B.P.A.A. News Letter - Spring 1996
Volume 4, Number 2

By Margaret A. Hill

ARACHNOIDITIS
WHAT IS ARACHNOIDITIS? WHAT ARE THE SYMPTOMS?
WHAT CAUSES IT?

Arachnoiditis is a progressive neurological inflammatory disease that may affect several different systems in the body. The immune system weakens as the disease progresses and it is thought that in some people the disorder begins because the immune system was weak to begin with. Arachnoiditis is very similar to multiple sclerosis (MS). This is probably due to the fact that as fibrous tissues surround the spinal cord, the white matter undergo extensive demyelination (8) and demyelination is the reason for the development of MS.

The symptoms of both MS and arachnoiditis are nearly identical. In fact, MS is often suspected by doctors—especially general practitioners, rheumatologists and doctors of internal medicine—when they first see a patient with arachnoiditis. The symptoms of the disorder are also similar to Lupus. Doctors should not be faulted for errors in diagnosis because not enough information about arachnoiditis is available to nonsurgical practitioners. Articles about arachnoiditis are rarely published in the professional journals these doctors access.

Arachnoiditis has been called various names, including chemical arachnoiditis, sclerosing spinal pachy-meningitis, meningeal inflammation, granulomatous meningitis, aseptic meningitis, lumbar radiculopathy, and adhesive arachnoiditis. Many people are told that they have failed back surgery syndrome (FBSS) because they have had multiple surgeries in an attempt to alleviate the pain. (But please keep in mind that a diagnosis of FBSS does not necessary mean that someone has arachnoiditis.) According to the ICD-9-CM Codes, 9 the diagnostic code of arachnoiditis is 322.9. The code for lumbar radiculopathy is 724.4.

A report by Dr. Don M. Long, (10) a Johns Hopkins neurosurgeon, lists the features suggestive of arachnoiditis as: partial or complete block, narrowing of the subarachnoid space, obliteration of nerve root sleeves, an apparent thickening of the nerve roots, irregular distribution of contrast medium, loculation of retained Pantopaque (Myodil) or newly injected material, formation of cysts, and mobility of oil-based contrast agents. Spinal cord atrophy may occur with lumbar arachnoiditis.

 

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Dr. Charles V. Burton, medical director of the Institute for Low Back Care in Minneapolis, Minnesota (and the honorary chairman of BPAA), describes arachnoiditis in three stages: (4) arachnoidal adhesions, adhesive arachnoiditis, and calcified arachnoiditis. The spectrum of arachnoiditis is shown in figure 1 on page 4 (Figure 1-a shows the normal anatomy.) The initial phase, Figure 1-b, reflects swelling and hyperemia (increased flow of blood to the area) of the nerve roots of the cauda equina as a response to infection, irritation, or the presence of a foreign body substance. If this response is greater, the subarachnoid space is often obliterated by the swollen nerve roots such that, if the dura is surgically opened, its contents may herniate through the dural opening. Fibroblast proliferation is initiated by the inflammatory response, and collagen deposition occurs.

In the next phase, Figure 1-c, the process has become less acute, and the nerve swelling and hyperemia have subsided. This phase is characterized by more extensive collagen deposition (hence the term “adhesive”), which serves to attach the nerves to each other as well as to the meninges. It is probably at this point that autoimmune response factors determine whether the process abates or progresses in intensity and scope.

Those rare cases in which fulminating arachnoiditis travels up the neuraxis to involve the brain stem and brain (capable then of producing hydrocephalus and death) most likely represent extreme examples of the autoimmune reaction to foreign body insult.

Figure 1-d shows nerves that have become atrophic, more adherent, and enmeshed in dense scar tissue. The nerves may become so completely covered with collagen that an observer, on opening the dura, is presented with what typically appears to be an empty tube devoid of identifiable nerve fibers. Because they have become an integral part of the dural membrane, it is possible to section these spinal nerves by a single lumbar puncture or by surgically opening the dural sac. In advanced lumbosacral adhesive arachnoiditis, residual iophendylate is the only foreign body commonly seen to cause this type of reaction.

The re-absorption of free postmyelographic residual iophendylate by the body is estimated to be about 1 ml per year, but after it becomes encysted, it may remain present and unchanged for many years. Diagnostic radiography, imaging studies, and operative visualization have also demonstrated that it is not unusual for cicatrix of adhesive arachnoiditis to completely block the spinal canal in a focal manner. (4)

According to Burton, in a very small percentage of patients, the dense scar tissue of adhesive arachnoiditis may progressively calcify. This appears to be a very slow change, and it is sometimes seen in association with a progressive sacral nerve root syndrome that produces bowel and bladder impairment.

It is estimated that at least 1 million people world wide have arachnoiditis, and approximately 412,500 of those people reside in the United States. (4) It is believed by many medical professionals that the number of people with arachnoiditis is much higher, but too many physicians are still saying clinically significant adhesive arachnoiditis is

 

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rare or a made-up disease. Unfortunately, there has been no in-depth research done in recent years to find the true incidence of the disease.

About 25% of the estimated 200,000-400,000 patients undergoing operations for lumbar disc disease in the United States each year are not improved or are made worse by surgery, so it is reasonable to calculate that, over the last 50 years in the United States, approximately 412,000 cases of clinically significant lumbosacral adhesive arachnoiditis have been produced. (4) In 1980, William H. Strain stated that “millions” of iophendylate myelograms had been performed since 1942, the year that he developed it. Even he stated that reactions from the use of his contrast agent were well known. He reported that arachnoiditis may exist in 10-15% of patients examined by myelography, and the sensitive arachnoid tissue is further irritated by the contrast agent (16)

Unfortunately, there has never been a formal investigation done in regard to the use of iophendylate in humans. The dye was introduced only in regard to the diagnosis of spinal tumors (delineation of the level of spinal block). How this agent came to be used for full-column myelography of discogenic disease is simply not to be found in the literature. (4)

Despite the fact that this oil based contrast was identified as being casually related to the production of arachnoiditis from the time of its introduction, its use in the United States has never been restricted by industry, government, or the medical profession. In Sweden and a number of other countries throughout the world (including the former Soviet Union), the situation has been quite different: on the basis of animal studies or clinical observation, iophendylate was removed from clinical practice in many countries as early as 1948.(4)

 

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Patients who had iophendylate myelograms 20 years before may suddenly develop symptoms. People who thought their back problems had resolved two decades before may not relate the sudden development of severe spinal pain and other problems to a procedure performed so long ago.

BPAA has been receiving calls from women who relate the development of their back pain to spinal anesthesia administered for childbirth. Some of them knew from the very beginning that this was the cause of their back pain. One women recalls her doctor telling her that he had accidentally inserted the needle in the “wrong” place and that he had to redo the procedure. She suffered some paralysis immediately and had such severe headaches that she had to stay in the hospital for several weeks while her baby went home. She was told that she would recover fully, but this did not happen.

One member developed back pain when she bent over to make her bed. Her doctor tried traditional treatment methods, including bed rest, ice and heat. But they didn’t seem to help her pain. Physical therapy didn’t help either. She was referred to an anesthesiologist for treatment. This doctor felt that she might benefit from epidural steroid injections. He explained that they seemed to help some people while others gleamed no benefit at all. The member felt it was worth a try, after all the worse that could happen was that the injections wouldn’t work. The doctor made arrangements for her to receive her first injection at a local medical centre. Unfortunately, he did not use fluoroscopy which would have helped him to guide the needle. The member was later diagnosed with clinically significant adhesive arachnoiditis.

Fortunately substances injected into the epidural space do not cause harm. But there is a down side. According to a report by Renfrew et al, “Failure to gain the epidural space is inversely proportional to experience, but even experienced operators have been shown to gain the epidural space reliably in only 62% of patients. (14) Lumbar injections may fail to gain the epidural space in 17% to 30% of patients. (4)

Burton finds it unfortunate that intrathecal steroid methylprednisolone acetate (Depo-Medrol/Depo-Medrone) prepared as a suspension was injected with myelography contrast media in an attempt to reduce the risk of arachnoiditis, when in fact the opposite result was produced.

Over the years, studies have shown that a mixture of blood and Pantopaque (Myodil) produce severe arachnoidal inflammatory reactions. (4)

Earlier in the century, the most common causes of the development of arachnoiditis were Tuberculosis (TB) and syphilis. Tuberculosis arachnoiditis was made worse with the introduction of intrathecal therapy. (16) The advent of antibiotics reduced the risk of these infections, but they are making a comeback. Since doctors have been prescribing antibiotics for all of the various problems that plague their patients, TB and syphilis are more antibiotic resistant. If patients with these diseases develop back problems, doctors may not realize immediately that they have arachnoiditis

 

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In the late 1930’s and early 1940’s the myelographic agent Thorotrast, a thorium dioxide solution containing radioactive thorium, was used. This dye produced inflammatory arachnoidal reactions and progressive nerve root damage, leading to cauda equina syndrome and malignant tumors of the nervous system. (11).

Trauma may cause arachnoiditis, and blood in the cerebrospinal fluid, although rare, may produce the disease. (10).

Long reported the advent of myelography and the rapid increase in surgery for diseases of the spine were associated with increasing recognition of arachnoiditis as a complication appearing in the clinical course of rare patients with structural spinal disease.

According to reports, all foreign materials injected into the sub-arachnoid space, including normal saline, produce an inflammatory reaction. All contrast agents that have been and are now employed cause a meningeal inflammatory reaction. However Long questions how significant these reactions are from a clinical standpoint, and whether contrast agent-induced reactions are related to the clinical syndrome of chronic adhesive arachnoiditis. (10)

In 1978, eighty cases of arachnoiditis were identified from a series of 7,600 myelograms. Twenty eight hundred of patients were operated on, and the frequency of arachnoiditis was 1%. Earlier in the century, the disease progressed to paraplegia, but the 1978 report presented a new group of patients who developed arachnoiditis as a complication of a combination of poorly done or multiple myelograms and extensive surgery. The new syndrome commonly occurred in the lumbar region and it did not appear to have the progressive nature described previously. (16).

A 1985 report by Garancis and Haughton studied the development of arachnoiditis using water-soluble contrast.

At first there were no changes and the iodine disappeared within 24 hours. After 8 days, fibrous and perineural adhesions occurred. This was followed by fibrosis of the meninges and dense perineural fibrosis. Progression was evident during the whole twelve weeks of the study .(5)

Don M. Long, a neurosurgeon at the Johns Hopkins School of Medicine in Baltimore, Maryland, has put great effort into studying and compiling information on arachnoiditis. (10) The following is information that has been excerpted from his report. Long’s report ends right before the subtitle “Symptoms of Arachnoiditis” later in this report.

From 1967 to 1992, long saw a total of 321 patients with arachnoiditis. There were 215 men and 106 women. They ranged in age from 26-72 years old, with the median age being 52 years. In 1967 to 1982, the average number of myelograms was 9 and the range was 1-19. The average number of surgical procedures was 6.5 and the range 0-14. From 1982-1992, the number of myelograms were markedly reduced from 9 to 3 and the number of surgical procedures, from 6.5 to 2.5. There was a small additional group of eight patients with arachnoiditis involving the spinal cord.

 

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Long did not find a consistent clinical pattern of symptoms. Instead he found the following: 302 patients (94%) had back pain that was aggravated by activity; 261 patients (81%) had leg pain; 40% had radicular pain and 41% had diffuse and apparently nonradicluar pain; 45 patients (14%) had major bowel and bladder dysfunction. The arachnoiditis was progressive in six patients (1.8%). Range of trunk motion was impaired in 292 patients (91%); forward and backward bending produced pain in all patients; chronic muscle contractions (usually secondary to surgery) were present in 301 patients (94%);motor loss in 237 (74%) and demonstrable sensory loss in 261 (81%); reflex changes were present in 308 (96%); straight leg raising was positive in 195 (61%).

Functional status was assessed by questioning the patients: 269 patients (84%) walked alone; 48 (15%) walked with aids; 1.2% were wheelchair bound; 295 (92%) complained of claudication with limitation of the distance they could walk; 36 (11%) were working full-time; 118 (37%) were working part-time or with limitations;

167 were retired secondary to back complaints (but could not be directly attributed to arachnoiditis in most); 82% drove without limitation; 12% were able to drive an automobile with aids; only 18 (5.6%) were unable to drive.

Sexual function was severely impaired. Only 8% described no abnormalities; 147 (45%) were able to perform sexually with impairment; 151 (47%) described severe impairment of sexual function.

Forty-five patients (14%) were taking no drugs of any kind and 64 (19%) were using non-narcotic analgesics (often in large quantities).

One patient sustained severe kidney damage from Propoxyphene (DarvonÒ) and two patients were found to have significant hepatic damage from the use of non-steroidal anti-inflammatory drugs; 167 (52%) used narcotics on a daily basis; 45 (14%) showed evidence of abuse and drug seeking behavior; 260 (81%) used a psychotropic drug, diazepam being the most common.

In the patients with spinal cord involvement, all had blocks on myelography; all had progressive neurological deficits; 2 with cervical arachnoiditis were quadriplegic with increasing respiratory deficiencies.

Patients with thoracic cord involvement were all becoming progressively paraplegic.

Therapy: Of the original 158 patients, 23 underwent microlysis of the adhesions, and 14, direct surgery upon an extradural defect without therapy of the arachnoiditis; 3 were given repetitive intrathecal steroids and in one patient, oral steroids were given as well; 32 patients were treated with spinal cord stimulation; 131 underwent in patient pain rehabilitation to maximize function while reducing drug utilization

In the second group of 163 patients, 12 underwent microlysis of the adhesions; 47 were operated on directly for other extradural problems; 42 were referred for spinal cord stimulation; one had an unsuccessful trial of brain stimulation; one was treated with intrathecal steroid injections; one had an intrathecal morphine pump; 72 were referred for inpatient pain rehabilitation.

 

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Intrathecal steroids were successful in the few patients treated. Otherwise, intractable pain was improved for at least 6 months. One patient developed loss of bowel and bladder control following one injection of Depo-Medrol, which was the only steroid used. Recovery was satisfactory, but residuals remained. There were no other side effects and all patients were able to discontinue therapy while maintaining satisfactory pain control.

Spinal cord stimulation (SCS) was most effective treatment and now is our (Long et al) standard therapy used. An immediate success rate of <70% was achieved; intermediate success was <50%; long term success was <30% and has been reported in detail elsewhere. None of the patients worsened. When pain is a major problem, SCS is favored

Direct operation with microlysis of the adhesions was used for the complaint of pain alone in the first series of patients and had a 55% success rate at 5 years. However, 13% had developed significant increases in bowel and bladder dysfunction. Major motor or sensory deficits following surgery was rare.

In the second series of patients, the criteria for surgical microlysis of the adhesions was changed. Surgery was reserved for those with a clearly progressive neurological syndrome. During this period, only three patients had progressed to paraplegia, but a small number off additional patients had evidence of progression of neurological syndrome. Twelve patients were operated on. Of the 12, five had satisfactory pain control, but none achieved lasting pain relief and all have received additional therapy for pain. One patient had complete resolution of a rapidly progressive cauda equina syndrome. The patient remained without neurological complaints for 5 years. In nine of the patients, the neurological deficit either stabilized or improved; in four patients, the progression stopped; in five, substantial improvement in strength occurred.

Sensory complaints did not improve and none of these nine patients had substantial change in bowel and bladder function. One patient initially stabilized and remained so for less than a year. The patient subsequently complained of progressive loss of function, but no change in neurological examination has occurred. In the final patient, stabilization occurred for 3 years and then demonstrable loss of neurological function resumed. That patient has become more severely paraparetic and now uses a wheelchair or transport vehicle for the disabled.

Based upon experience, Long feels that the majority of patients with arachnoiditis are not candidates for direct operation. He based this upon 35 patients evaluated for more than 20 years. Long-term success of lysis of adhesions for pain control is not high. Satisfactory pain control occurs in less than 50% initially and this percentage reduces with time. When the problem is loss of neurological function, which is progressive, re-operation has an excellent chance of stopping the progression and occasionally improving function. A fixed neurological deficit is unlikely to improve. When bowel and bladder deficiencies are the major problem, improvement is even more unlikely. Spinal stenosis should be corrected since the diagnosis of arachnoiditis is uncertain in the face of stenosis. Other extradural abnormalities can be corrected if appropriate indications exist. The presence of

 

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arachnoiditis does not prelude a good result if the indications for the procedure are present.

Surgery: Long reserves surgery for the small number of patients with progressive neurological deficits or a fixed deficit that is incapacitating, or for those few patients with believable intractable pain for whom nothing else seems to work.

He uses CT myelogram because he feels MRI does not sufficiently show the normal areas above and below the abnormality and the full extent of and location of the arachnoiditis. Scars that are posteriorly placed are most favorable and napkin ring lesions are next. When the entire canal is filled with scar, the surgery is likely to be unsuccessful, and the presence of calcifications means surgery is going to be difficult even for this extremely demanding surgery. It is the most tedious, time consuming, and meticulous dissection that neurosurgeons do. It requires the dissection of individual nerves from dense scar under high magnification. The roots are easily injured and only the most careful dissection will allow them to be extricated from the scar. Patients are likely to worsen during the process.

Long feels that before a surgeon undertakes this kind of surgery, the patient was to be told of the potential results, risks, and magnitude of the procedure. Despite all of this, the doctor feels the operation is gratifying and will often preserve or restore function in patients who are losing neurological capabilities.

Arachnoiditis calcifications is a particularly difficult problem. If a calcified mass is posteriorly placed, it can be dissected out with ease. But when calcifications are scattered, the surgical challenge is even greater than usual. Typically, the nerves enter and exit the calcified masses. To obtain adequate decompression, it is necessary to break the calcium, dissect the calcified masses away from the nerves, and remove them leaving the nerves intact. Sometimes it will not be possible to free the roots from calcified scar, so the surgery should be stopped to prevent undue risk of increasing the patients’ neurological deficits.

SYMPTOMS OF ARACHNOIDITIS:

There is no cure for arachnoiditis, but many of the symptoms can be treated. There are some symptoms that are more common than others. Below is a list of both the common (*) and uncommon symptoms. They are:

*Back Pain—A feeling of severe pressure in the lumbar spine, especially after standing or walking. The burning pain is difficult to treat and usually radiates into one or both legs.

*Foot Pain—There is a feeling as if one us walking on rocks or broken glass. There may be severe burning in the feet and ankles. Some people use ice to “cool” these areas, but the skin must be protected so a cloth or towel should be placed between the skin and ice pack.

 

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The proper shoe is important. An orthopedist who specializes in foot pain will be able to assist you in choosing the proper shoe. Since heel pain is very common, arachnoiditis sufferers frequently wear thick-soled house shoes (slippers). There are a variety of inserts that can be purchased over the counter and placed in shoes for more comfort.

*Esophageal spasms—This is a problem that can make swallowing very difficult. It is more common in people who have problems at the thoracic level. At first, the painful spasms may be mistaken for a heart attack. (It is always best to have chest pains checked out since it might mean you really are having a heart attack.) Anti-depressants may help to keep esophageal spasms to a minimum. Other medications can be prescribed to control the spasms. Your doctor may order tests that identify or rule out the cause of the spasms.

*Eye Problems—May include *visual disturbances, *double vision, *sensitivity to light, and rarely blindness. Blindness may occur if the contrast was injected into or allowed to enter the cistern of the brain. There has been some concern that arachnoiditis may cause glaucoma, but there is no evidence to support this belief.

In animal studies, (19) dogs administered Pantopaque developed eye problems. Another dog’s left eye appeared reddened. Although the dogs developed redness and mucous discharge in the eyes, this was not even mentioned in the final analysis of the study. This might have helped us to understand why so many people experience eye problems. Why does blindness occur in patients who have no contrast medium in the cistern of the brain?.

*Lungs—In animal studies, (19) the dogs had respiratory problems. They had a small number of red to blue nodules approximately 2 mm in diameter in the lungs. One dog had scattered red nodules in parenchyma.

*Skin rashes and hives—Rashes may appear as red splotches, spots or hives on one or more areas of the body, but they primarily appear on the arms or trunk. In very rare cases, a pustular rash may appear on hands and feet, but whether or nor pustulosis or other rashes are related to arachnoiditis is unknown at the present time.

*Weakness—May affect one or both legs. Bilateral weakness is very common in people who have had arachnoiditis for a long period of time. When problems are higher up in the spine there may be weakness in the arms as well.

*Parasthesia (numbness or tingling)—This feeling may affect the back, legs, feet, arms and fingers.

*Paralysis—Arachnoiditis may progress to this stage, but at the present time this is very rare. Most cases of paralysis are the result of errors occurring during surgery or the myelographic procedure or when other invasive procedures are performed. The paralysis may be complete or incomplete.

*Bladder dysfunction and kidney problems (12)—Bladder dysfunction usually occurs when there is loss of sphincter muscle function. Bladder spasms are a common problems. It begins as urgency and frequency and proceeds to incontinence. If the disease progresses, there may be retention of urine. Urine can back up into the kidneys and cause damage, including atrophy of one or more kidneys and loss of function.

 

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Anti-spasmodics such as oxybutynin chloride (DitropanÒ) or propantheline bromide may be prescribed to control the spasms. Anti-depressants may help prevent involuntary loss of urine. In more severe cases, intermittent catheterisation may have to be performed. Sometimes a fixed catheterisation device is more desirable.

Bowel dysfunction—Bowel problems are more common and more serious in patients with a progressive sacral nerve root syndrome. Problems begin with loss of function of the sphincter muscle. Constipation is common and there may be problems with constipation for years before the painful spasms begin. Spasms occur in the lower part of the abdomen, they may also occur higher up in the intestine. In the final stage of bowel dysfunction, there is an inability to have a bowel movement. (impaction). Problems may progress until there is complete loss of feeling in the rectum. When impaction and loss of feeling occurs, patients must begin a bowel maintenance program.

Frequently, drinking a glass of prune juice at bedtime will result in successful elimination of the feces. It is also important to drink several glasses of water and other non-carbonated drinks. In more difficult cases, the patient may need to wear a latex glove to manually remove the feces. (Contact your physician or BPAA before doing this. Improper procedures can damage the rectum.) Laxatives such as DulcolaxÒ or glycerine suppositories may be needed. Water enemas should not be used on a daily basis and only in rare instances when feces cannot be removed. Bowel dysfunction can be critical in some cases. The spasms can last hours at a time and they can become severe enough to cause extreme weight loss. Severe spasms can cause nausea and vomiting and a feeling of intense warmth. It is important to cool the head and body with cool cloths. If the spasms persist, emergency care may be required. (15).

Sexual dysfunction—Pain in the back and legs may make it difficult to engage in sex. Pain in the vagina or testicles may also interfere with sexual function. The inability to maintain an erection is common in men with clinically significant arachnoiditis. There are now many devices, procedures and medications prescribed for impotence that may make sexual relations possible. A urologist who specializes in neurogenic bladder problems should be able to help or refer you to someone who can.

*Hypersensitivity of the skin and muscles—Skin and muscles may be extremely sensitive to touch. Beginning a program of gentle massage may help to relax the muscles. Anti-depressants may help both the muscle spasms and skin sensitivity. Relaxation techniques and self-hypnotism may be beneficial as well.

Spasticity—Loss of muscle control (jerking and uncontrollable movements) in the legs and arms usually occur in the later stages of the disease. Some patients may require anti-spasmodic medications such as Baclofen.

Allergy to shellfish and iodized products and substances—Retained iophendylate contains iodine. Some people experience problems after eating shellfish, iodized salt, or products containing a large amount of iodized salt (listed as sodium in the ingredients). Symptoms may include diarrhea, vomiting, headaches and sometimes fever.

 

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There may also be a reaction to studies using other iodized contrast agents. Precautions can be taken by the physician if you must have these tests. If you have had a reaction from previous studies, it is imperative that you tell your doctor. The doctor may want to know if you have retained iodized dye in your back, especially if you have had a reaction to iodized foods and products. Anti-histamines may help to protect you, but a qualified doctor will know more about preventing reactions. Sometimes other methods of diagnosis are used when the practitioner is aware that there is a problem with iodized dyes.

In animal studies, urine contained elevated levels of iodine. (15).

Allergy to shellfish and iodized products is a fairly new finding. Until research is performed, we will not know the true number of people with this problem.

*Thyroid function tests—If your doctor suspects a thyroid problem, it may be necessary to treat your symptoms. The Package insert out of PantopaqueÒ (iophendylate) states, “Diagnostic test of thyroid function involving measurements of iodine may be invalidated for several years following the intrathecal injection of Pantopaque.”

Syringomyelia—How syringomyelia occurs and its treatment methods are still controversial. Researchers are trying to find answers to those questions so the proper treatment can be administered.

There are two schools of thought on why syringomyelia occurs in people with arachnoiditis. In the first, it is thought that the scarring of the arachnoid membranes causes changes in the vascular supply, producing areas of cord ischemia and subsequent softening with cavity formation. In the second, meningeal scarring may alter CSF flow with consequent increase in pressure within the central canal and expansion of this structure. Symptoms may include one or more of the following: motor weakness, numbness, sensory deficits, clumsiness, spasticity, muscle atrophy, and / or paralysis. The symptoms of syringomyelia may be delayed as long as 13 to 17 years. Surgical intervention may be needed to decompress an enlarged syrinx. Subarachnoid shunting is another method of drainage. In some instances, failure may cause further neurological deterioration due to shunt blockage or further ischemia (reduced blood supply) or gliosis (growth of neuroglial tissue). (2)

A syrinx can be either “low-pressure” or “high pressure.” Reports indicate that a high-pressure syrinx has better postsurgical results ( 1)

Basal arachnoiditis—Basal arachnoiditis is the result of scarring of the basal meninges with subsequent syrinx formation. (3). In one reported case, (18) a man had weakness and wasting of the right side of his tongue and had pain in the back of his head. He had progressive stiffness of the lower left extremity with difficulty in walking. These symptoms evolved over the next few years. There was no headache, dizziness or vomiting.

When the doctor first saw the man in 1987, general examination was unremarkable. Neurologic examination showed normal cognitive functions. His gaze was mildly impaired in the right eye. Tests, including blood work-up, were performed. Plain x-rays of the spine showed only a few droplets of iophendylate in the thoracic region. Cranial computed tomography showed multiple droplets of iophendylate in the subarachnoid space of the brain. His neurological signs were confined to the posterior fossa. Further

 

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studies showed that the man had adhesive basal arachnoiditis. He was treated with a course of oral steroids with no response. Over the next few months his neurological status remained stable except for the evolution of a right sixth nerve palsy. He continued to work and remained ambulatory and otherwise functional in his daily activities.

This gentleman’s problems occurred when iophendylate injected via lumbar puncture was allowed to ascend into the cervical region during myelography. Unfortunately, the contrast was not removed after completion of the procedure. Symptoms of basal arachnoiditis developed two years after the procedure was performed.

Weight loss—Most patients tend to gain weight, especially in the beginning when the pain becomes severe and life-styles become more sedentary. But weight loss and the inability to regain it occurs in many people with arachnoiditis. In most cases, weight loss occurs in later stages of the disease. Weight loss is a problem in animals administered Pantopaque. (15)*

Depression—Due to the severity of pain and other problems experienced by people with arachnoiditis, depression is common. They feel degraded by medical professionals who usually do not understand the pain they are suffering. In the Journal of the Royal Society of Medicine (Volume 83, 1990), IHJ Bourne stated, “In many instances doctors, relatives, and friends fail to realize that the pain can be as bad as terminal cancer without the prospect of death to end the suffering.” So it is no wonder that arachnoiditis sufferers become depressed when they are called “drug addicts” and placed in detoxification centers that take one of their only forms of relief—narcotics medications—away from them. They are frequently labeled as drug seekers when they ask for medications to relieve the pain. They don’t mind being monitored by compassionate physicians who are willing to prescribe drugs that help their pain. People with arachnoiditis don’t want to get “high,” they only want to feel as normal as possible again. Until researchers find non-narcotics that work, narcotics are the only thing they have to rely on.

Animals administered Pantopaque. (15) Even when the depression subsided, it came back again. People with arachnoiditis experience depression every time they have a massive flair-up or when they see doctors who know virtually nothing about clinically significant arachnoiditis, and they feel degraded by doctors who can’t comprehend why they just can’t “get on with their lives.”

TREATMENTS

The standard treatment of clinically significant adhesive arachnoiditis is the implanted spinal cord stimulator. In cases where the pain is especially severe, implanted depth brain stimulation may be used. (4, 10) More and more people with arachnoiditis now have the intrathecal infusion pump. Morphine, Dilaudid, Baclofen or another medication is used in the pump to control pain and other problems. Before the pump is implanted, the patient is tested to see if it will work and if the drug can be tolerated. Another incision is made in the spine and a tube is inserted. A portable pump containing morphine or another drug is attached to the tubing. The patient is then monitored for several days. If the pain subsides, the pump is surgically implanted in the abdomen. The downside to the pump is that once

 

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it is implanted, it may take a few months of trial and error before a satisfactory dose of a drug is found. And not everyone can tolerate the drugs used in the pump.

Most people who have call BPAA seem pretty satisfied with it. Only two of them had an adverse reaction and one of those was quite severe.

Oral analgesics, including non-steroidal anti-inflammatory drugs are used to control the symptoms, but they are often discontinued because they cause gastric upset. In some cases there is liver damage. Oral steroids are often prescribed to help control the inflammation in people who can tolerate them. Unfortunately, the side effects of steroids can be devastating. They may cause osteoporosis, diabetes, depression, and sometimes death. (NEVER stop taking oral steroids suddenly or without your doctor’s permission. It can be very dangerous to do so.) Acetaminophen and drugs containing acetaminophen help many people but they must be monitored by a doctor. Large doses can cause kidney and liver damage, so it is imperative to have periodic blood tests. Muscle relaxants are often prescribed for spasms in the back. Narcotic analgesics may benefit arachnoiditis sufferers with intractable pain, but most doctors reserve them for patients with intractable pain.

Alternative treatments are beneficial as well. Relaxation techniques, guided imagery, biofeedback and self-hypnosis may help when pain becomes intolerable. Trigger point injections may also help. This method of treatment can be taught to a partner, friend or family member, who then administers the daily injections. Some people have found acupuncture helps their pain. Alternative medicine is becoming more acceptable in modern medicine.

In India, Dr. M Gourie-Devi used intrathecal injections of hyaluronidase to treat non-infective arachnoiditis after there were favorable results in the treatment of tuberculous spinal arachnoiditis. The following results are only preliminary, but promising. In one group of 15 patients administered the enzyme, eleven of them had satisfactory recovery and three had mild but significant improvement, and in one no change occurred. According to Gourie-Devi, “Except for mild pain during injection, worsening of spinal cord or bladder functions and other serious toxic effects like allergic reactions or convulsions were not observed in our study.” (7)

BPAA has written to Dr. Gourie-Devi to see if she has found continued success with hyaluronidase in the treatment of arachnoiditis patients. You will receive more information when we receive it.

In Argentina, Dr. Luis Gegalian, (5) a neurosurgeon, also began using hyaluronidase to treat arachnoiditis.

In 1976. A 73 year old man with paraparesis had a myelogram that revealed a block to the flow of contrast medium at T-10. Surgery revealed adhesive arachnoiditis and the adhesions were lysed as far as possible. He developed bedsores over the sacrum and both heels. The man also had a large second degree burn on his right leg from contact with a hot stove, which had failed to heal. After surgery, he showed slow functional improvement and he was able to walk up stairs. A year later, paraparesis reappeared and he progressed to paraplegia. He developed muscular atrophy of the lower extremities and there was loss of sensation up to the umbilical area.

 

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In 1977, the man was administered 10 injections of hyaluronidase. He received five injections in the lumbar region and five injections in the cistern at intervals of three days. After the hyaluronidase injections, improvement was dramatic. The atrophy of the lower extremities improved, although full reversal has not returned. The bedsores healed within one month and sensation returned. Muscular strength improved. The man developed spasticity, but this subsided as voluntary movement in the legs increased. Physical therapy has permitted him to walk with a cane.

In a comment added to his report, Dr. Gegalian wrote that he felt hyaluronidase might be a possible application in the treatment of syringomyelic cysts.

A Commonly Asked Question: Should myelography be outlawed? According to Burton, Long and other experts, the procedure still has its place in diagnostic medicine. Although all water-soluble myelographic contrast media may cause arachnoiditis, the newer ones are less toxic than the dyes used in the past.

Myelography is still the most comprehensive method used to diagnose patients who have had spinal fusion using instrumentation, especially if a lesion is suspected. Burton feels myelography should be used only under the most exceptional of circumstances. (4) Researchers are investigating new fusion materials that are MRI-able, but too many spines still contain metals that make this impossible.

A Final Note: It is evident that research on arachnoiditis is sorely needed. The only research on iophendylate (Pantopaque; Myodil; Ethiodan) was performed on animals. People worldwide have contacted BPAA to obtain and share information and to tell me about their experiences. Organizations and people, including doctors from all over the world, are seeking the same information. What is arachnoiditis and what can be done to treat it??

Too many people have been told that arachnoiditis really doesn’t exist or that it is a “made-up” disease. People are being told that nothing can be done to help them. Even more frightening, people are being advised not to have surgery, These are cases when surgery is needed. In one case, a member had an arachnoid cyst that was causing pressure on the brain. The member’s doctor said surgery was imperative, but the member was told by someone to never have surgery. If this advice had been heeded, the member might have died of stroke. In another case, a person with arachnoiditis had shrinking down of the vertebrae around the spinal cord in the thoracic area.

This person had heard that surgery should never be performed.

If surgery had not been performed to open up the area around the spinal cord, quadriplegia would have occurred.

 

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It is important for people with arachnoiditis to find a caring, compassionate and knowledgeable physician to oversee their treatment. It may take a whole team of medical professionals to treat the myriad of medical problems related to the disease, so a gatekeeper (doctor) is needed to monitor treatments. These professionals can ensure that treatments do not overlap or interfere with one another.

You have the right to participate in decisions about your treatment. Let your doctor know what does and doesn’t work for you. Tell your doctor that you want to know the risks and benefits of each treatment you receive. You have the right to be an INFORMED Patient.

As new information becomes available, BPAA will share it with its members. And if you find new information please share it with us. If you know the names of doctors or medical facilities that are familiar with and treat the symptoms of arachnoiditis, please call or send us their names.

A special thank you to Dr Charles V Burton for his dedication and contributions to BPAA. His broad knowledge about arachnoiditis and failed back surgery syndrome (FBSS) has helped numerous people worldwide. We thank him for his generosity in developing the video tape, “Spinal Arachnoiditis: Its Clinical Significance in Regard To Worldwide Spine Care,” and for allowing BPAA to distribute it to raise funds to help the organization financially, and for caring enough to help us educate people about this complicated disorder.

A special thank you goes to Maureen and Bill Evans from the United Kingdom for supervising the video taping of the September 1995 Arachnoiditis Conference presented by Dr. Burton in London, England and for giving it to BPAA to distribute-----Margaret A. Hill

Resources:

1. Asano M, Fujiwara K, Yonenobu K, Hiroshima K. Post-Traumatic Syringomyelia. Spine. 1996; 21(12): 1446-1453.

2. Barnett H. Syringomyelia associated with spinal arachnoiditis. Editors: Barnett H, Foster J, Hudgson P. In Syringomyelia. 1973; 220-44.

3. Brammah TB, Jayson MIV. Syringomyelia as a Complication of Spinal Arachnoiditis. Spine. 1994; 19(22): 2603-05.

4. Burton CV. Lumbosacral Arachnoiditis. In Neurological Surgery. 1996,3; 112: 2483-91.

5. Garancis JC, Haughton VM. Pathogenesis of postmyelographic arachnoiditis. Invest Radiol. 1985; 1: 85-9.

6. Gegalian L. Use of Hyaluronidase in Central Nervous System. Surg Neurol. 1979; 12: 3-5.

7. Gourie-Devi M. Intrathecal Hyaluronidase Treatment of Chronic Spinal Arachnoiditis of Non-infective Etiology. Surg Neurol. 1984;22: 231-4.

8. Hurteau EF, Baird WC, Sinclair E. Arachnoiditis following the use of iodized oil. J Bone Joint Surg. 1954;36A:393-400

9. International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM)

10. Long DM. Chronic Adhesive Spinal Arachnoiditis: Pathogenesis, Prognosis, and Treatment. Neurosurgery Quarterly. 1992, 2;4:296-319.

11. Maltby GL. Progressive thorium dioxide myelography. N England J Med. 1964, 270: 490-96

12. Mostwin J. (James Buchanan Brady Urological Institute, Johns Hopkins Hospital.) Fact Sheet # 3: Urinary Problems and Diseases of the Spine. Back Pain Association of America 1994.

13. National health and Medical Research Council. Epidural use of steroids in the management of back pain. Canberra: Commonwealth of Australia, National Health and Medical Council. 1994.

 

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Resources (cont’d)

14. Renfrew DL, et al. Correct placement of epidural steroid injections: Fluoroscopic guidance and contrast administration. AJNR Am J Neuroradiol 1991; 12: 1003-7

15. Schuster MM. Francis Scott Key Medical Center, Johns Hopkins University, Baltimore, Maryland. Personal Correspondence. 1993.

16. Shaw MD, Russell JA, Grossart KW. The changing pattern of spinal arachnoiditis. J Neurol Neurosurg Psychiatry.1978, 41:97-107.

17. Strain WH. Radiocontrast Agents For Neuroradiology, In Radiocontrast Agents Volume II. 1980, Chapter 9. Page 365-393.

18. Wong CK, WOO E, Yu YL. Iophendylate-induced Basal Arachnoiditis. In Clinical and Experimental Neurology. Page 199-204.

19. 15-Week Intrathecal Toxicity Study - Dogs: Pantopaque I or Pantopaque II, Final Report, Hazelton Laboratories, Inc. February 1969.

(***Before it was published, this article was read and approved by Charles V.Burton, MD., Senior Medical Director of the Institute for Low back and neck Care (ILBNC), Minneapolis, Minnesota. If you have a computer, you can obtain general information on arachnoiditis on the internet by accessing ILBNC’s Web page at: http://www.ilbnc.com).

 

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Addendum; October 1997: 1. On page 22 of this report, I told you that a letter was sent to Dr. M. Gourie-Devi, Bangalore, India, about her use of the enzyme hyaluronidase. In her reply, Dr. (Ms.) Gourie-Devi stated that hyaluronidase has been used in the United States under the brand name of WygaseÒ. This brand has also been used in Korea to treat arachnoiditis. But in both cases, the enzyme was used successfully only in the treatment of tuberculous arachnoiditis.

When I wrote to Dr. (Ms.) Gourie-Devi, I was not aware that WygaseÒ was the brand name for hyaluronidase. A couple of years ago, I asked Dr Burton about using Wygase to treat arachnoiditis.

At that time, he said that it is true that Wygase dissolves scar tissue but unfortunately it will grow back again.

He also said that, so far, no substance has been found to permanently dissolve scar tissue.

2. Many people are endorsing the bill in Congress to outlaw myelograms. If you would like to see the package inserts from water-soluble myelograms, contact BPAA to request copies.

This will allow you to make an INFORMED decision when you are trying to decide if you should encourage your legislators to vote for passage of this bill. –Margaret A. Hill