Transverse Myelitis

Original Editor - Wendy Walker

Lead Editors  Wendy Walker

Introduction

Transverse Myelitis (TM), AKA Acute Transverse Myelitis (ATM) is a rare neurological disorder of the spinal cord, caused by inflammation and occurring across one spinal segment, leading to severe motor, sensory and autonomic dysfunction.

The term Transverse Myelitis (TM) was first coined in 1948 by Dr Suchett-Kaye, an English neurologist. He uses this to describe a case of rapidly progressive paraparesis with a thoracic sensory level, occurring as a post-infectious complication of pneumonia.

In the MRI scan opposite, the TM lesion  is the lighter, oval shape at center-right.

Epidemiology

The incidence of TM is 1 (severe) to 8 (mild) cases/million per year.

It occurs in adults and children, in both genders, and in all races.

A peak in incidence rates (the number of new cases per year) appears to occur between 10 and 19 years and 30 and 39 years.

Mechanism of Injury / Pathological Process

The mechanism of injury is inflammation of the spinal cord causing damage to the myelin sheath of the nerves.  

Clinical Presentation

• Sensory, motor, or autonomic dysfunction attributable to the spinal cord
• Bilateral signs and/or symptoms
• Clearly defined sensory level

The symptoms of TM include muscle weakness, paralysis, parasthesia, neuropathic pain, spasticity, as well as bladder, bowel and sexual dysfunction.

In some cases, symptoms progress over hours whereas in other instances, the presentation is over days. Neurologic function tends to decline during the 4-21 day acute phase, while eighty-percent of cases reach their maximal deficit within 10 days of symptom onset. At its worst point, 50% of individuals have lost all movements of their legs, 80-94% experience numbness, paresthesias or banding or girdling, and almost all have some degree of bladder dysfunction.

There is tremendous variability in the presentation of symptoms, which are based on the level of the spinal cord affected and on the severity of the damage to the myelin and the neurons in the spinal cord.

Some patients recover from transverse myelitis with minor or no residual problems.
Others suffer permanent impairments that affect their ability to perform ordinary tasks of daily living.
Most people will have only one episode of transversemyelitis.
A small percentage (10-20%) may have a recurrence.

Diagnostic Procedures

MRI and/or CT scanning are invariably used in diagnosing TM. Up to 40% of cases have no findings on MRI.

Lumbar puncture is used to look for surrogate markers for inflammation in the cerebrospinal fluid (CSF).

Differential Diagnosis

The most common etiologies to be distinguished from idiopathic acute transverse myelitis are:

Multiple Sclerosis

Guillain Barre Syndrome

Disc herniation

Parainfectious myelitis
NMO (Devic's disease)
Myelitis related to systemic disease, such as systemic lupus erythematosis
Compression of spinal cord caused by trauma eg. vertebral fractures
Epidural or subdural hematoma
Epidural and/or paraspinal abscess
Tumour

Medical Management

Treatments are intended to reduce spinal cord inflammation and alleviate symptoms:

• Corticosteroids given intravenously
• Plasmapheresis - for moderate to severe cases, or those who do not respond to steroids after 3-5 days
• Immunosuppresent medication

Physiotherapy

1. Stretching programme – to prevent contractures
2. Strengthening
3. Transfers
4. Gait training
5. Wheelchair training
6. Reduce risk of pressure ulcers
7. Aid control of spasticity
8. Pain reduction/management

Bladder Problems

Bladder function is almost always at least transiently impaired in patients with TM.

Early stage, 1st 6-12 weeks:

Immediately after the onset of TM, there is frequently a period of transient loss or reduction of neural activity below the involved spinal cord lesion; this is known as “spinal shock,” and generally lasts approximately 6 to 12 weeks, although it can persist for 6 months or more.

Later stage, post spinal shock:

At this stage, two general problems can affect the bladder.

1. The bladder can become overly sensitive, and empty after only a small amount of urine has collected, OR
2. the bladder comes relatively insensitive, and thus becomes over extended and tends to 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 self-catheterization, an indwelling catheter, Botox or electrical stimulation. Surgical options may be appropriate for some people.

Prognosis

Some case series have estimated that approximately one third of patients recover with few or no lasting effects; another third have a moderate degree of residual disability, and one third remain severely disabled.

Resources

• The Transverse Myelitis Association is the world-wide organisation for TM and related rare autoimmune neurological diseases. This organisation is based in the USA and has close associations to medical and research communities specialising in neurological disorders. Their website is a good source of scientific information and articles on symptoms, treatments.
• The TM Society is a small UK based charity which offers information and support; they hold support group meetings throughout the UK, and also provide a telephone support service, answering questions on TM from patients, relatives or carers.

Case Studies

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References

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