Cauda Equina Syndrome Education
Cauda Equina Foundation, Inc. (The Foundation) believes that a key component to advancing the field of cauda equina syndrome (CES) is education. There is a severe lack of resources for education on CES for healthcare providers, patients, caregivers, the community, and researchers. Without education, progress can not be made to improve patient outcome- let alone find a cure. CES is a complex disease that requires a multidisciplinary approach and intensive medical management for patients to live their best lives- without education this is an impossible feat.
The Foundation's Medical Advisory, Scientific, and Review Board (MARSB) works diligently collecting research, conducting research, collating it into easy to understand educational materials for both patients and healthcare providers. Many serving on the MASRB live with CES themselves which gives a unique perspective to our educational materials. The MARSB works with other patients to gather information about the disease to ensure they are delivering the highest quality and most current information on hot patient topics such as- sexual dysfunction and constipation. If there is something you need to know about CES- you've come to the right place. *Remember to keep checking back for the latest information and new topics.
The Foundation's Medical Advisory, Scientific, and Review Board (MARSB) works diligently collecting research, conducting research, collating it into easy to understand educational materials for both patients and healthcare providers. Many serving on the MASRB live with CES themselves which gives a unique perspective to our educational materials. The MARSB works with other patients to gather information about the disease to ensure they are delivering the highest quality and most current information on hot patient topics such as- sexual dysfunction and constipation. If there is something you need to know about CES- you've come to the right place. *Remember to keep checking back for the latest information and new topics.
CAUDA EQUINA SYNDROME EDUCATION
What is Cauda Equina Syndrome?
The Cauda Equina Foundation, a non-profit organization, is focused on chronic Cauda Equina Syndrome (Ch-CES). Ch-CES has been defined as living with CES for 6 continuous months or more after initial clinical onset. The remainder of this document speaks further to the chronic stages of CES. This document is for educational purposes only and does not constitute medical advice.
Doctors typically think of Cauda Equina Syndrome as an acute condition. If you are experiencing the “red flag” symptoms of acute cauda equina as described elsewhere on this website, you may have a medical emergency and should seek help immediately. It is generally accepted, because it is medically logical, that the sooner the compression of the cauda equina nerve roots is relieved, the better for patient outcomes. Current recommendations are to proceed with surgery within 24-48 hours of symptom onset, although the clinical trial data supporting this concept is mixed.
Based on the amount of time you have had symptoms, cauda equina syndrome can be considered “chronic” Cauda Equina Syndrome Ch-CES. If you continue to have abnormal function of the main organs innervated by the cauda equina nerves including the bowel, bladder, sexual function as well as lower extremity sensory and motor function for more than 6 months it can be classified as chronic CES (Ch-CES). The abnormal sensory function can include intermittent or chronic pain, paresthesias (tingling, pricking, chilling, burning, numbness), and anesthesia (complete numbness) anywhere from the level of the low back, down the legs, the glutes, genitalia, all the way down to the bottom of the feet.
Doctors typically think of Cauda Equina Syndrome as an acute condition. If you are experiencing the “red flag” symptoms of acute cauda equina as described elsewhere on this website, you may have a medical emergency and should seek help immediately. It is generally accepted, because it is medically logical, that the sooner the compression of the cauda equina nerve roots is relieved, the better for patient outcomes. Current recommendations are to proceed with surgery within 24-48 hours of symptom onset, although the clinical trial data supporting this concept is mixed.
Based on the amount of time you have had symptoms, cauda equina syndrome can be considered “chronic” Cauda Equina Syndrome Ch-CES. If you continue to have abnormal function of the main organs innervated by the cauda equina nerves including the bowel, bladder, sexual function as well as lower extremity sensory and motor function for more than 6 months it can be classified as chronic CES (Ch-CES). The abnormal sensory function can include intermittent or chronic pain, paresthesias (tingling, pricking, chilling, burning, numbness), and anesthesia (complete numbness) anywhere from the level of the low back, down the legs, the glutes, genitalia, all the way down to the bottom of the feet.
Anatomy of CES
The Cauda Equina (see Figure 1) is a bundle of nerves descending from the end of the spinal cord. Anatomically, it is the major nerve roots below the end of the spinal cord typically starting at the L1 intervertebral disc and down through the end of the sacrum. The cauda equina (which translates to horses’ tail) was given this name because of its resemblance to the tail of a horse. The beginning parts of these nerve fibers are contained within the dural sac, which ends at about the level of S2. These nerve roots in the lumbar and sacral locations control the mechanical movement, function, and sensations of all organs within and below the pelvic cavity. These include the urinary bladder and urethra, genital and sex organs, lower colon, rectum and anus, muscles within the pelvic cavity, as well as the lower extremities.
Figure 1
Figure 1
Posterior cross section of the lower spine, sacrum and pelvis, showing the conus and cauda equina, parts of the nervous system affected by cauda equina syndrome or a conus injury.
From Vancouver Coastal Health G.F.Strong Rehab Centre.
From Vancouver Coastal Health G.F.Strong Rehab Centre.
Normal Physiology
Nervous system control of the legs and pelvic region can be divided into two groups of nerves. The upper motor neurons (also known as central motor neurons) are long nerve cells which run from the brain to the base of the spinal cord ending somewhere between the 12th vertebra and and the 2nd lumbar vertebra. The place where the spinal cord ends is cone shaped and hence known as the conus medularis. There, the nerves meet and send information through the peripheral neurons (known as lower motor neurons), which run from the nerve roots in the cauda equina to the pelvic organs and the lower body.
Cauda Equina Syndrome (CES)
Cauda Equina Syndrome (CES) is a term used to describe the dysfunction that occurs when one or more nerve roots which emerge from the cauda equina are damaged, by any mechanism. Injury here can result in a syndrome (CES) with a constellation of signs and symptoms which vary from person to person. One of the most common causes for CES is an extruded disc compressing the cauda equina nerve roots and also releasing harmful exudates from the core of the disc. There are many other causes for CES besides nerve root compression, per the list provided below.
Chronic CES can be difficult to diagnose as the symptoms may vary in intensity and may continue to evolve over an extended time frame depending upon the cause. CES is a clinical diagnosis and can be supported with diagnostic imaging. Currently in the US, MRI is the imaging modality of choice, demonstrating compression of the nerve roots inside the spinal canal. However, a computed tomography myelography can be used as an alternative when MRI is not available. Non-compressive CES can be more difficult to diagnose and require further testing. These tests are chosen based on the patient’s clinical presentation and the associated signs & symptoms.
The incidence of CES is thought to be around 1 per 65 thousand people making it a rare disease. It is more common in females and has no association with socio-economic demographics.
Chronic CES can be difficult to diagnose as the symptoms may vary in intensity and may continue to evolve over an extended time frame depending upon the cause. CES is a clinical diagnosis and can be supported with diagnostic imaging. Currently in the US, MRI is the imaging modality of choice, demonstrating compression of the nerve roots inside the spinal canal. However, a computed tomography myelography can be used as an alternative when MRI is not available. Non-compressive CES can be more difficult to diagnose and require further testing. These tests are chosen based on the patient’s clinical presentation and the associated signs & symptoms.
The incidence of CES is thought to be around 1 per 65 thousand people making it a rare disease. It is more common in females and has no association with socio-economic demographics.
Documented Possible Causes for CES
- Spinal lesions and tumors
- Spinal infections or inflammation
- Degenerative (lumbar disc extrusion, lumbar spinal stenosis, spondylolisthesis)
- Violent injuries to the lower back (gunshots, falls, auto accidents)
- Birth abnormalities such as spina bifida
- Spinal arteriovenous malformations (AVMs)
- Spinal hemorrhages (subarachnoid, subdural, epidural)
- Postoperative lumbar spine surgery complications
- Spinal anesthesia
- Complications after spinal surgery
- Autoimmune or paraneoplastic mechanisms
Possible Symptoms of CES
While every person may have their own set of symptoms, generally, cauda equina injuries affect the body in a well-recognized pattern. Symptoms the patient may experience include:
- Reduced or absent bladder, bowel and sexual function.
- A weakened (semi-flaccid) bowel and bladder leading to leakage (incontinence)
- Inability to empty the bowel and bladder completely (urinary retention and constipation). Many people with CES are unable to empty their bladder normally and must use a catheter on a regular schedule.
- Reduced or absent genital sensation and sexual arousal functions.
- The muscle stretched reflexes may be impaired.
- Reduced or absent skin touch sensation in the legs and pelvic region.
- Weakness and loss of muscle mass in hips and lower extremities leading to walking difficulties up to and including paralysis. This can take 5 to 8 years to fully manifest.
- Nerve pain in the pelvis, buttocks, groin and lower extremities, often down the back of the legs and can include the bottom of the feet.
- Some people with C-CES can develop “foot drop” in which they drag their toes when walking due to weakness or even paralysis of certain muscles in the legs and feet leading to difficulty walking and are prone to tripping and falling
Possible Signs of CES
- Impaired to absent leg reflexes
- Reduced to absent lower extremity muscle strength on examination
- Abnormal MRI findings including mass affect and compression of the cauda equina nerve roots
- Abnormal EMG (electromyelogram) findings including a low response to electrical muscle stimulation
- Urinary retention after voiding as measured by ultrasound
- Abnormal rectal tone as measured by manometry
- Abnormal colon muscle control and sensation
Recovery and Prognosis
An individual’s recovery is very challenging to predict and heavily dependent upon the underlying cause of CES. In chronic CES with therapy, some people can recover use of lower extremities and / or bowel, bladder and sexual function while others may not. Research into life expectancy following CES is quite limited. The natural history and scope of disability associated with chronic cauda equina syndrome has not yet been fully established.
A recent study used structured interviews with patients who had surgery for acute CES ranging from 2 to 10 years prior. The average age of the study population was 46 years and the average time since the operation was 62 months (5 yrs and 2 months). Interviews had a mean length of 45 minutes (range 27–72). The authors state that "this is the first qualitative study to explore the lived experience of CES patients according to the severity of the condition (CES-I and CES-R) in the “medium to long-term". Participant’s experiences of living with C-CES and its consequences were captured by 4 main themes: 1) Varying priorities of physical health; 2) A fragmented healthcare service; 3) The process of adjustment; and 4) Anticipatory anxiety and diminished sense of self-worth. These themes are described in great detail in the paper. Reference: Srikandarajah et al Ref #10. The data themes indicate that CES can be a chronic condition which requires holistic support to address long-term outcomes. This paper also highlights the importance of using the Cauda Equina Syndrome Core Outcome Set (CESCOS) in CES research studies wherever outcomes are recorded.
A recent study used structured interviews with patients who had surgery for acute CES ranging from 2 to 10 years prior. The average age of the study population was 46 years and the average time since the operation was 62 months (5 yrs and 2 months). Interviews had a mean length of 45 minutes (range 27–72). The authors state that "this is the first qualitative study to explore the lived experience of CES patients according to the severity of the condition (CES-I and CES-R) in the “medium to long-term". Participant’s experiences of living with C-CES and its consequences were captured by 4 main themes: 1) Varying priorities of physical health; 2) A fragmented healthcare service; 3) The process of adjustment; and 4) Anticipatory anxiety and diminished sense of self-worth. These themes are described in great detail in the paper. Reference: Srikandarajah et al Ref #10. The data themes indicate that CES can be a chronic condition which requires holistic support to address long-term outcomes. This paper also highlights the importance of using the Cauda Equina Syndrome Core Outcome Set (CESCOS) in CES research studies wherever outcomes are recorded.
Professional Medical Organizations and CES
Two key surgical organizations have general information about CES on their websites. We are providing their links below so you can access additional information about CES.
AAOS (American Association of Orthopedic Surgeons): https://orthoinfo.aaos.org/en/diseases--conditions/cauda-equina-syndrome/
AANS (American Association of Neurological Surgeons): https://www.aans.org/en/Patients/Neurosurgical-Conditions-and-Treatments/Cauda-Equina-Syndrome
AAOS (American Association of Orthopedic Surgeons): https://orthoinfo.aaos.org/en/diseases--conditions/cauda-equina-syndrome/
AANS (American Association of Neurological Surgeons): https://www.aans.org/en/Patients/Neurosurgical-Conditions-and-Treatments/Cauda-Equina-Syndrome
References
1. Woodfield, J et al. Presentation, management, and outcomes of cauda equina syndrome up to one year after surgery, using clinician and participant reporting: A multi-centre prospective cohort study. www.thelancet.com Vol 24 January, 2023
2. Srikandarajah N, Noble A, Clark S, et al. (2020) Cauda Equina Syndrome Core Outcome Set (CESCOS): An international patient and healthcare professional consensus for research studies. PLoS ONE 15(1): e0225907. https://doi.org/10.1371/journal. pone.0225907
3. Dearden A, Ghannam J, Wang AM, Barry KA, Krishnan A. The onus on the conus: a neuroimaging spectrum of conus medullaris pathologies. ASNR22/SNR XXII Proceedings. 2022 May 16-18:638-639.
4. Vancouver CoastalHealth G.F.Strong Rehab Centre. Online April 12, 2023
5. Teach Me Anatomy. Cauda Equina nerves in the Lumbar spine and The Sacrum. Written by Lorenzo Crumbie, Writer at Kenhub. Accessed online October 1, 2023.
6. Netter, F. (2014). Atlas of Human Anatomy. 6th ed. Philadelphia, PA: Elsevier: Saunders, pp.157-158, 160, 161, 167, 332-334, 477-478.
7. Sinnatamby, C. and Last, R. (2011). Last’s Anatomy. 12th ed. Edinburgh: Churchill Livingstone/Elsevier, pp.323-324.
8. Julie Woodfield, Simon Lammy, Aimun A.B. Jamjoom, et al. Demographics of Cauda Equina Syndrome: A Population-Based Incidence Study. Neuroepidemiology. 2023 Feb; 56(6): 460–468.
9. Nisaharan Srikandarajah, Simon Clark, Martin Wilby, et al. “I still don’t know if I am doing the right thing”: A qualitative study of experiencing cauda equina syndrome and its aftercare in the UK. Posted as a pre-print on October 4th, 2023. DOI: https://doi.org/10.21203/rs.3.rs-2698472/v1
2. Srikandarajah N, Noble A, Clark S, et al. (2020) Cauda Equina Syndrome Core Outcome Set (CESCOS): An international patient and healthcare professional consensus for research studies. PLoS ONE 15(1): e0225907. https://doi.org/10.1371/journal. pone.0225907
3. Dearden A, Ghannam J, Wang AM, Barry KA, Krishnan A. The onus on the conus: a neuroimaging spectrum of conus medullaris pathologies. ASNR22/SNR XXII Proceedings. 2022 May 16-18:638-639.
4. Vancouver CoastalHealth G.F.Strong Rehab Centre. Online April 12, 2023
5. Teach Me Anatomy. Cauda Equina nerves in the Lumbar spine and The Sacrum. Written by Lorenzo Crumbie, Writer at Kenhub. Accessed online October 1, 2023.
6. Netter, F. (2014). Atlas of Human Anatomy. 6th ed. Philadelphia, PA: Elsevier: Saunders, pp.157-158, 160, 161, 167, 332-334, 477-478.
7. Sinnatamby, C. and Last, R. (2011). Last’s Anatomy. 12th ed. Edinburgh: Churchill Livingstone/Elsevier, pp.323-324.
8. Julie Woodfield, Simon Lammy, Aimun A.B. Jamjoom, et al. Demographics of Cauda Equina Syndrome: A Population-Based Incidence Study. Neuroepidemiology. 2023 Feb; 56(6): 460–468.
9. Nisaharan Srikandarajah, Simon Clark, Martin Wilby, et al. “I still don’t know if I am doing the right thing”: A qualitative study of experiencing cauda equina syndrome and its aftercare in the UK. Posted as a pre-print on October 4th, 2023. DOI: https://doi.org/10.21203/rs.3.rs-2698472/v1
Additional information
Natural History Study
NATURAL HISTORY STUDY
Cauda equina syndrome (CES) is a devastating complication that can be caused by at least 20 known etiologies. The syndrome typically includes loss of bladder control, weakness of the lower extremities and varying degrees of bowel dysfunction, along with aberrations in sexual function. The natural history and scope of chronic disability associated with cauda equina syndrome has not yet been fully established.
CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for select rare diseases, including Cauda Equina Syndrome.
The Cauda Equina Foundation launched a Natural History study for C-CES on March 1, 2022. The purpose is to establish the typical patterns and level of disability from CES, categorized by the underlying etiology, over time by following a large cohort of participants with chronic CES by establishing the first US based registry for CES. This project will enlist participants with CES caused by a diverse set of underlying pathologies across the world. Understanding how the syndrome behaves over time will help healthcare providers to know what to expect in their patients and help to drive therapies and treatments. It will also help drive further research into this condition.
Participants in this prospective longitudinal observational cohort international registry study will complete a brief socio-demographic enrollment section of the CoRDs database before completing an approximately 45 question CES survey. Any subsequent entries in the database by participants will be to the CES survey only and not the CoRDS sociodemographic section. Participants will be permitted to update their answers in the registry at any time and will be encouraged to do so on an annual basis at a minimum going forward.
This study plans to enroll several hundred participants from around the world making it a unique and diverse international registry. Participants must have a diagnosis of CES, made by a qualified physician, and be at least 6 months out from their diagnosis. The investigators chose 6 months as the minimum time period to define the “chronic” phase of CES based on our collective experience. This study will include C-CES participants from any documented cause. There are no exclusion criteria.
CoRDS collects contact, sociodemographic and health information about participants. This information is entered into the CoRDS database and linked to a unique coded identifier.De-identified information in CoRDS will be made available to researchers, if they have obtained approval for their research project from (1) the Institutional Review Board (IRB) at the researcher's institution and (2) a panel of experts at Sanford Health.
The data generated from this registry will help the Cauda Equina Foundation (CEF) and our broader CES medical and social community to better understand the natural history of individuals living with chronic CES and how they can best be supported. We will look at potential differences in the natural history based on the underlying primary cause of CES. For example, is the natural history any different for patients who develop CES due to a herniated lumbar disc compared to a patient who develops CES from non-compressive causes. The Cauda Equina Foundation is the lead sponsor of this sentinel research.
THANK YOU TO THOSE WHO HAVE ALREADY PARTICIPATED IN THE NATURAL HISTORY STUDY. PLEASE LET US KNOW IF YOU QUALIFY AND WOULD LIKE TO PARTICIPATE IN THE ONLINE SURVEY TO HELP DETERMINE THE NATURAL HISTORY OF CES!!
Cauda equina syndrome (CES) is a devastating complication that can be caused by at least 20 known etiologies. The syndrome typically includes loss of bladder control, weakness of the lower extremities and varying degrees of bowel dysfunction, along with aberrations in sexual function. The natural history and scope of chronic disability associated with cauda equina syndrome has not yet been fully established.
CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for select rare diseases, including Cauda Equina Syndrome.
The Cauda Equina Foundation launched a Natural History study for C-CES on March 1, 2022. The purpose is to establish the typical patterns and level of disability from CES, categorized by the underlying etiology, over time by following a large cohort of participants with chronic CES by establishing the first US based registry for CES. This project will enlist participants with CES caused by a diverse set of underlying pathologies across the world. Understanding how the syndrome behaves over time will help healthcare providers to know what to expect in their patients and help to drive therapies and treatments. It will also help drive further research into this condition.
Participants in this prospective longitudinal observational cohort international registry study will complete a brief socio-demographic enrollment section of the CoRDs database before completing an approximately 45 question CES survey. Any subsequent entries in the database by participants will be to the CES survey only and not the CoRDS sociodemographic section. Participants will be permitted to update their answers in the registry at any time and will be encouraged to do so on an annual basis at a minimum going forward.
This study plans to enroll several hundred participants from around the world making it a unique and diverse international registry. Participants must have a diagnosis of CES, made by a qualified physician, and be at least 6 months out from their diagnosis. The investigators chose 6 months as the minimum time period to define the “chronic” phase of CES based on our collective experience. This study will include C-CES participants from any documented cause. There are no exclusion criteria.
CoRDS collects contact, sociodemographic and health information about participants. This information is entered into the CoRDS database and linked to a unique coded identifier.De-identified information in CoRDS will be made available to researchers, if they have obtained approval for their research project from (1) the Institutional Review Board (IRB) at the researcher's institution and (2) a panel of experts at Sanford Health.
The data generated from this registry will help the Cauda Equina Foundation (CEF) and our broader CES medical and social community to better understand the natural history of individuals living with chronic CES and how they can best be supported. We will look at potential differences in the natural history based on the underlying primary cause of CES. For example, is the natural history any different for patients who develop CES due to a herniated lumbar disc compared to a patient who develops CES from non-compressive causes. The Cauda Equina Foundation is the lead sponsor of this sentinel research.
THANK YOU TO THOSE WHO HAVE ALREADY PARTICIPATED IN THE NATURAL HISTORY STUDY. PLEASE LET US KNOW IF YOU QUALIFY AND WOULD LIKE TO PARTICIPATE IN THE ONLINE SURVEY TO HELP DETERMINE THE NATURAL HISTORY OF CES!!
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Red Flags of CES
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Clinical Trials
To learn more about clinical trials related to Cauda Equina Syndrome, visit: https://clinicaltrials.gov/search?cond=Cauda%20Equina%20Syndrome