such as the Perrin Technique, that it resonates with. In the Perrin Technique, abnormalities within the spine lead to the breakdown of the 'cranial mechanism', which is apparently responsible for the movement and drainage of cerebral spinal fluid. As a result of this breakdown, the theory goes, there is a build-up of toxins in the central nervous system which triggers chronic sympathetic hyperactivity.

I have spoken with a number of people who report a lot of improvement with the Perrin Technique, however there are many more who do not gain at all. Perhaps it is applicable to a subset of patients.

 

[daisybell]

Compressing the spinal cord results in lots of different symptoms - including weakness, tingling and sensory loss, poor balance etc etc. I think it's rare in young people, but as you age, you can get bony spurs growing on the vertebrae (osteophytes) which can compress the spinal cord...

 

[trishrhymes]

I think what they are saying in effect is that these patients had been misdiagnosed as ME/CFS when their symptoms were actually caused by structural compression of the spinal cord in the neck which resolved with surgery. Looks to me like another condition that should be watched for out in diagnosis. I hope they will do a bigger study to find how prevalent this is.

 

[anciendaze]

Does this remind anyone of Paulo Zamboni's surgery to correct CCSVI?

 

[taniaaust1]

Compressing the spinal cord results in lots of different symptoms - including weakness, tingling and sensory loss, poor balance etc etc. I think it's rare in young people, but as you age, you can get bony spurs growing on the vertebrae (osteophytes) which can compress the spinal cord...

I have one of those bone spurs (due to osteroarthritis which I have in several places in my spine) in my neck area which 4-5 years ago I was told I'd one day need surgery on it due to the way its facing and that it will compress. It makes me wonder though I treated the osteroarthritis with supplements for a couple of years which got rid of the neck pain and inflammation there, it makes me wonder reading this if it could be impacting my ME.

the more research comes out, the more and more things I can see probably are wrong in my case.

 

[taniaaust1]

for anyone wondering "The Hoffman reflex has also been used as a measure of spinal reflex processing (adaptation) in response to exercise training. The test involves tapping the nail or flicking the terminal phalanx of the middle or ring finger. A positive response is seen with flexion of the terminal phalanx of the thumb."

2/3 of those ones had this, how many of us here do have this? (why havent we heard of this before)

youtube video of this reaction

(I cant seem to invoke this on myself) . The below on testing is doing it a bit different

 

[taniaaust1]

video of findings of someone with cervical spine stenosis and the testing. Hoffmans test from 1:40

Ive been diagnosed with having severe hyper-reflexia (dr almost got kicked in face when testing me as my leg jumped up full expension when hit with little hammer , 90 degree) .. though I dont know if I currently have it.. and myoclonus. I need to find someone to test me for this other thing.

 

[CCC]

@taniaaust1 I have messaged you ... Well, I think that's what I've done

 

[Chezboo]

I had never heard of the Hoffman sign but it appears I have it. Guess I can add it to my very long list.

 

[nandixon]

So what theory does this study fit into ?

Inflammatory cytokines are generated with compression of the spinal cord. Perhaps these cytokines are similar to those produced with other ME/CFS triggers (e.g., viral, etc), and perhaps those cytokines are mediating the disease.

 

[anciendaze]

Just want to mention that MS is strongly associated with weakness in the legs, and Dr. Zamboni has found some patients with what he call CCSVI who improve dramatically after surgical correction. This is clearly not a general treatment for MS, but it raises the question of how many "MS" patients actually have spinal stenosis in the lumbar region, in addition to those with restriction in the spinal cervical (neck) region like these patients. A second question has to do with autoimmune disease triggered by sterile cell death due to ischemia. How common is such a thing?

One reason we have no official etiology for MS could be the confounding effects of mixing cohorts with different problems.

 

[Denise]

I think what they are saying in effect is that these patients had been misdiagnosed as ME/CFS when their symptoms were actually caused by structural compression of the spinal cord in the neck which resolved with surgery. Looks to me like another condition that should be watched for out in diagnosis. I hope they will do a bigger study to find how prevalent this is.

I interpreted them as saying that the cervical spine problems are something that should be considered but that having problems with the cervical spine doesn't necessarily negate a diagnosis of ME. If there is something treatable that will improve patients' quality of life, let's do find it.

A(t least a) few ME specialists regularly test Hoffman signs, reflexes, etc. but the authors seem to think that all those who treat ME should check these things. I wonder if they have suggestions on how often to check them.

 

[Marky90]

The improvement on SF-36 was very very slight, they were still severely ill.

 

[Denise]

The improvement on SF-36 was very very slight, they were still severely ill.

I thought so also so I queried Rowe who said they did not norm the SF-36 (to be x/100) so the results were 30 out of 30 and 26 out of 30.
They need to make this clearer.

 

[melihtas]

The improvement on SF-36 was very very slight, they were still severely ill.

Indeed there is inconsistency between SF-36 and Karnofsky scores.

KARNOFSKY PERFORMANCE STATUS SCALE DEFINITIONS RATING (%) CRITERIA

100 Normal no complaints; no evidence of disease.
90 Able to carry on normal activity; minor signs or symptoms of disease.
50 Requires considerable assistance and frequent medical care.
40 Disabled; requires special care and assistance.

 

[melihtas]

I thought so also so I queried Rowe who said they did not norm the SF-36 (to be x/100) so the results were 30 out of 30 and 26 out of 30.
They need to make this clearer.

Thanks for clarifying, now this paper gives me more hope than any other therapy options. I have already made an appointment with a neurosurgeon for next week.

 

[anciendaze]

A central problem for these patients seems to have been orthostatic intolerance, and SF-36 scores seem to be almost independent of OI. In fact, I would hazard a guess that many measures of health have been designed to exclude OI on the grounds that patients can do things if they push themselves, they just don't want to.

 

[melihtas]

A central problem for these patients seems to have been orthostatic intolerance.

Why do you assume that their central problem was OI? According to the paper, they were clearly diagnosed with ME/CFS.

three young adult women whose disabling ME/CFS symptoms and orthostatic intolerance improved

three consecutive individuals who met the Fukuda and criteria for CFS, had evidence of refractory orthostatic intolerance,

After 16-40 months of follow-up, all reported improved fatigue, cognitive dysfunction, PEM, lightheadedness, and anxiety.