Transverse myelitis (TM) is a neurological disorder that affects around 1-8 per million people annually. It involves inflammation of the spinal cord, which is made up of neurons (nerve cells) responsible for carrying nerve impulses between the brain and the rest of the body. ‘Transverse’ refers to the spinal cord being affected across a particular level, although damage or inflammation could be localised at a particular focal point, too.

While TM could manifest at any age, research shows that incidence is higher between the ages of 10-19 years and 30-39 years. Recovery varies, with some patients showing complete or at least partial improvement of symptoms over varying periods of time – from months to years, while some may experience permanent repercussions.

Symptoms

There are four main symptoms associated with the incidence of TM. Patients may experience one or more of the following:

• sensory alteration – this may include numbness, tingling, increased sensitivity to touch, coldness, burning, or even loss of sensitivity to pain and/or temperature
• pain – this may be experienced as a localised pain in the back region, or a pain that extends out into the arms and/or legs or around the torso
• weakness in legs and/or arms – this often happens more commonly in the legs, with patients experiencing dragging of feet, stumbling, a feeling of heaviness; in cases where arms are affected, coordination may become challenging; these symptoms may be symmetric or asymmetric
• bowel and/or bladder dysfunction – this may manifest as incontinence, constipation, increased frequency in urinating/defaecating, difficulty in or incomplete voidance

Complications

In most cases of TM, the patient may experience some form of long-term or permanent impact of the disease, depending on the extent of damage to the spinal cord as well as effectiveness and response to treatment. These may include:

• spasticity – This refers to muscle stiffness or spasming, which can be painful and reduce control over movement. Trigger factors may include movement, changes in temperature and humidity, infection. Severe spasticity could lead to contractures (shortening of muscles or joints), particularly if not managed effectively.
• immobility / paralysis – Most TM patients experience some form of temporary or permanent paralysis. A prompt diagnosis followed by the correct medication and therapeutic treatment in a generally healthy person may help to minimise the degree and period of immobility or paralysis. However, in cases where treatment is delayed or not administered effectively, or the patient responds poorly, there may be long-term consequences in the form of partial paralysis, depending on which part of the spinal cord has been affected.
• bladder and bowel function complications – As mentioned previously, bladder and/or bowel dysfunction may be one of the early symptoms of TM. These effects may be long-term in some cases.
• neurological complications – TM patients may experience changes in sensation such as numbness or excessive pain, referred to as neuropathic pain.
• frequent fatigue – This may be a direct result of TM itself (primary fatigue), or a side effect of medication, discomfort, lifestyle changes, etc. (secondary fatigue).
• psychological complications – The often sudden and drastic changes in an individual’s life that result from TM can be overwhelming for some, particularly those lacking access to treatment and a good support system. This could lead to conditions such as depression, which could in turn hamper the recovery process and how the patient subsequently gets on with their life. TM can significantly impact the patient’s family as well as they, too, need to adapt to a new lifestyle that is more compatible with the patient’s needs and limitations.
• recurrent TM – In cases where other disorders have been ruled out by extensive testing, TM may recur, giving rise to multiple episodes of TM in a patient’s lifetime.

Causes

The cause of TM is generally considered to be idiopathic. However, a few aspects that have been associated with TM include:

• disorders of the immune system, such as multiple sclerosis (where the immune system mistakenly attacks the myelin sheath of nerves in the brain, spinal cord or optic nerves), neuromyelitis optica (which affects the optic nerves and spinal cord), post-infection/vaccination autoimmunity complications, autoimmunity in response to an underlying cancer
• infections, which may include viral (e.g. varicella zoster, herpes simplex, influenza, hepatitis B, mumps, measles, rubella, etc.), bacterial (e.g. tuberculosis, tetanus, diphtheria, syphilis, Lyme disease, bacterial pneumonia, etc.), fungal (in the spinal cord) and parasitic (e.g. toxoplasmosis, shistosomiasis, etc.) infections
• inflammatory disorders, such as systemic lupus erythematosus (SLE), sarcoidosis, Sjogren’s syndrome, etc.
• vascular disorders, such as intra-spinal cavernous or arteriovenous malformations, disk embolisms

Diagnosis

Prior or concurrently to the scans mentioned below, a patient suspected of having TM may be tested for other systemic disorders such as infections, metabolic disorders, rheumatologic disorders by means of blood tests.

Diagnosis of TM is usually done by means of:

• magnetic resonance imaging (MRI), which is used to observe for inflammation, lesions, signal abnormalities in the spinal cord; paramagnetic contrast may or may not be used when scanning
• lumbar puncture (spinal tap / spinal fluid analysis), where a sample of cerebrospinal fluid (CSF) is drawn from the spinal cord in order to determine the level of inflammation by studying the sample for the presence and quantity of white blood cells involved in inflammation

Prevention

While it may not be possible to prevent the incidence of TM, long-term treatment with immunosuppressants (such as steroid-sparing drugs) may help to prevent the recurrence of TM in patients.

Treatment

Usually, the first line of treatment as soon as an individual is diagnosed with TM is the administration of intravenous steroids. This often involves a high dose of methylprednisolone or dexamethasone for about 3-5 days, as per the patient’s condition. Treatment may be extended, if required. The purpose of this is to reduce acute inflammation in the spinal cord and tone down the immune system’s activity.

Plasma exchange (PLEX) therapy (plasmapheresis) is used in cases of moderate to aggressive TM where the patient shows little or slow response to intravenous steroids. The process involves replacing the patient’s blood plasma (which contains antibodies) with an alternative fluid, thereby intending to reduce the inflammatory response. However, there still is only a limited amount of clinical data on this method of treatment although it has been found to show positive results in some cases, particularly when preceded by intravenous steroids.

Another form of therapy that is being researched but has still not been tried and tested sufficiently is the administration of intravenous immunoglobulin (IVIG), where donor antibodies are injected into the patient’s plasma in order to try to bind to and remove the patient’s antibodies from circulation. However, only time and sufficient testing will prove how effective this treatment could be.

Apart from these forms of direct treatment, patients may also be prescribed with analgesics (painkillers – mostly for muscle aches), muscle relaxants, antidepressants (for nerve discomfort), anticonvulsants, antiviral medication (for viral spinal cord infections), as well as medication for other symptoms such as incontinence, constipation, etc.

Long-term Management

Long-term management or life-long therapy is often required to optimise recovery and maintain the maximum possible mobility and functionality of the patient. As such, measures taken may include:

• rehabilitative physiotherapy – in order to improve muscular strength and flexibility, and coordination. This would help to subsequently achieve optimal mobility for the patient and help them to attain the maximum possible independence in their life. Patients are often given exercises to be done daily in order to maintain mobility and muscle tone. Physiotherapy and exercises may also help to deal with other complications such as bladder and bowel dysfunction.
• use of assistive devices – such as braces, canes, wheelchairs, etc. that would support the patient to achieve better mobility.
• suitable diet – if bladder and/or bowel dysfunction persist, food and fluid intake may have to be monitored and controlled accordingly.
• psychotherapy – to assist the patient to deal with the mental and emotional challenges of dealing and living with the various impacts of TM and subsequent lifestyle changes.
• vocational / occupational therapy – to assist the patient to rebuild or develop skills that would help them in everyday life, as well as for employment and independence.

Living with any form of paralysis is not easy for any individual – particularly when onset is unexpected and life-changing. Patients with TM hence need to be given every support that they can receive from family and friends. Likewise, it is important to consider the physical, mental and emotional implications on their families and caregivers, and to reach out to them, too, in whatever ways possible.

Cover illustration adapted from 123RF