Transverse Myelitis

It is our great honor to announce that Dr. Joanne Lynn and Dr. Charles Levy will serve on the Transverse Myelitis Association Board of Directors. We look forward to working with Drs. Lynn and Levy and know that their participation in the Association will both greatly enhance our organization and result in invaluable contributions to our members.

Dr. Lynn is an Assistant Professor of Neurology at The Ohio State University. She received her medical degree from The Ohio State College of Medicine and then served residencies in internal medicine and neurology at Stong Memorial Hospital, University of Rochester. She then returned to The Ohio State University for fellowship training in neuromuscular disease. She is currently on the staff of The Ohio State University Multiple Sclerosis Center and has special interests in clinical research on the treatment of MS.

Dr. Levy is an Assistant Professor, Department of Physical Medicine and Rehabilitation at The Ohio State University. Dr. Levy also serves as the Directors of Orthotics and Prosthetics Clinic, Seating and Positioning Clinic, and Stroke and Orthopedic Rehabilitation of the Department of Physical Medicine and Rehabilitation at The Ohio State University. He received his medical degree from The Ohio State College of Medicine. Dr. Levy served his residency in Physical Medicine and Rehabilitation at the Rehabilitation Institute of Chicago, Northwestern University Medical School, Chicago, IL.

Transverse Myelitis: Symptoms, Causes and Diagnosis

Transverse myelitis (TM) is a neurologic syndrome caused by inflammation of the spinal cord. TM is uncommon but not rare. Conservative estimates of incidence per year vary from 1 to 5 per million population (Jeffery, et.al., 1993). The term myelitis is a nonspecific term for inflammation of the spinal cord; transverse refers to involvement across one level of the spinal cord. It occurs in both adults and children. You may also hear the term myelopathy, which is a more general term for any disorder of the spinal cord.

TM symptoms develop rapidly over several hours to several weeks. Approximately 45% of patients worsen maximally within 24 hours (Ibid.). The spinal cord carries motor nerve fibers to the limbs and trunk and sensory fibers from the body back to the brain. Inflammation within the spinal cord interrupts these pathways and causes the common presenting symptoms of TM which include limb weakness, sensory disturbance, bowel and bladder dysfunction, back pain and radicular pain (pain in the distribution of a single spinal nerve).

Almost all patients will develop leg weakness of varying degrees of severity. The arms are involved in a minority of cases and this is dependent upon the level of spinal cord involvement. Sensation is diminished below the level of spinal cord involvement in the majority of patients. Some experience tingling or numbness in the legs. Pain (ascertained as appreciation of pinprick by the neurologist) and temperature sensation are diminished in the majority of patients. Appreciation of vibration (as caused by a tuning fork) and joint position sense may also be decreased or spared. Bladder and bowel sphincter control are disturbed in the majority of patients. Many patients with TM report a tight banding or girdle-like sensation around the trunk and that area may be very sensitive to touch.

Recovery may be absent, partial or complete and generally begins within 1 to 3 months. Significant recovery is unlikely, if no improvement occurs by 3 months (Feldman, et. al., 1981). Most patients with TM show good to fair recovery. TM is generally a monophasic illness (one-time occurrence); however, a small percentage of patients may suffer a recurrence, especially if there is a predisposing underlying illness.

Transverse myelitis may occur in isolation or in the setting of another illness. When it occurs without apparent underlying cause, it is referred to as idiopathic. Idiopathic transverse myelitis is assumed to be a result of abnormal activation of the immune system against the spinal cord. A list of illnesses associated with TM includes:

Parainfectious (occurring at the time of and in association with an acute infection or an episode of infection).

Postvaccinal (rabies, cowpox)

The cause of idiopathic transverse myelitis is unknown, but most evidence supports an autoimmune process. This means that the patient's own immune system is abnormally stimulated to attack the spinal cord and cause inflammation and tissue damage. Examples of autoimmune diseases which are more common include rheumatoid arthritis, in which the immune system attacks the joints, and multiple sclerosis, in which myelin, the insulating material for nerve cells in the brain, is the target of autoimmune attack.

TM often develops in the setting of viral and bacterial infections, especially those which may be associated with a rash (e.g., rubeola, varicella, variola, rubella, influenza, and mumps). Approximately one third of patients with TM report a febrile illness (flu-like illness with fever) in close temporal relationship to the onset of neurologic symptoms. In some cases, there is evidence that there is a direct invasion and injury to the cord by the infectious agent itself (especially poliomyelitis, herpes zoster, and AIDS). A bacterial abscess can also develop around the spinal cord and injure the cord through compression, bacterial invasion and inflammation.

However, experts believe that in many cases infection causes a derangement of the immune system which leads to an indirect autoimmune attack on the spinal cord, rather than a direct attack by the organism. One theory to explain this abnormal activation of the immune system toward human tissue is termed "molecular mimicry." This theory postulates that an infectious agent may share a molecule which resembles or "mimics" a molecule in the spinal cord. When the body mounts an immune response to the invading virus or bacterium, it also responds to the spinal cord molecule with which it shares structural characteristics. This leads to inflammation and injury within the spinal cord.

Vaccination is well known to carry a risk of the development of acute disseminated encephalomyelitis (ADEM) which is an acute inflammation of the brain and spinal cord. This was particularly common with the older antirabies vaccine which was grown in animal spinal cord cultures; the use of the newer antirabies vaccine grown in human tissue culture has almost eradicated this complication. This is also thought to occur as an immune system response.

Transverse myelitis may be a relatively uncommon manifestation of several autoimmune diseases including systemic lupus erythematosis (SLE), Sjogren's syndrome, and sarcoidosis. SLE is an autoimmune disease of unknown cause which affects multiple organs and tissues in the body. Features of this illness include arthralgias (joint pain) and arthritis (joint inflammation), rashes, kidney inflammation, low blood counts (including white and red blood cells, platelets), oral ulcers and the presence of abnormal autoantibodies (antibodies which are directed against the person's own tissues) in the blood. The fully developed syndrome of SLE is easy to recognize; however, this illness may begin with just one or two signs and is then more difficult to diagnose.

Sjogren's disease is another autoimmune disease characterized by invasion and infiltration of the tear and salivary glands by (lymphocytes) white blood cells with resultant decreased production of these fluids. Patients complain of dry mouth and dry eyes. Several tests can support this diagnosis: the presence of a SS-A antibody in the blood, ophthalmologic tests that confirm decreased tear production and the demonstration of lymphocytic infiltration in biopsy specimens of the small salivary glands (a minimally invasive procedure). Neurologic manifestations are unusual in Sjogren's syndrome, but TM can occur.

Sarcoidosis is a multisystem inflammatory disorder of unknown cause manifested by enlarged lymph nodes, lung inflammation, various skin lesions, liver and other organ involvement. In the nervous system, various nerves, as well as the spinal cord, may be involved. Diagnosis is generally confirmed by biopsy demonstrating features of inflammation typical of sarcoidosis.

Multiple sclerosis is an inflammatory autoimmune disease of the central nervous system (brain and spinal cord) which results in demyelination or loss of myelin (the insulating material on nerve fibers) with resultant neurologic dysfunction. A definite diagnosis of MS is not given until a patient has had at least two attacks of demyelination (hence, multiple) at two different sites in the central nervous system. The spinal cord is frequently affected in multiple sclerosis and may be the site of involvement of the first attack of MS. This presents the possibility that patients with acute transverse myelitis could later go on to have a second episode of demyelination and receive a diagnosis of MS.

Just what percentage of patients with a first attack of acute transverse myelitis will go on to develop MS is unclear in the medical literature, ranging from 15 to 80%; however, the majority of studies show a low risk. We do know that patients who have abnormal MRI scans of the brain with lesions like those seen in MS are much more likely to go on to develop MS than those who have normal brain MRIs at the time of their myelitis (between 60 and 90% for those with abnormal brain scans, less than 20% for those with normal scans in one study). It is also suggested in the medical literature that patients with "complete" transverse myelitis (which means severe leg paralysis and sensory loss) are less likely to develop MS than those who had a partial or less severe case. The literature also suggests that patients who have abnormal antibodies in their spinal fluid, called oligoclonal bands, are at higher risk to develop MS subsequently.

Myelitis related to cancer (called a paraneoplastic syndrome) is uncommon. There are several reports in the medical literature of a severe myelitis occurring in association with a malignancy. In addition, there are a growing number of reports of cases of myelopathy associated with cancer in which the immune system produces an antibody to fight off the cancer and this cross-reacts with the molecules in the spinal cord neurons. It should be emphasized that this is an unusual cause of myelitis.

Vascular causes are listed because they present with the same problems as transverse myelitis; however this is really a distinct problem primarily due to inadequate blood flow to the spinal cord instead of actual inflammation. The blood vessels to the spinal cord can close up with blood clots or atherosclerosis or burst and bleed; this is essentially a "stroke" of the spinal cord.

The general history and physical examination are first performed, but often do not give clues about the cause of spinal cord injury. The first concern of the physician who evaluates a patient with complaints and examination suggestive of a spinal cord disorder is to rule out a mass-occupying lesion which might be compressing the spinal cord. Potential lesions which might compress the cord include tumor, herniated disc, stenosis (a narrowed canal for the cord), and abscess. This is important because early surgery to remove the compression may sometimes reverse neurologic injury to the spinal cord. The easiest test to rule out such a compressive lesion is magnetic resonance imaging of the appropriate levels of the cord. However, if MRI is not available or the images are equivocal, myelography must be performed. A myelogram is a set of X-rays taken after a lumbar puncture has been performed either in the neck or in the low back and a contrast agent (dye) is injected into the sac that surrounds the spinal cord. The patient is then tilted up and down to let the dye flow and outline the spinal cord while the X-rays are taken.

If the MRI or myelogram shows no mass lesion outside or within the spinal cord, then the patient with spinal cord dysfunction is thought to have transverse myelitis or vascular problems. The MRI can sometimes show an inflammatory lesion within the cord. It is difficult to get to the cause of the inflammation, because biopsy is rarely done on the spinal cord because of the damage this would cause. The physician would next send blood for general bloodwork and studies for SLE and Sjogren's syndrome, HIV infection, vitamin B12 level to rule out deficiency and a test for syphilis. The next test which is commonly performed is a lumbar puncture to obtain fluid for studies, including white cell count and protein to look for inflammation, cultures to look for infections of various types, and tests to examine for abnormal activation of the immune system (immunoglobulin level and protein electrophoresis). A MRI of the brain is often performed to screen for lesions suggestive of MS. If none of these tests are suggestive of a specific cause, the patient is presumed to have idiopathic transverse myelitis or parainfectious transverse myelitis, if there are other symptoms to suggest an infection.

1. Jeffery DR, Mandler RN, Davis LE. "Transverse myelitis: retrospective analysis of 33 cases, with differentiation of cases associated with multiple sclerosis and parainfectious events." Arch Neurol, 1993; 50:532.

2. Berman M, Feldman S, Alter M, et. al. "Acute transverse myelitis: incidence and etiological considerations." Neurology, 1981; 31:966.

3. Stone LA. "Transverse Myelitis" in Rolak LA and Harati Y (eds.) Neuroimmunology for the Clinician. Boston, MA: Butterworth-Heinemann, 1997; 155-165.

Transverse Myelitis: Medical Specialists Medical
and Rehabilitation Treatment

Anyone that has been diagnosed with transverse myelitis has probably dealt with a number of medical specialists. Below is a brief description of each specialist's orientation and sphere of expertise. Please note, this summary is intended to be full of generalizations. Any individual physician can take it upon her or himself to acquire a greater knowledge base and expertise. These "definitions" are my own, and I apologize in advance if I offend any of my professional colleagues. I, myself, am a physiatrist (see below).

Family practitioners are physicians who are trained to treat virtually anyone who might walk in the door of a doctor's office. Their training includes rotations in pediatrics, obstetrics and gynecology, emergency medicine, and internal medicine. The strength of this training is an ability to formulate a treatment plan for patients with a multitude of complaints. Often, family practitioners act as a kind of triage. Patients with routine complaints are treated and followed, but for patients with relatively uncommon diseases such as TM, a referral to a neurologist is often in order.

Internists are specialists in internal medicine. The scope of an internist's practice typically includes diseases of the heart, lung, liver, kidney, blood, digestive system, joints, and endocrine systems. Internists are trained to treat adults, and many will seek subspecialty training in a particular organ system. Like family practitioners, internists tend not to have advanced training in the neuromuscular system, and would typically refer a patient with TM to a neurologist.

Neurologists are specialists in the neurologic system. They study and treat diseases of the central nervous system (the brain and the spinal cord) and the peripheral nervous system (the nerves from the spinal cord to the muscles and the sensory nerves). Neurologists also treat some diseases of the muscle. The neurologist will often be the one called on to make the diagnosis of TM and determine if a cause can be found. If a specific cause can be found (i.e., a bacterial infection), then efforts are focused on fighting the root cause, if possible (i.e., antibiotics).

Someone who is a specialist in physical medicine and rehabilitation is called a physiatrist. While other medical specialists aim at preserving and maintaining life, physiatrists are trained to preserve quality of life. When a neurologist examines the patient, he or she is trying to determine the cause of the disease with the hopes of prescribing a medical or surgical treatment. When a physiatrist examines the patient, he or she is trying to determine what the patient's ambitions, abilities, and limitations are. The physiatrist then tries to map a strategy to improve the patient's ability to function. Both neurologists and physiatrists typically prescribe medicines to alleviate symptoms, such as pain or spasticity; neurologists are trained to be experts regarding diagnosis and providing specific treatment. Physiatrists typically concern themselves with treating the problems brought on by loss of function, regardless of the diagnosis. They are expert at determining what kind of devices can make life and work easier (which brace, crutch, walker, reacher, wheelchair, etc. is needed). Physiatrists often consult and coordinate care delivered by physical, occupational and speech therapists, orthotists and prosthetists, psychologists, social workers and others. Physiatry is one of the smallest medical specialties with less than 6000 practitioners nationwide. Perhaps for this reason, physiatrists are a resource that is often overlooked in the treatment of people with debilitating diseases.

Medical treatment for people with TM can be divided into three phases. The first is the acute phase which might last from days to weeks. This phase begins when a person first falls ill. Typically, that person would go to a physician for help, and the medical community would try to discover what is wrong and try to fix it. If the problem was a broken bone in the leg, this process would usually be relatively simple. X-rays would be taken, and the bone would be set or casted, if needed. In the case of transverse myelitis, a person would probably be hospitalized and have lots of tests taken, including blood tests, magnetic resonance imaging (MRI's), or computed tomography (CT or CAT) scans. A "spinal tap" might be performed to analyze the cerebrospinal fluid. Depending on the seriousness of the illness, a catheter might be inserted into the bladder to help drain urine, and a breathing tube might be inserted to help with respiration. During this time, a cause might be found and specific treatment tried, or no cause might be found. In this case, sometimes intravenous (IV) steroids may be given. Some people will recover completely. Many others will be left with lasting deficits and will need help learning how to live their lives.

After the acute phase, people with TM enter a rehabilitation phase. During this time, the focus of care shifts from trying to find a cause and treatment to learning to live with a terrible disease. Two types of accommodations must take place. First, there is coming to terms psychologically. Here a person might feel the stages of grieving as if someone had died. The loss that a person with TM feels is real. Abilities that all healthy people take for granted vanish. Even the simplest tasks become effortful. Feelings of sadness, rage, grief, remorse, and guilt are not unusual. The task confronting the person with the disease is to rebuild his or her life. Most people base their self-worth, value, and satisfaction in living, at least partially, on what they are able to accomplish every day. When a person's self-sufficiency and independence are damaged, that person must rebuild an identity that allows the person to feel proud and whole from a new set of standards. Likewise, the family and loved ones are challenged to rebuild their relationship to the injured person. This can be excruciatingly difficult, yet many people accomplish this successfully. Despite the multitude of sorrows, there are often unexpected joys, such as finding support from those who were thought unsympathetic or unavailable, and finding talents that were hidden. Although I am not disabled myself, it is my impression that this accommodation is a lifelong task. Resources that might make this adjustment easier are psychological assistance from a counselor, discussions and meetings with religious leaders and congregations, and making contact with other people who have experienced the same or similar disease or injury.

The second set of adaptations is physical. I am not aware of medical literature specifically dealing with rehabilitation after transverse myelitis. However, much has been written regarding recovery from spinal cord injury (SCI), in general, and I think that this literature applies. The physical issues include bowel and bladder management, sexuality, maintenance of skin integrity, spasticity, activities of daily living (i.e., dressing), mobility, and pain.

Of primary importance is the level at which the spinal cord has been injured. The spinal cord is typically divided into four sections: the highest is the cervical (neck) region; then in descending order are the thoracic (chest), lumbar (low back), and sacral (lowest back) regions. Nerve roots exiting the cervical cord carry messages from the brain to the arms, thoracic to the chest and abdomen (i.e., the belly), lumbar to the legs, and sacral to the leg below the knee and bowel, bladder, and sexual organs. Because the nerves must travel through the spinal cord to connect with the brain, an injury to the spinal cord at a particular level usually effects function at that level and below. Therefore, a person affected at a specific thoracic level would typically have function disrupted in trunk balance (the thoracic nerves), as well as problems with leg movement and bowel and bladder control which are supplied by the lumbar and sacral regions of the spinal cord.

The bladder is controlled by nerves exiting the low thoracic, high lumbar, and mid sacral spinal cord. Bladder function may thus be impaired in SCI. Two general problems can affect the bladder. The bladder can become overly sensitive, and empty after only a small amount of urine has collected, or relatively insensitive, causing the bladder to become over extended and overflow. An overly distended bladder increases the likelihood of urinary tract infections and, in time, may threaten the health of the kidneys. Depending on the dysfunction, treatment options include timed voiding, medicines, external catheters for males (a catheter connected to a condom), padding for women, intermittent internal catheterization, or an indwelling catheter. Surgical options may be appropriate for some people.

A common problem in spinal cord injury is difficulty with evacuation of stool, although fecal incontinence can also occur. The neurologic pathways for defecation are similar to those of the bladder. Many lacking voluntary control of the bowel may still be able to achieve continence by diet, strategic use of stool softeners and fiber, and the technique of rectal stimulation. In rectal stimulation, a finger is inserted into the rectum to cause the internal and external anal sphincters to relax allowing the stool to pass. Other aids include suppositories and oral medications. There are some surgical options, although this is rarely necessary.

Sexuality is a complex issue. The bad news may be that sexual experience is impacted by spinal cord injury. Genital function is often altered (i.e., difficulties with erection and ejaculation for men and difficulties with lubrication for women). The good news is that sensual experience and even orgasm are still possible. Lubricants and aids to erection and ejaculation (for fertility) are available. Many individuals with SCI find unexpected erogenous zones. Ultimately, sexual experience happens in the brain, not in any specific organ. Adjustment to altered sexuality is aided by an attitude of permissive experimentation, as the previous methods and habits may no longer serve.

At The Ohio State University Medical Center, a nursing clinic is dedicated to provide practical help in matters of bowel, bladder, and sexuality for people with disabilities.

Skin breakdown occurs if the skin is exposed to undo pressure for a sufficient amount of time. Skin integrity is maintained in people without disabilities by two related mechanisms. First, the able-bodied have sensation, so that if they sit in one position for too long, they get uncomfortable. Secondly, they have the strength to shift position as necessary. Either or both of these mechanisms can be impaired in SCI. Sitting position should be changed at least every 15 minutes. This can be accomplished by standing, by lifting the body up while pushing down on armrests, or by just leaning and weight shifting. Wheelchairs can be supplied with either power mechanisms of recline or tilt-in-space to redistribute weight bearing. A variety of wheelchair cushions are available to minimize sitting pressure. Redness that does not blanch when finger pressure is applied may signal the beginning of a pressure ulcer. Good nutrition, vitamin C, and avoidance of moisture all contribute to healthy skin. Pressure ulcers are much more easy to prevent than to heal.

When the spinal cord is injured, muscle groups below the level of injury may become spastic. This manifests as stiffness and resistance to movement. They may also become hyper-reflexic and jerk when touched or hit. The cause of this is not fully understood. The management of spasticity must always be based on the person's function. For example, some people with TM will use the spasticity in their legs to help them walk. If this is treated, they may lose this ability. In contrast, someone whose spasticity prevents them from sitting in a wheelchair must be treated. If there has been a recent increase in spasticity, it is important to search for a cause. Noxious stimuli such as ingrown toenails, urinary tract infections, bowel impaction, kidney or gallbladder stones must be suspected.

Medical treatment of spasticity centers around four medications. Baclofen (Lioresal) is thought to inhibit reflex activity. It is considered the drug of choice for spasticity due to spinal cord injury. It is generally well-tolerated although it can be sedating. Abrupt discontinuation of baclofen can cause seizures and hallucinations. Diazepam (Valium) works by a similar mechanism, but is more likely to be sedative, and has been implicated in slowing recovery from brain injury. Dantrolene sodium (Dantrium) affects the muscles directly. While it is considered to be the drug of choice to treat spasticity due to brain injury, it may also play a role as an adjunct in the treatment of SCI spasticity. Tizanidine (Zanaflex) is a new drug to the US, but has been available in Europe for a long period of time. It reduces spasticity by a different mechanism than baclofen or dantrium and is generally well tolerated. Because it is more expensive than baclofen, and because most US physicians have less experience with it, it usually would not be the first choice.

Individuals with TM may find ordinary tasks such as dressing, bathing, grooming, and eating very difficult. Many of these obstacles can be mastered with training and specialized equipment. For example, long handled sponges can make bathing easier as can grab bars, portable bath seats and hand-held shower heads. For dressing, elastic shoe laces can eliminate the need to tie shoes while other devices can aid in donning socks. Occupational therapists are specialists in assessing equipment needs and helping people with limited function perform activities of daily living. A home assessment by an experienced professional is often helpful.

Physical therapists assist with mobility. Besides teaching people to walk and transfer more easily, they can recommend mobility aids. This includes everything from canes (single point vs. small quad cane vs. large quad cane) to walkers (static vs. rolling vs. rollator) and braces. For a custom-fabricated orthotic (brace), an orthotist is necessary. Careful thought should go into deciding whether the brace should be an ankle-foot orthosis, whether it should be flexible or stiff, and what angle the foot portion should be in relationship to the calf portion. Some will benefit by a knee-ankle foot orthosis.

Each person should be evaluated individually. I believe that the best results occur when the team is coordinated by a physician so that the therapists and orthotists are united with the patient on what is to be achieved. The physician best trained to take this role is the physiatrist.

Pain is common following SCI. The first step in treating pain effectively is obtaining an accurate diagnosis. Unfortunately, this can be very difficult. Causes of pain include muscle strain from using the body in an unaccustomed manner, nerve compression (i.e., compression of the ulnar nerve at the elbow due to excessive pressure from resting the elbow on an armrest continuously) or dysfunction of the spinal cord from TM. Muscle pain might be treated with analgesics, such as acetaminophen (Tylenol), non-steroidal, anti-inflammatory drugs such as naproxen or ibuprofen (Naprosyn, Alleve, Motrin), or modalities such as heat or cold. Nerve compression might be treated with repositioning and padding (i.e., an elbow pad for an ulnar nerve compression).

Nerve pain from the spinal cord is sometimes called "dysasthetic pain". Because of the SCI due to TM, nerve messages traveling through the spinal cord may become scrambled and misinterpreted by the brain as pain. Besides the treatments listed above, certain antidepressants such as amitriptyline (Elavil), or anticonvulsants, such as carbamazepine, phenytoin, or gabapentin (Tegretol, Dilantin, Neurontin) may be helpful. Stress and depression should also be addressed since these conditions make pain harder to tolerate.

This brief overview is not meant to include all possible areas of concern. I am grateful for the editing provided by Cindy Gatens.

Dr. Lynn and I would both be happy to entertain specific questions in the future. Please send your questions to Sandy Siegel either by postal service or e-mail, and he will pass the questions on to Dr. Lynn and I. We will attempt to provide you with answers to your questions in the next TMA Newsletter.