Transverse myelitis

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at August 3, 2016
StartDiseasesTransverse myelitis

When a rare disease strikes, the effects can be devastating to the individual and their families. One such disease is transverse myelitis (TM), which weakens the central nervous system to varying degrees. Transverse myelitis has been documented since 1882, but incidences of this disease are rare.

Contents

Definition & Facts

Transverse myelitis is a disorder that causes inflammation of the spinal cord, in turn affecting the myelin, or the soft, insulating tissues covering nerve cells. When such inflammation occurs, the myelin is destroyed or heavily damaged by scarring.

This results in reduced communication between the spinal cord, nerves, and the rest of the body. A person’s reaction time is not only hindered; involuntary activities such as breathing might also be affected.

Transverse myelitis was discovered in the late 1880s and was described as “vascular lesions and acute inflammatory events.” England in the 1920s saw an eruption of TM cases as an unintended side effect of the smallpox vaccine and rabies vaccines.

The National Institute of Neurological Disorders states that one-third of patients with transverse myelitis make a full recovery, one-third make a partial recovery, and one-third do not recover at all.

Symptoms & Complaints

Because transverse myelitis involves inflammation across the length of the spinal cord, it can stretch over multiple vertebral sections, and symptoms may vary depending on the location of the inflamed regions. For example, if the cervical spine is affected by transverse myelitis, the individual might have respiratory failure.

Despite the variability of symptoms, there are four key symptoms that are associated with this disorder:

Generally, someone affected by TM will lose the function of the spinal cord over a period of time that may be hours or weeks. A swift onset of lumbar pain, muscular weakness (stumbling, dragging limbs, unexplained heaviness) and tingling sensations will progress to symptoms like distorted sensations, paralysis, and urinary incontinence and fecal incontinence.

When it comes to sensory alteration, this tends to be unique for each individual patient. Temperatures in the body might be perceived as shifting between hot (hot flashes) and cold (chills). Pins and needles are also common. 

The severity of the myelitis can influence how much pain one experiences. A person might have localized pain or shooting, radiating stabs. Up to 80 percent of those suffering from TM have allodynia, which is acute pain at the slightest of touches. Even wearing clothing might become unbearable.

Lastly, bladder and bowel issues—incontinence or the inability to control urination and defecation, urinary retention and constipation—occur at high frequencies. 

Causes

There is no clear cause for many cases of transverse myelitis. However, it often occurs as a consequence of or in association with another health condition. TM is strongly linked to various viral infections, including sexually transmitted infections (STIs). The chickenpox and shingles virus can spur spinal inflammation. Other possibly causal viral infections include cytomegalovirus (CMV) infection, influenza, herpes simplex, hepatitis A, rubella, and human immunodeficiency virus (HIV).

Bacterial infections such as Lyme disease, ear infections, and mycoplasma pneumoniae are also associated with TM. Rather than these specific diseases and infections directly causing TM, it is thought to be the body’s own misdirected defenses that propagate the spinal inflammation. 

Vaccinations are also associated with transverse myelitis. Between 1970 and 2009, TM was noted as a possible side effect of measles-mumps-rubella vaccine and diphtheria-tetanus-pertussis vaccine, amongst others. 

Another way transverse myelitis might form is due to autoimmune diseases like systemic lupus erythematosus, Sjögren’s syndrome, sarcoidosis, and myasthenia gravis. There is evidence supporting the belief that TM could be another form of autoimmune disease, due to the way if affects the body’s immune system.

Diseases of the central nervous system, like multiple sclerosis (MS) and neuromyelitis optica (also known as Devic’s disease), are known to cause inflammation of the myelin.

Diagnosis & Therapy

If myelopathy is suspected, there are a few methods for diagnosis. In addition to a physical examination and asking the patient for a medical history and family history, imaging tests will be done.

One imaging scan that will likely be performed is a gadolinium-enhanced magnetic resonance imaging (MRI). The images obtained from the test will provide insight into whether or not spinal lesions or inflammation are present. MRIs can also rule out conditions like compressive myelopathy, tumors, hernias, stenosis, abscesses, bruising, and other causes of spinal pain.

Physicians may consider other diagnostic testing like a computed tomography (CT) scan with myelography. This involves dye being injected into the sac surrounding the spinal cord. Should TM be a suspected symptom of something else, like an autoimmune disease, blood tests will be ordered to rule out potential causes. 

Lumbar punctures, or spinal taps, are also used. Cerebrospinal fluid (CSF) is drawn to determine how much inflammation is present via laboratory analysis of white blood cells.

Treatment & Therapy

Upon identification of TM, there are several routes a patient's treatment may take. The start of therapy includes the use of intravenous corticosteroids. Corticosteroids can serve as an anti-inflammatory and an immunosuppressive drug. The standard is either 1000 mg of methylprednisolone or 200 mg of dexamethasone for three to five days. 

Next, a patient who has suffered from moderate to severe TM or has not recovered after a week or so of intravenous steroid usage will need plasmapheresis (PLEX). PLEX is a treatment similar to kidney dialysis but involves the removal, filtration, and transfusion of plasma.

In the event that both steroids and PLEX provide little relief, doctors may administer cyclophosphamide, a chemotherapy drug for lymphomas and leukemia that aggressively suppresses the immune system. 

Once TM is brought under control, rehabilitative care is crucial to getting an immobilized patient back on their feet. Physical therapy and occupational therapy are used to help mitigate any muscle atrophy and spasticity that has resulted as a side effect of treatment.

Spinal shock may affect the individual for some time, resulting in lingering paresthesia and transient sexual dysfunction and dysfunction of the bladder and bowel. However, through therapeutic measures, counseling, and the appropriate equipment (wheelchairs, braces, et cetera), the patient may be able to improve his or her quality of life.

Prevention & Prophylaxis

TM cannot be prevented in those cases where the cause is unknown or idiopathic, though awareness of the disease and early diagnosis increase the chances for positive treatment outcomes.

Receiving proper vaccinations against infectious agents and viruses known to cause TM is a sound preventative approach. Individuals with autoimmune disorders are advised to avoid potential flare-up triggers.