Rare Disease Spotlight: Cauda Equina Syndrome
/By Barby Ingle, PNN Columnist
The rare disease spotlight this month shines on cauda equina syndrome (CES), a serious neurological disorder that affects a bundle of nerves at the end of the spinal cord called the cauda equina. The condition is relatively rare and affects about 1 in 70,000 people.
Cauda equina syndrome (CES) was first described in the medical literature by Drs. William Mixter and Joseph Barr in 1934, although the earliest documented case happened to a man in the late 1800’s whose cauda equina was damaged during a surgical procedure that left him incontinent.
Nerves in the cauda equina give both motor and sensory function to the legs, bladder, anus and perineum. When damaged, CES can develop quickly or gradually, causing low back pain, numbness or weakness in the lower extremities, pain that radiates down the leg, and loss of bowel or bladder control.
In an acute onset, sensory and motor deficits in the lower body typically develop within 24 hours. Gradual onset can develop progressively and symptoms may come and go over the course of several weeks or months.
A sudden onset of sexual impotence, bladder dysfunction or sensory loss in the saddle region of your legs and buttocks can also be a sign of CES. Imagine sitting on a saddled horse and not being able to feel anything in the body areas that sit on a saddle. This would be a telling sign.
There are multiple causes of CES, including trauma, herniated discs, spinal stenosis, tumors, infections and inflammatory conditions such as Paget’s disease or ankylosing spondylitis. Medical errors like poorly positioned screws in the spine and spinal taps can trigger CES as well.
When there is an acute onset of CES, it usually requires an emergency surgical procedure. You need fast treatment to prevent lasting damage that causes incontinence or paralysis of the legs.
To diagnose CES, a patient will need an MRI (magnetic resonance imaging). This is the best method of imaging the spinal cord, nerve roots, intervertebral discs, ligaments and soft tissues affected by CES. If you are diagnosed with CES, you should see a neurosurgeon or spinal orthopedic specialist to assess the need for an urgent spinal decompression and other options.
Short of surgery, what can providers do to help reduce the symptoms of CES? This depends on what caused it to develop. One of the first options is the use of high dose corticosteroids, which can reduce swelling in the spine. If there is a mass or tumor involved, radiation or chemotherapy may be needed after surgery. If the cause is an infection, you will need antibiotics within 2 days of the onset of symptoms
Receiving proper and timely care is critical for CES and can assist in the recovery of bladder and bowel function. This may take years to fully reverse.
If you have CES or know someone with it, you can find support groups on Facebook here, here and here. The Cauda Equina Foundation and the Cauda Equina Champions Charity are also good resources for CES patients seeking advice and information.
Barby Ingle lives with reflex sympathetic dystrophy (RSD), migralepsy and endometriosis. Barby is a chronic pain educator, patient advocate, and president of the International Pain Foundation. She is also a motivational speaker and best-selling author on pain topics. More information about Barby can be found at her website.
