On February 25, 2002 The New Zealand Ministry of Health released a report commissioned by the government to review the subject of arachnoiditis. This report: Arachnoiditis: A brief summary of the literature was prepared by the Christchurch School of Medicine and Health Sciences, Christchurch, New Zealand staff headed by Peter Day and associates who have graciously allowed Burton Report® to make available a copy on Adobe .pdf format.
by Charles V. Burton, M.D.
Editor, Burton Report®
This report, by Peter Day and associates at the Christchurch School of Medicine, prepared under the auspices of the New Zealand Health Technology Assessment Clearing House, is a landmark document. This is the first time, in a century of global medical practice, that any government agency, in any country, has commissioned a report on this important subject.
The people of New Zealand, The New Zealand Ministry of Health and the Christchurch School of Medicine are to be complimented for having taken on this difficult challenge.
Any unbiased review of the subject of arachnoiditis is a difficult task because of the paucity of prior hard science on the subject. Part of this problem has been a lack of awareness on the part of the medical profession in general as well as some of the medical reporting which has reflected hidden agendas and conflicts of interest not made apparent to their readers. The foundation for legitimate health care planning is well-performed incidence and prevalence studies and data. Such have never yet been developed for this disease entity.
In a manner similar to that demonstrated by the tobacco industry, there has been an expenditure of many millions of dollars intended to obfuscate and provide “damage control” by the manufacturers of oil myelogram substances to thwart their being held responsible, in the legal arena, for their transgressions against the public. This is also an important part of the history of the arachnoiditis saga. Today, many of the leading medical journals require full disclosure, by authors, of any real or potential conflicts of interest. These requirements were not in place when the literature reviewed by Day and associates was published.
Day and associates accurately point out the many limitations of the information they reviewed but also make the point that this information base “can produce valid results.”
How “rare” is clinically significant adhesive arachnoiditis?
It has become clear that every person who has ever had a oil myelogram (i.e. lipiodol, pantopaque or myodil) has been left with permanent scarring of their pia-arachnoid membranes and some related impairment of cerebrospinal fluid production. From the 1940s to the 1980s there were approximately 1 million oil myelograms performed each year throughout the world. Scarring of the meninges secondary to exposure to these foreign body substances occurred in every single case. How many of these situations progressed to the stage of advanced “chronic adhesive arachnoiditis” is simply not known. The actual number of cases is which this inflammatory process ascended up the spine to the brain producing death is also not known. Sensitivity to inflammation is now known to a complex process involving issues such as the individual’s own immunologic makeup.
It is readily apparent that although prevalence data is lacking the numbers of those afflicted with meningeal scarring is quite high. What then is the incidence of those individuals from this group who have become disabled by this condition (referred to as:(“clinically significant adhesive arachnoiditis”)? What is “rare”? It’s meaning is different to each beholder. Long has estimated that 1% of those with adhesive arachnoiditis are “clinically significant.” This editor believes that 5% is a more accurate estimate.
Why is the prevalence of chronic adhesive arachnoiditis so high and clinically significant adhesive arachnoiditis so low? The answer to this enigma may very well lie in the remarkable ability of the human body to successfully deal with insult and injury if the progression of adversity is sufficiently slow. This is particularly true of the nervous system. This means that if the progression of an inflammatory process is sufficiently slow the nerves are then allowed to have the opportunity of surviving in their function despite progressive encapsulation with scar, progressive loss of vascular supply and progressive decrease in nutrition normally supplied by the surrounding cerebro-spinal fluid. This also means that if the nerves are not allowed to have the opportunity of accommodating they then signal their distress to the brain by transmitting constant nociceptive information. The nature of the resulting regional complex pain disorder is very often totally disabling to the individual.
This also means that many who have the scarring and are asymptomatic exist in a precarious state. Additional insult can, in these cases, upset the balance producing decompensation and associated clinically evident problems. This type of situation is well known in medicine where large, benign, brain tumors progressively enlarge over many years and a minor incident (i.e. being struck in the head with a soccer ball) causes decompensation, unconsciousness, and even death. In the adhesive arachnoiditis cases the additional insult can be another myelogram, trauma such as a motor vehicle accident, or even an additional spinal surgery.
It is interesting to observe that clinically significant “chronic adhesive arachnoiditis” may be infrequent, or even “rare”, compared to the huge reservoir of existing cases. But it is also important to point out that even if these individuals appear normal they live with a “sword hanging over their heads” and are typically unaware of this liability. It is also important to recognize that even if there is no apparent clinical problem significant bodily injury has occurred. This is a situation similar to the “post-polio syndrome” where individuals afflicted with poliomyelitis at a early age loose many of their spinal neurons to the viral infection. Many individuals appear to recover completely and clinical problems may only become evident later in life when the paucity of remaining neurons is diminished further by the process of aging, are no longer able to meet the needs of the body.
If it is a “rare” entity why should New Zealand, and the rest of the world, be concerned with “clinically significant adhesive arachnoiditis”?
There are few disease processes more cruel and disabling than adhesive arachnoiditis when it is “clinically significant.” The nature of the constant pain is such that it prevents normal activity, intellectual pursuits and sleep. Adhesive arachnoiditis does not affect longevity and sufferers do not the relative blessing of the limited life expectancy afforded by terminal cancer. These individual are non-productive and require long-term supportive care.
It would have been nice to see that with the phasing out of oil myelography in the early 1980s that the issue of adhesive arachnoiditis would have become something of only historic interest. This has not been the case. The advent of epidural steroid injection as a primary treatment for back pain has created new populations of sufferers. How rare is this? Once again data on incidence and prevalence do not exist. In the United States he most reliable data on incidence are the number of physicians being brought to court by their patients. This sad state of affairs seems to reflect only ignorance on the part of physicians and their patients as epidural steroid administration can (and should be) a safe procedure performed with appropriate informed consent.
What needs to be done?
As correctly noted by Day and associates scientific study and further assessment of this disease entity are required. The most important role of this report, in my opinion, is being a first step in promoting awareness. In 1968 ago a physician wrote a letter to the editor of the New England Journal of Medicine noting that whenever he ate at a Chinese restaurant he would experience symptoms similar to those of a heart attack. Before long there were similar experiences shared by a multitude of other physicians. The entity became referred to as “the Chinese restaurant syndrome.” Investigation finally determined that a hypersensitivity to monosodium glutamate (MSG) and high salt content in the food appeared to be the etiology of this entity.
Only with increased awareness will physicians and patients begin to suspect, and then identify adhesive arachnoiditis. It should not be that a popular treatment for low back pain be allowed to create devastating disease for the patient. How much is the prevention of this sad patient experience worth to a concerned health care system?
The importance of awareness
In 1926 French neurologists Foix and Alajouanine published the description of a pathologic entity producing adhesions, spinal cord degeneration and paralysis. We now appreciate that the Foix-Alajouanine syndrome probably represented a congenital arterio-venous malformation of the spinal cord associated with small intermittent bleeds producing local adhesive arachnoiditis, spinal cord restriction and impairment of blood supply producing myelomalacia, cavitation and neurologic problems. This appears to have been the first medical description of adhesive arachnoiditis. If this something of only historic interest?
Recently the editor has become aware of a number of cases in which epidural injections for the purpose of analgesia were used to assist in childbirth in young and previously healthy women. Following these injections the women developed severe, and in some cases permanent, neurologic problems. Subsequent imaging studies documented thoracic adhesive arachnoiditis. Although these were standard epidural injections the anesthesiologists involved have been accused of producing the problem. From reviewing the MRI studies I am convinced that these situations represented long-standing cases of clinically insignificant Foix-Alajouanine syndrome activated by the epidural injection (probably the included epinephrine) and thus becoming “clinically significant.”
How many anesthesiologists know about the Foix-Alajouanine syndrome, adhesive arachnoiditis, or the dangers of injecting foreign body substances into the subarachnoid space? This knowledge is truly a “rare” situation.
Appreciation to the New Zealand Ministry of Health
The literature review by Day and associates is a really important contribution, it is also something, which should have been done a long time ago by Health agencies in the United States or England. New Zealand clearly has less resource available than these world neighbors. The fact that concerned citizens were able to reach the responsive ears of government to commission a valuable first-step technology assessment is exemplary and worthy of acknowledgement. The editor’s highest compliments and personal appreciation are extended to all involved.
by Sarah Smith, M.D.
COMMENTARY ON THE 2001 NZHTA REPORT ON ARACHNOIDITIS
As a campaigner who has been trying to raise awareness of adhesive arachnoiditis in both the medical and public arenas, I welcome this report as an important step forward in helping to further this aim. Being the first of its kind, the report is groundbreaking and I hope that it will occasion further such work in other countries. Arachnoiditis is, after all, a global issue.
One of the chief aspects of adhesive arachnoiditis has been appropriately highlighted by the report: a continuing dearth of cohesive medical literature, in particular with regard to the prognosis of this incurable condition. As the report remarks, it has a heavy “reliance on the work of several key authors” which denotes the very limited number of experts within the field. The authors go on to suggest that this very problem may be a hindrance to future work on this condition.
Peter Day and his associates have also identified and elaborated on a number of other highly important issues which include:
1) difficulties in terminology
2) lack of correlation between medical evidence and symptomatology
3) statistics of incidence and prevalence
These three issues have already been discussed at length by experts such as Dr. Charles Burton, who has recently reiterated them in his Supplementary Observations on this report. (available here)
He believes that around 5% of people with adhesive arachnoiditis have a “clinically significant” condition. He ascribes the low prevalence of clinically significant adhesive arachnoiditis to the ability of the nervous system to compensate for insults, provided that these are not too frequent or numerous. However, he goes on to make a further, vital point: “This also means that many who have the scarring and are asymptomatic exist in a precarious state”; he notes that the “huge reservoir of existing cases” involves individuals who “appear normal” but live essentially with the sword of Damocles hanging over them, albeit they remain unaware of this. Of course, medical professionals also remain ignorant of the time bomb, and in the course of instituting further invasive intervention, set the clock ticking.
Until or unless clinicians develop an index of suspicion of adhesive arachnoiditis, based upon a patient’s history of risk factor(s) for the condition, then those at risk are unlikely to be identified : prevention of an irrevocable situation will remain impossible and furthermore, recognition of cases of the condition will continue to be patchy.
This lack of awareness is of prime importance in the continuing battle against arachnoiditis. The Day report does clarify for us the lamentable lack of progress in this matter. Both the NZHTA report and Dr. Burton’s observations thereon note that statistics in incidence and prevalence of adhesive arachnoiditis remain lacking. This is something of a vicious circle: lack of awareness and perception by the medical profession of adhesive arachnoiditis as rare inevitably precludes routine consideration of the condition in differential diagnosis and the resultant low rate of diagnosis gives a feedback loop of low statistics that ‘confirm’ the notion of the condition’s rarity. In addition, it is not considered to be a sufficiently common risk to warrant mandatory warning during discussion of consent for invasive spinal procedures. As the Day report comments, quite how we are to resolve this conundrum is far from clear at this stage.
On more specific issues: the NZHTA report notes that “well designed clinical trials in the efficacy and safety of steroidinjections and infusions are needed to better determine the benefits and hazards of their therapeutic role.” Whilst in New Zealand, perispinal use of preparations such as Depo-Medrol is viewed as ‘experimental’ and thus carries a statutory requirement of specific informed consent by the patient, no such approach is taken in countries such as the UK and USA. As the report details, work and literature reviews to date call into question the advisability of continued use of epidural steroid injections; inparticular, the 2001 Cochrane Review should be viewed as a clarion call to reassess this practice. Personally, I feel that the body of literature currently available carries sufficient weight to preclude the necessity for further studies and that bearing in mind that these procedures are now the major cause of new cases of adhesive arachnoiditis, use of epidural steroid injections should be discontinued forthwith. I am petitioning theUK Department of Health on this matter and intend to approach the National Institute of Clinical Excellence (NICE) about it.
I am disappointed that there is an absence of commentary in the report on issues such as intraspinal chemotherapy, which is known to be associated with cases of adhesive arachnoiditis. Thus, children who achieve remission from their leukaemia are now living to face the interminable damage wrought by the toxic chemicals on their arachnoid membrane. I feel that the report has failed to address this sort of issue because it was limited to literature search which excluded “articles where arachnoiditis was an incidental finding not mentioned in the title or abstract.” This may have limited the scope of the report somewhat, but I fully appreciate that arachnoiditis is a cross-discipline and thus problematic subject and one that requires sourcing reference material which, by and large, tends to be “descriptive and anecdotal and relate to a small number of cases.”
It is of interest and highly significant to note that the report found patient support groups to be a useful resource and, indeed, commented that whilst “it is not clear how coordinated and systematic research into arachnoiditis will proceed…support groups and clinicians working in the area remain an important impetus to future research.” As we have already seen, there are remarkably few specialist clinicians, so the impetus must devolve, as ever, to thesupport groups. I would like to take this opportunity to applaud the sterling work by the ASAMS group, in particular, Lynne Elmslie, Dr. Wendy Anderson and Denise Sumner. Despite their own illnesses, these courageous individuals have worked incredibly hard to raise awareness and were undoubtedly instrumental in bringing the need for the report to the attention of the authorities.
However, I think it reflects poorly on the medical profession that there is not a concomitant level of effort within the medical community as a whole.
I sincerely hope that Governments in other countries may follow the New Zealand Ministry of Health example and commission similar report. As Dr. Burton remarks: “The literature review…should have been done a long time ago by Health agencies in the United States or England.”
I would very much like to see my home country lead the way in this and follow up on this report with further work, in particular working towards:
1) raised awareness and thus:
2) proactive management for arachnoiditis sufferers and
3) prevention of preventable cases.
In particular, I would suggest that iatrogenic adhesive arachnoiditis be made a notifiable condition with its own specific ID code. (I note in New Zealand there is some difficulty with statistics due to use of meningitis codes).
Sadly, adhesive arachnoiditis is not a historical condition, but one which persists, with new cases arising. Bearing in mind that these are predominantly iatrogenic in origin, it behoves the medical profession to avoid complacency and strive towards upholding the closing recommendation in the NZHTA report:
“Prevention will be an important aspect of health strategies to address this condition given the recognised etiology…particularly the prevention of post-operative and post-injection complications.”
As Napoleon remarked in 1820, “I do not want two diseases – one nature-made, one doctor-made.”
Dr. Sarah Smith (nee Andreae-Jones) MB BS Patron of the Arachnoiditis Trust, UK and ASAMS March 2002.