Cauda equina syndrome - Clinicals, Diagnosis, and Management

Neurology

Clinicals - History

Fact Explanation
Introduction Cauda equine syndrome [CES] is a collection of clinical features that results from the compression of the cauda equina, which is the lowermost part of the spinal cord comprising of the lumbosacral roots . The compression of the motor & sensory nerve roots supplying the lower limb, bladder, rectum, sphincters and the sexual organs gives rise to a collection of signs & symptoms related to their dysfunction .
The causative factors of compressing the cauda equina include central/ centrolateral disc prolapse, spinal stenosis ans spinal neoplasms . The non-compressive causes include ischemic insults, spinal arachnoiditis infections such as meningitis, as a complication after surgery/ anesthetic procedures / spinal manipulation and epidural injections . Lumbar disc herniation is the most common cause , with the herniation most commonly at L4/L5 or L5/S1 level . The compression/ischemia causes impairment of impulse propagation and change of neurotransmitters in the spinal cord, leading to the motor/sensory impairment resulting in CES .
Introduction
Cauda equine syndrome [CES] is a collection of clinical features that results from the compression of the cauda equina, which is the lowermost part of the spinal cord comprising of the lumbosacral roots . The compression of the motor & sensory nerve roots supplying the lower limb, bladder, rectum, sphincters and the sexual organs gives rise to a collection of signs & symptoms related to their dysfunction .
The causative factors of compressing the cauda equina include central/ centrolateral disc prolapse, spinal stenosis ans spinal neoplasms . The non-compressive causes include ischemic insults, spinal arachnoiditis infections such as meningitis, as a complication after surgery/ anesthetic procedures / spinal manipulation and epidural injections . Lumbar disc herniation is the most common cause , with the herniation most commonly at L4/L5 or L5/S1 level . The compression/ischemia causes impairment of impulse propagation and change of neurotransmitters in the spinal cord, leading to the motor/sensory impairment resulting in CES .
Symptoms of bladder dysfunction Patients commonly present with bladder problems due to the compression of S2,S3,S4 nerve roots which is the main innervation of the bladder and the urethral sphincter, and the most common symptoms include painless urinary retention and overflow incontinence. However, some patients may not present with bladder symptoms . In incomplete CES, patients may have altered urinary sensation, loss of desire to void and poor urinary stream due to loss of bladder innervation.
Note: asking for bladder symptoms is a very important component of the history before excluding CES .
Symptoms of bladder dysfunction
Patients commonly present with bladder problems due to the compression of S2,S3,S4 nerve roots which is the main innervation of the bladder and the urethral sphincter, and the most common symptoms include painless urinary retention and overflow incontinence. However, some patients may not present with bladder symptoms . In incomplete CES, patients may have altered urinary sensation, loss of desire to void and poor urinary stream due to loss of bladder innervation.
Note: asking for bladder symptoms is a very important component of the history before excluding CES .
Symptoms of bowel dysfunction This also results from the dusfunction of S2, S3 & S4 nerve roots that innervate the rectum and the anus. Patients do not present commonly with bowel symptoms as the bladder symptoms, and include non-specific symptoms ranging from constipation to incontinence. However, these symptoms may not be apparent in the acute stage due to loss of sensation in complete CES .
Important: as the the bladder & bowel symptoms have a gradual onset it is important to monitor for bladder & bowel symptoms in suspected CES .
Symptoms of bowel dysfunction
This also results from the dusfunction of S2, S3 & S4 nerve roots that innervate the rectum and the anus. Patients do not present commonly with bowel symptoms as the bladder symptoms, and include non-specific symptoms ranging from constipation to incontinence. However, these symptoms may not be apparent in the acute stage due to loss of sensation in complete CES .
Important: as the the bladder & bowel symptoms have a gradual onset it is important to monitor for bladder & bowel symptoms in suspected CES .
Anesthesia of the perianal area Most of the patients present with anesthesia of the perianal area . There is only partial/ unilateral loss of saddle/ genital sensation in incomplete CES, with preserved sensation of the trigone area . Anesthesia of the perianal area
Most of the patients present with anesthesia of the perianal area . There is only partial/ unilateral loss of saddle/ genital sensation in incomplete CES, with preserved sensation of the trigone area .
Weakness of lower limbs Although motor dysfunction is not a feature of classic CES, patients may present with lower limb weakness . Therefore it is important to ask this in the history of suspected CES. Weakness of lower limbs
Although motor dysfunction is not a feature of classic CES, patients may present with lower limb weakness . Therefore it is important to ask this in the history of suspected CES.
Sexual dysfunction Although not a common clinical finding of CES, patients may have sexual dysfunction. Thus patients should be inquired about erectile dysfunction, impotence & ejaculatory dysfunction .
Note: As patients are reluctant to express problems in their sexual function, it is the responsibility of the physician to obtain the relevant details from the patient as it helps in early and proper diagnosis .
Sexual dysfunction
Although not a common clinical finding of CES, patients may have sexual dysfunction. Thus patients should be inquired about erectile dysfunction, impotence & ejaculatory dysfunction .
Note: As patients are reluctant to express problems in their sexual function, it is the responsibility of the physician to obtain the relevant details from the patient as it helps in early and proper diagnosis .
Lower back pain Severe lower back pain is quite a rare symptom in cauda equina syndrome, with only 1 in 2000 patients presenting with this symptom . Sciatica may be associated with the back pain and often bilateral when present. However, sciatica may be absent when the compression is at or below the level of L5/S1 .
Important: if a patient presenting with back pain is having bladder & bowel symptoms and/or motor/sensory deficit in the pelvis and the lower limbs, CES should be suspected always . Severe lower back pain, sciatica, bladder, bowel & sexual dysfunction and saddle/genital sensory impairment are regarded as red flag symptoms and immediate action is needed in view of management .
Lower back pain
Severe lower back pain is quite a rare symptom in cauda equina syndrome, with only 1 in 2000 patients presenting with this symptom . Sciatica may be associated with the back pain and often bilateral when present. However, sciatica may be absent when the compression is at or below the level of L5/S1 .
Important: if a patient presenting with back pain is having bladder & bowel symptoms and/or motor/sensory deficit in the pelvis and the lower limbs, CES should be suspected always . Severe lower back pain, sciatica, bladder, bowel & sexual dysfunction and saddle/genital sensory impairment are regarded as red flag symptoms and immediate action is needed in view of management .
Lower limb pain This is not a common symptom and is non-specific. When present, lower limb pain is regarded as a poor prognostic factor of CES. When the compression is caused by a central disc herniation, the lateral lumbar nerve roots are spared hence symptoms of lower limb pain will be absent . Lower limb pain
This is not a common symptom and is non-specific. When present, lower limb pain is regarded as a poor prognostic factor of CES. When the compression is caused by a central disc herniation, the lateral lumbar nerve roots are spared hence symptoms of lower limb pain will be absent .

Clinicals - Examination

Fact Explanation
Motor examination of the lower limbs Although motor signs are not a feature of classic CES as the lumbar roots that gives motor supply to the lower limbs are usually spared in CES, weakness of lower limbs is found in most of the patients with CES . Also, straight leg raising test may be abnormal . Therefore, motor examination should be carried out in all the patients suspected of CES .
As reflexes of the lower limbs may be affected as well in some patients, it is advised to check for reflexes in the motor examination. The reflexes are diminished as the lower motor neurones are affected .
Motor examination of the lower limbs
Although motor signs are not a feature of classic CES as the lumbar roots that gives motor supply to the lower limbs are usually spared in CES, weakness of lower limbs is found in most of the patients with CES . Also, straight leg raising test may be abnormal . Therefore, motor examination should be carried out in all the patients suspected of CES .
As reflexes of the lower limbs may be affected as well in some patients, it is advised to check for reflexes in the motor examination. The reflexes are diminished as the lower motor neurones are affected .
Sensory examination of the pelvis Testing for both light touch and pin-prick sensation is a must in any person suspected of CES . The most common findings are anesthesia of the perineum & the saddle area .
Note: saddle anesthesia with sphincter dysfunction is known to be the most striking feature of CES , and complete perianal anesthesia at the presentation is considered to be a poor prognostic factor .
Sensory examination of the pelvis
Testing for both light touch and pin-prick sensation is a must in any person suspected of CES . The most common findings are anesthesia of the perineum & the saddle area .
Note: saddle anesthesia with sphincter dysfunction is known to be the most striking feature of CES , and complete perianal anesthesia at the presentation is considered to be a poor prognostic factor .
Digital examination of the rectum (DER) This should be done in every patient to asses for the anal tone and reflex contracture, as it helps in early diagnosis CES. However, most of the patients presenting with bladder & bowel symptoms have normal findings in the DER . Digital examination of the rectum (DER)
This should be done in every patient to asses for the anal tone and reflex contracture, as it helps in early diagnosis CES. However, most of the patients presenting with bladder & bowel symptoms have normal findings in the DER .
Abdominal examination Palpable bladder may be present due to urinary retention . Abdominal examination
Palpable bladder may be present due to urinary retention .
Complications of the condition It is necessary to be alert on deep vein thrombosis and pressure ulcers as a complication of CES, due to prolonged immobilization. The patient may complaint of severe pain on calf muscle and signs include tenderness, warmth, erythema & swelling of the leg . Complications of the condition
It is necessary to be alert on deep vein thrombosis and pressure ulcers as a complication of CES, due to prolonged immobilization. The patient may complaint of severe pain on calf muscle and signs include tenderness, warmth, erythema & swelling of the leg .

Investigations - Diagnosis

Fact Explanation
Magnetic resonance imaging of the spine (MRI spine) Emergency MRI spine should be carried out as soon as possible to prevent delay in diagnosis . Obliterated spinal canal by the herniated disc/ intraspinal mass can be seen in the MRI, confirming the diagnosis . Magnetic resonance imaging of the spine (MRI spine)
Emergency MRI spine should be carried out as soon as possible to prevent delay in diagnosis . Obliterated spinal canal by the herniated disc/ intraspinal mass can be seen in the MRI, confirming the diagnosis .
Computed tomography of spine (CT spine) This is recommended when MRI spine is not accessible . Computed tomography of spine (CT spine)
This is recommended when MRI spine is not accessible .
Urodynamic tests Useful in assessing bladder dysfunction and aids in the early diagnosis of CES. The ultra sound scan findings include large volumes of urine in post void residual testing despite no bladder complaints . Urodynamic tests
Useful in assessing bladder dysfunction and aids in the early diagnosis of CES. The ultra sound scan findings include large volumes of urine in post void residual testing despite no bladder complaints .
Spinal radiograph Helpful in excluding the differential diagnosis of vertebral fracture . Spinal radiograph
Helpful in excluding the differential diagnosis of vertebral fracture .
Electromyelogram Helps in excluding the diagnosis of GBS and other related muscle problems . Electromyelogram
Helps in excluding the diagnosis of GBS and other related muscle problems .
Erythrocyte sedimentation rate/ C reactive protein Helps to exclude inflammatory and other infective conditions such as GBS . Erythrocyte sedimentation rate/ C reactive protein
Helps to exclude inflammatory and other infective conditions such as GBS .
Capillary blood sugar/ HbA1C Useful in assessing the blood sugar in suspected/known diabetics as a guide to diagnosis. HbA1C gives an impression on the long term glucose control and used as a screening method for diabetes . Capillary blood sugar/ HbA1C
Useful in assessing the blood sugar in suspected/known diabetics as a guide to diagnosis. HbA1C gives an impression on the long term glucose control and used as a screening method for diabetes .

Investigations - Management

Fact Explanation
MRI spine This may be needed in the post surgical period to evaluate the condition, if the clinical features continue to persist. MRI spine
This may be needed in the post surgical period to evaluate the condition, if the clinical features continue to persist.
Urodynamic tests These may be needed in the long term if the patients are left with residual urinary symptoms. Urodynamic tests
These may be needed in the long term if the patients are left with residual urinary symptoms.

Management - Supportive

Fact Explanation
Prompt & early diagnosis and early referral to specialist care Early diagnosis and prompt action is mandatory in the outcome of the CES. Hence physicians should be well aware about the red flag symptoms and make necessary steps for early referral to a specialist unit to prevent delay in specific treatment and thereby offer the patient a good outcome . Prompt & early diagnosis and early referral to specialist care
Early diagnosis and prompt action is mandatory in the outcome of the CES. Hence physicians should be well aware about the red flag symptoms and make necessary steps for early referral to a specialist unit to prevent delay in specific treatment and thereby offer the patient a good outcome .
Patient education & counseling This is a very crucial aspect in the management to avoid unpleasant medico-legal issues which are notorious with the CES . The patient and the relatives should be explained about the condition and its short term & long term prognosis. Consent should be taken for surgery after explaining the possible residual neurological deficits that are likely to persist post surgery . Patient education & counseling
This is a very crucial aspect in the management to avoid unpleasant medico-legal issues which are notorious with the CES . The patient and the relatives should be explained about the condition and its short term & long term prognosis. Consent should be taken for surgery after explaining the possible residual neurological deficits that are likely to persist post surgery .
Treatment of the underlying cause The underlying cause that led to the CES should be investigated and treated to prevent recurrence. Treatment of the underlying cause
The underlying cause that led to the CES should be investigated and treated to prevent recurrence.
Treatment of complications Complications of CES such as pressure ulcers, deep vein thrombosis should be assessed and appropriate measures should be taken to prevent progression. Treatment of complications
Complications of CES such as pressure ulcers, deep vein thrombosis should be assessed and appropriate measures should be taken to prevent progression.
Follow-up care This should be carried out by a multi-disciplinary team (MDT) involving the neuro- surgery unit, urologist/gynecologist, physiotherapist, occupational therapist, gastroenterologist and a social worker . About 20% of patients with poor prognostic factors may need continuing care and support for self-catheterization, management of sexual dysfunction, colostomy, urological & gynecological surgery . The aim of follow-up is to rehabilitate the patient to become independent and to provide good quality of life. Follow-up care
This should be carried out by a multi-disciplinary team (MDT) involving the neuro- surgery unit, urologist/gynecologist, physiotherapist, occupational therapist, gastroenterologist and a social worker . About 20% of patients with poor prognostic factors may need continuing care and support for self-catheterization, management of sexual dysfunction, colostomy, urological & gynecological surgery . The aim of follow-up is to rehabilitate the patient to become independent and to provide good quality of life.
Physiotherapy Physiotherapy is necessary in the early period for recovery of motor functions. Physiotherapy
Physiotherapy is necessary in the early period for recovery of motor functions.
Occupational therapy Some patients may not be able to go back to their previous occupations or may need assistance, thus occupational therapy should be carried out as well. Occupational therapy
Some patients may not be able to go back to their previous occupations or may need assistance, thus occupational therapy should be carried out as well.
Psychological support As this can cause devastating effects to the quality of life of the patient, affecting his work & income/ personal & sexual life, the patient and the family members should be offered psychological support to overcome/ prevent depression and stress as a consequence of the condition. Psychological support
As this can cause devastating effects to the quality of life of the patient, affecting his work & income/ personal & sexual life, the patient and the family members should be offered psychological support to overcome/ prevent depression and stress as a consequence of the condition.
Social support The social worker has an important role in the assessment of the living conditions of the patient and identifying the areas of help required and making the necessary arrangements in order to give better quality of care and social support to the patient. Social support
The social worker has an important role in the assessment of the living conditions of the patient and identifying the areas of help required and making the necessary arrangements in order to give better quality of care and social support to the patient.

Management - Specific

Fact Explanation
Decompressive surgery Emergency decompression is indicated in CES as the clinical features become apparent when the cauda equina is severely compressed . The recovery of the bladder function is most likely to be found in patients who underwent surgery less than 24 hours after the onset of symtoms . Decompressive surgery
Emergency decompression is indicated in CES as the clinical features become apparent when the cauda equina is severely compressed . The recovery of the bladder function is most likely to be found in patients who underwent surgery less than 24 hours after the onset of symtoms .
Post-operative care A sympathetic approach should be followed with supportive care given for the patient and the family members . Initial bed rest should be carried out post surgery until recovery of bladder & bowel function. Long term follow-up and rehabilitation is discussed in the general management. Post-operative care
A sympathetic approach should be followed with supportive care given for the patient and the family members . Initial bed rest should be carried out post surgery until recovery of bladder & bowel function. Long term follow-up and rehabilitation is discussed in the general management.

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