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What is Transverse Myelitis?

By March 20, 2021No Comments

Transverse Myelitis

An introduction

Transverse myelitis is a rare disease condition of the nerves, mainly the spinal cord. The spinal cord and its coverings are inflamed at the level of the cervical or thoracic vertebra. There is sharp, shooting type of pain, abnormal sensation, and weakness or even the paralysis of the body part (mainly both the lower limbs) below the level of the involvement. Although the limbs are equally weak on both sides of the body, the weakness may be partial or unequal sometimes. However, the disease condition commonly affects the bowel and bladder function in almost all the patients and adds up the morbidity.

How does a person develop transverse myelitis?

Transverse myelitis is not a disease that we get by birth. It is an acquired disease condition but most often the cause of the disease is not known. [1] Viral infections caused by enteroviruses, herpes viruses, HIV, West Nile virus, etc. are linked with the development of inflammation of nerve sheath leading to Transverse Myelitis. Apart from infections, other disease conditions like rheumatoid arthritis (RA), ankylosing spondylitis, antiphospholipid syndrome, sarcoidosis, scleroderma, and systemic lupus erythematosus (SLE) has also been found to cause transverse myelitis. [1]

Who are at the risk of getting this disease?

Transverse myelitis is a rare disease in itself and has been found to affect 1 to 8 people per million people in a year. [1] The disease can affect both men and women equally. Although the disease can affect patients of all age groups, the incidence is more amongst the people between 10 and 19 years of age and also amongst the people between ages 30 and 39. [1]

Patients with other disease conditions like SLE, RA, HIV, etc. are even at more risk of developing this condition.

How do I know whether someone has developed Transverse Myelitis?

As the white matter of the spinal cord gets involved the patient starts developing symptoms like pain, tingling, and burning sensation of the body part below the level of involvement. More often the whole segment of the spinal cord is involved and when this happens there is the weakness of the limbs. The weakness may be complete or incomplete and may affect all four limbs when the disease is severe. The symptoms arise suddenly and the limbs are floppy (flaccid) initially which later on becomes stiff (spastic).

Autonomic nervous system involvement may give rise to problems like urinary and/or fecal incontinence. There might be other symptoms like difficulty voiding urine, constipation, and even sexual difficulties.

In severe cases, there is difficulty breathing when the spinal cord is affected in the level of the neck (C5 nerve root involvement). Respiratory failure can be devastating and can lead to death, unfortunately.

How does a doctor diagnose Transverse Myelitis?

Doctors initially take a detailed history and perform a relevant clinical examination and then ask for investigations. The most useful investigation modality is magnetic resonance imaging (MRI) of the brain and spinal cord. Sometimes, lumbar puncture (LP) is done by the doctors to examine the cerebrospinal fluid. Apart from these tests, blood is withdrawn to test for HIV, thyroid disorders, autoantibodies like rheumatoid factor (RA factor), serum antinuclear antibodies (ANA), Ro/SSA, and La/SSB autoantibodies, etc.

What are the treatment options?

The treatment of transverse myelitis should begin as soon as possible. Hospitalization of the patient is needed for newly diagnosed cases to monitor vital signs, to carry out investigations, or when symptoms are severe. Intravenous glucocorticoids in high doses are the preferred treatment modality. Medicines like methylprednisolone or Dexamethasone are used for 3 to 5days and are continued as per the progression of the disease. There are very few contraindications of steroid use and that’s why steroid treatment is started even before the test results are available. Steroids are believed to decrease the ongoing inflammation and reduce swelling of nerve sheath around the spinal cord. [2]

Intravenous immunoglobulin (IVIG) therapy and Plasma exchange therapy has gained popularity these days and this treatment modality may be effective when there is no appropriate response to the steroid therapy. [3]

Other medicines under study are cyclophosphamide, mycophenolate, and rituximab. These medicines are believed to have an immunomodulatory effect and can decrease the severity of the disease. [1]

Physiotherapy and rehabilitation programs also have an important role to prevent the development of contractures and deformity of the joints.

What are the chances of recovery once the patient develops this condition?

According to the researchers, almost one-third (33%) of the patients recover completely with minimal or almost none of the residual paralysis. The recovery starts within 1-3months of disease onset and progress speeds up with exercise and rehabilitation therapy. The complete recovery may take 2years in total.

 Another one-third patient population (33%) recover incompletely and have some degree of paralysis. These subsets of patients have a moderate degree of disability while carrying out daily activities. The remaining one-third of the patient population (33%) don’t recover at all and develop a permanent disability. [4]

The outcome is poor when the patient has severe symptoms including spinal shock during the initial visit, or has presented late, or has developed extensive lesions and syringomyelia when seen on MRI. [5]

How can the condition get complicated?

There are several complications of transverse myelitis and many of them are preventable. Complications like Urinary tract infections, pressure sores, spasticity, chronic pain, depression, and sexual dysfunction are common. Appropriate care and rehabilitation measures with frequent position change, urinary catheter exchange, appropriate antibiotics, and antispasmodic agents can prevent most of these and may improve outcomes.

References:

1. Simone CG, Emmady PD. Transverse Myelitis. StatPearls [Internet]. 2020 Jun 9.

2. Scott TF, Frohman EM, De Seze J, Gronseth GS, Weinshenker BG. 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. 2011 Dec 13;77(24):2128-34.

3. Cortese I, Chaudhry V, So YT, Cantor F, Cornblath DR, Rae-Grant A. Evidence-based guideline update: plasmapheresis in neurologic disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2011 Jan 18;76(3):294-300.

4. Krishnan C, Kaplin AI, Pardo CA, Kerr DA, Keswani SC. Demyelinating disorders: update on transverse myelitis. Current neurology and neuroscience reports. 2006 May 1;6(3):236-43.

5. Lim PA. Transverse myelitis. Essentials of Physical Medicine and Rehabilitation. 2020:952.

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