Transverse Myelitis

Transverse myelitis (TM) is a subgroup category in a larger diagnostic grouping called neuromyelitis optica spectrum disorder (NMOSD) which also includes neuromyelitis optica, a paralysis of the optic nerve.

Transverse myelitis is an inflammation of the spinal cord, a major part of the central nervous system. The spinal cord carries nerve signals to and from the brain through nerves that extend from each side of the spinal cord and connect to nerves elsewhere in the body. The term myelitis refers to inflammation of the spinal cord; transverse refers to the pattern of changes in sensation—there is often a band-like sensation across the trunk of the body, with sensory changes below.

Causes of transverse myelitis include infections, immune system disorders, and other disorders that may damage or destroy myelin, the fatty white insulating substance that covers nerve cell fibers. Inflammation within the spinal cord interrupts communications between nerve fibers in the spinal cord and the rest of the body, affecting sensation and nerve signaling below the injury. Symptoms include pain, sensory problems, weakness in the legs and possibly the arms, and bladder and bowel problems. The symptoms may develop suddenly (over a period of hours) or over days or weeks.

Myelin is the fatty covering that surrounds the outside of nerves. If myelin is disrupted or destroyed, the nerve loses the ability to effectively transmit messages through the nerve. This interrupts messages from the body to the brain and from the brain to the body. Damage to myelin, called demyelination, disrupts the correct messaging of nerve impulses. As myelin becomes injured, protection of the nerve decreases leaving the nerve vulnerable to damage. The communication of messages along the nerve is also disrupted. There are two subgroups of TM, acute complete transverse myelitis (ACTM) which has long areas of nerve demyelination, and acute partial transverse myelitis (APTM) which has shorter areas of nerve demyelination.

The demyelination of nerves sounds very similar to the same effect in the development of multiple sclerosis (MS) and neuromyelitis optica (NMO). In fact, in many individual’s TM event can be a first episode of MS with eventual evolution into the disease.

TM produces decreased to absent sensation and motor function in the body starting at the level of injury in the spinal cord where myelin is attacked. Individuals with TM will often report a band around their body where the level of TM begins. The autonomic nervous system, the part of the nervous system that controls the automatic functions of your body such as heartbeat, is also affected.

Some experts believe that infection causes a derangement of the immune system, which leads to an indirect autoimmune attack on the spinal cord. The immune system, which normally protects the body from foreign organisms, mistakenly attacks the body’s own tissue, which causes inflammation and, in some cases, damage to the spinal cord myelin.

There are various causes to trigger the condition. It might be started by an immune disease or reaction that particularly affects the nervous system from:

• Infection
  • Viral due to varicella zoster (the virus that causes chickenpox and shingles), herpes simplex, Epstein-Barr, influenza, human immunodeficiency virus (HIV), hepatitis A, or rubella.
  • Bacterial skin infections, middle-ear infections, and bacterial pneumonia
  • Fungal infections such as Aspergillus, Blastomyces, Coccidioides, and Cryptococcus.
• Parasites including Lyme disease
• Vascular disorders such as malformations of the arteries and veins
• An unknown or untraceable cause

TM develops most often over hours, but some cases evolve up to four weeks. The symptoms may appear as pain, especially in the lower back, muscle weakness, abnormal or lack of sensation especially in the toes or feet or even higher up in the spine and involving the fingers and hands. As demyelination rapidly occurs, the symptoms increase, eventually to loss of sensation and paralysis. Demyelination usually occurs at the thoracic level, causing problems with leg sensation and movement as well as bowel and bladder control but it can be higher in the spinal cord adding loss of arm sensation and function.

Most people have one TM episode while a very few others might have more than one. Some people recover from TM with minor or no lasting problems, while others have long lasting impairments that affect their ability to perform ordinary tasks of daily living. Rehabilitation is needed to help restore function.

Source: NINDS

Symptoms

Transverse myelitis appears differently in individuals depending on the severity of the demyelination. Some will have partial losses, others will have complete loss of sensation and function starting at the level of the spinal cord where the TM attack has occurred. TM usually includes the following symptoms:

• Feeling like there is a band around the midsection
• Weakness of the legs and arms
• Pain
• Sensory alteration
• Bowel, bladder and sensory dysfunction

Pain is the primary symptom of transverse myelitis in about half of all patients. The pain may be localized in the lower back or may consist of sharp sensations that shoot down the legs or arms or around the torso. Sometimes, severe spasticity leads to pain.

Most people with transverse myelitis report heightened sensitivity to heat, cold, or touch; for some a light touch with a finger may cause significant pain (called allodynia).

Depression and anxiety can occur due to lifestyle changes and slow recovery of the disease. Daily challenges of chronic disease such as TM can be physically and emotionally taxing.

The effects of TM can be temporary or long lasting.

Diagnosis of TM

As with many neurological conditions, diagnosing TM can be difficult. First emergency conditions must be eliminated. The following assessments and tests are used to make a TM diagnosis.

A history and physical examination are completed. It is important to describe all your symptoms to your healthcare provider if you think they are TM symptoms or not. This should include when symptoms occur, and if you had symptoms in the past which have now resolved.

Your provider will perform an extensive neurological examination which includes assessment of your central nervous system (CNS) as well as the nerves and muscles of your body. Physical examination will include an assessment of all the nerves and joints of your body and testing the sensation of your body using a sharp object for pin prick touch and a cotton tip for light touch. Hot and cold sensation may be tested depending on your ability to tolerate the test due to heightened sensation.

MRI most often is used to view internal structures. Images of the spinal cord and brain are obtained. A lesion in the spinal cord but not in the brain is indicative of TM as are long lesions (long areas of demyelination). Lesions in the spinal cord and brain indicate MS and are typically shorter. Occasionally, a CT scan or x-rays are done depending on the individual’s needs.

Blood tests will be performed to assess antibodies within your body as well as monitoring your general state of health. Indicative of TM are aquaporin-4 antibody and anti-myelin oligodendrocytes. Later, follow up blood work will provide assessment of the function of medications.

Lumbar puncture or spinal tap will collect cerebral spinal fluid (the cushioning fluid around the brain and spinal cord to assess for increased protein and infection. Pleocytosis is present which is a large amount of white blood cells in the cerebral spinal fluid.

Outcomes of the MRI, lumbar puncture and blood tests might be within normal ranges at the start of the acute phase of TM. These tests might need to be repeated in about a week to find changes indicative of a diagnosis. Sometimes a diagnosis of TM might be made based on your clinical presentation by physical assessment.

Treatment and Rehabilitation

As with many disorders of the spinal cord, treatment of transverse myelitis is aimed at symptom reduction. A cure for TM has yet to be discovered.

Therapy generally begins when the patient first experiences symptoms. Treatment is guided by the symptoms and results of testing. Those diagnosed with TM may have different treatments depending on individual symptoms.

Inflammation is the body’s natural way of protecting itself. However, since the spinal cord is encased in the boney vertebrae, there is no room for inflammation or swelling. Therefore, the inflammation applies pressure to the affected area of the spinal cord and surrounding healthy tissue.

Steroids are mainly prescribed during the first few weeks of illness to decrease inflammation even though the effectiveness is unclear. Some who don’t respond to steroids may undergo plasma exchange therapy (plasmapheresis). Intravenous immunoglobulin (IVIG) might be provided to boost the immune system. Other medications are provided to help control pain and secondary symptoms. The goal is to keep the body functioning, while waiting for complete or partial spontaneous recovery of the nervous system.

Recovery from transverse myelitis usually begins within two to 12 weeks of the onset of symptoms. The nervous system will continue to attempt recovery throughout a person’s lifetime. However, if there is no improvement within several months, significant recovery is a slower process.

People with acute symptoms, such as paralysis, are most often treated in a hospital followed by care in a rehabilitation facility provided by a team of a specialized professionals. Physical and occupational therapy is started almost immediately to help improve muscle strength, coordination, and range of motion. Neurological recovery occurs with activity which is integrated into care at the level of function of the patient.

As with all chronic illness, psychological support may be needed to improve mental health. Long term illnesses with unpredictable outcomes can be mentally fatiguing for both the individual and family. Vocational therapy can assist in return to the work place.

The outcomes of TM are extremely variable. Predictions of recovery are difficult. Some people affected with TM experience good or full recovery. Others have a fair recovery and are left with deficits which might include a spastic gait, sensory dysfunction, and urinary urgency or incontinence. The remaining individuals will require assistance such as using a wheelchair for mobility and perhaps with dependence on others for basic functions of daily living.

A clinical practice guideline for health care professionals is available for diagnosis and treatment:

T.F. Scott, E.M. Frohman, J. De Seze, G.S. Gronseth, B.G. Weinshenker. Evidence-based guideline: Clinical evaluation and treatment of transverse myelitis. Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology, December 13, 2011; 77 (24) Special Article. First published December 7, 2011, DOI: https://doi.org/10.1212/WNL.0b013e31823dc535

Research

Extensive research is being conducted to find the cause of TM at the molecular level. Once the source of the problem is established, advancements in treatment options, reduction of conversion to other diseases such as MS and cure can be found. Many laboratory studies are being conducted to add to the body of existing knowledge.

Laboratory studies include those that are discovering why myelin gets attacked by the immune system, how myelin can be rebuilt around the nerves by oligodendrocyte progenitor cells (OPCs) and genetic studies focusing on genes such as Brg1 (Brahma-related gene).

Clinical trials are research studies with people. These include medications, disease progression and recovery and reduction of secondary complications.

Medications to reduce recurrence of TM are under study. These include Mitoxantrone and Rituximab. Both drugs are FDA approved for other diagnoses. The effectiveness in stopping TM recurrence has not yet been demonstrated.

Long term studies of disease progression in the acute phase and long-term recovery are being conducted. Secondary complications such as gait patterns and independence in activities of daily living are also underway.

Pediatric Research

Although children do not have a higher incidence of TM, it is being extensively researched.

Facts and Figures

Transverse myelitis occurs in adults and children, slightly more in women, and in all races. No familial predisposition is apparent.

Although TM can develop at any age, there are two peak times when the number of new cases per year appears to occur. These times occur between ages ten and 19 years and between 30 and 39 years of age.

About 1,400 new cases of transverse myelitis are diagnosed annually in the United States, and approximately 33,000 Americans have some type of disability resulting from TM.

No known onset trigger occurs in 16-60% of cases. This is called idiopathic onset. The wide percentage is due to difficulty in diagnosis which takes time. Much like other neurological diseases, the diagnosis of TM is made by ruling out other diagnoses first.

Individuals who present with acute partial transverse myelitis (APTM) might have a higher risk of converting to a diagnosis of Multiple Sclerosis (MS).

Resources

If you are looking for more information on TM or have a specific question, our Information Specialists are available business weekdays, Monday through Friday, toll-free at 800-539-7309 from 9:00 am to 8:00 pm ET.

Additionally, the Reeve Foundation maintains a transverse myelitis fact sheet with additional resources from trusted sources. Check out our repository of fact sheets on hundreds of topics ranging from state resources to secondary complications of paralysis.

We encourage you to reach out to transverse myelitis support groups and organizations, including:

• Siegel Rare Neuroimmune Disorders (formerly Transverse Myelitis Association (TMA))
• Johns Hopkins Hospital Department of Neurology, Transverse Myelitis Research Center
• The Cody Unser First Step Foundation
• The National Association for Rare Disorders
• National Institute of Neurological Disorders and Stroke
• The National Multiple Sclerosis Society

Your community might have a local chapter of the Siegel Rare Neuroimmune Disorders or National Multiple Sclerosis Society that can be helpful with resources and support groups.

Further Reading

Introduction

Beh SC, Greenberg BM, Frohman T, Frohman EM. Transverse myelitis. Neurol Clin. 2013 Feb;31(1):79-138. doi: 10.1016/j.ncl.2012.09.008. Review.

Young V, Quaghebeur G. Transverse Myelitis and Neuromyelitis Optica Spectrum Disorders. Semin Ultrasound CT MR. 2016 Oct;37(5):384-95. doi: 10.1053/j.sult.2016.05.004. Epub 2016 May 7. Review.

Diagnosis of TM section

West TW. Transverse myelitis–a review of the presentation, diagnosis, and initial management. Discov Med. 2013 Oct;16(88):167-77. Review.

Kitley JL, Leite MI, George JS, Palace JA. The differential diagnosis of longitudinally extensive transverse myelitis. Mult Scler. 2012 Mar;18(3):271-85. doi: 10.1177/1352458511406165. Epub 2011 Jun 13. Review.

Treatment and Rehabilitation section

Gupta A, Kumar SN, Taly AB. Neurological and functional recovery in acute transverse myelitis patients with inpatient rehabilitation and magnetic resonance imaging correlates. Spinal Cord. 2016 Oct;54(10):804-808. doi: 10.1038/sc.2016.23. Epub 2016 Mar 1.

Calis M, Kirnap M, Calis H, Mistik S, Demir H. Rehabilitation results of patients with acute transverse myelitis. Bratisl Lek Listy. 2011;112(3):154-6.

Research section

Kalita J, Misra UK, Mandal SK. Prognostic predictors of acute transverse myelitis. Acta Neurol.Scand. 1998; 98:60-3.

Ropper AH, Poskanzer DC. The prognosis of acute and subacute transverse myelopathy based on early signs and symptoms. Ann.Neurol. 1978; 4:51-9.

Christensen PB, Wermuth L, Hinge HH, Bomers K. Clinical course and long-term prognosis of acute transverse myelopathy. Acta Neurol.Scand. 1990; 81:431-5.

Pediatric Research section

Absoud M, Greenberg BM, Lim M, Lotze T, Thomas T, Deiva K. Pediatric transverse myelitis.Neurology. 2016 Aug 30;87(9 Suppl 2):S46-52. doi: 10.1212/WNL.0000000000002820. Review.

Wolf VL, Lupo PJ, Lotze TE. Pediatric acute transverse myelitis overview and differential diagnosis. J Child Neurol. 2012 Nov;27(11):1426-36. doi: 10.1177/0883073812452916. Epub 2012 Aug 21. Review.

van der Heijden LB, Janse AJ. Transverse myelitis in measles. Pediatr Neurol. 2015 Jan;52(1):132. doi: 10.1016/j.pediatrneurol.2014.09.018. Epub 2014 Oct 5

Facts and Figures section

National Institute of Neurological Disorders and Stroke

Bhat A, Naguwa S, Cheema G, Gershwin ME. The epidemiology of transverse myelitis. Autoimmun Rev. 2010 Mar;9(5):A395-9. doi: 10.1016/j.autrev.2009.12.007. Epub 2009 Dec 24. Review.