S Podobnik Šarkanji (Zagreb):
Bladder, bowel and sexual dysfunctions
in spinal cord disorders and peripheral neuropathies
Spinal cord injury
Spinal cord injury (SCI) is a cause of multisystemic impairments and thus a potential source of serious complications. Bladder, bowel and sexual dysfunctions are almost always present in patients with spinal cord injury. A very serious complication and secondary cause of death are urologic complications.
From the topographical point of view there are two types of lesions: upper motor neuron (UMN) bladder, due to suprasacral lesions, and lower motor neuron (LMN) bladder, due to a lesion situated in the sacral gray matter or cauda equina.
UMN bladder. The lesion is rostral to the lumbosacral level; the supraspinal and voluntary control of micturition is eliminated. First is the period of spinal shock - initial period of cessation of spinal reflexes including bladder areflexia, after which bladder hyperactivity occurs. Simultaneous contractions of the bladder and the external urethral sphincter leading to insufficiency of micturition (detrusor – sphyncter dyssynergia; DSD) are
often present. The uninhibited contractions cause severe incontinence, while DSD causes dysuria and chronic retention. DSD is the cause of high intravesical pressure, deterioration of the upper urinary tract, vesicouretheral reflux and hydronephrosis.
LMN bladder. The spinal pathways are interrupted, leading to the detrusor areflexia associated with denervation of the striated sphincter, dysuria and stress incontinence are clinically present.
Mixed lesions. Injury of the conus medullaris causes detrusor areflexia and nonrelaxing striated sphincter.
The choice of micturition management depends on the location of the lesion. The main goals of the chosen management are:
1. To preserve the urologic and genital function in the future
2. To correlate the mode of micturition with the functional abilities and the sex of the patient
3. To obtain continence if possible
4. To take into account the capacity of the patient to understand and accept the adapted technique
The management strategies bring into play: rehabilitation, pharmacologic and surgical interventions.
UMN bladder. The technique of reflex voiding is based on the reappearance of archaic spinal reflexes. In tetraplegic men the leakage can be collected by a sheath. In paraplegic men the choice of management is clean intermittent catheterization (CIC) in conjunction with anticholinergic drugs (ACH drugs).
In paraplegic women the choice of management is CIC with ACH drugs. In tetraplegic women a permanent catheter or suprapubic cystostomy is preferred.
The combination of CIC and ACH drugs is most often employed, but for patients who do not respond, other strategies must be considered: posterior sacral rhizotomies associated wit CIC, sacral anterior root stimulation. Posterior sacral rhizotomy allows better compliance, a decrease of detrusor sphyncter dysynergia, suppresion of reflex bladder contractions, and reduction of autonomic dysreflexia (AD). The procedure causes loss of reflex detrusor and erectile function and is therefore indicated in patients with a complete cord lesion. Stimulating electrodes are placed on the anterior roots of S2-S4 and the radio receiver is placed in the lower anterior chest. Micturition can be initiated by application of a mirror-image radio transmitter over the receiver.
LMN bladder. Guttman introduced clean intermittent catheterization in the 1970s. At the same time Lapides introduced the concept of host resistance, based on the notion that any bacteria introduced are neutralized by a healthy bladder wall. Bladder distension, the cause of the bladder wall ischemia in case of chronic retention and elevated pressures, was considered to be at the heart of most urinary infections. The frequency of catheterization is more important than sterility. The technique is simple: catheterization 6-7 times per day, meatus cleaning and hand washing with soap and water, rinsing the catheter with tap water, drying, and catheter storage in a dry bag.
Bowel dysfunction in SCI
The bowel dysfunction, loss of voluntary control of defecation, is a major physical and psychological problem. About ninety-five percent of SCI patients require at least one therapeutic method to initiate defecation and half of them are dependent on others for toileting. In most of them bowel evacuation takes more than 30 minutes and the time taken correlates with measures of distress and depression.
Management. Establishment of a bowel program should be individualized. Patients have different bowel patterns in terms of the frequency of bowel movements. The majority of SCI patients practice regular manual evacuation to prevent impaction and reduce the risk of autonomic dysreflexia. Hyperosmotic agents, such as lactulose and sorbitol can contribute to facilitating bowel motility and regularity.
Sexual dysfunction following SCI
SCI often occurs in young patients between 25 and 35 years of age, the sex ratio shows 80% being males.
Most men following suprasacral lesions are able to obtain reflex erections, but these are not usually adequate for intercourse. Patients with a lower motor neuron lesion rarely obtain erections, and those are most often of psychogenic origin. The recovery of erectile functions appears to be 70 to 80% in the SCI male population. Spontaneous ejaculation is rare in those patients , in only 5 to 10 % of the cases.
Management of erectile dysfunction must be counselled during the initial hospitalization. The vacuum erection device, intracorporeal injection of vasoactive medications, presently sildenafil, are all possible treatment of dyserection in SCI patients.
Penile vibratory stimulation (PVS) can induce ejaculation in almost 80% of SCI men with an injury higher than Th 10. Rectal electro-ejaculation may be used if penile vibratory stimulation fails.
In SCI women the resting phase is characterized by loss of sensitivity of the internal organs (with lesions above Th10) and external genitalia (above S2), and lack of motor control of the pelvic floor muscles. The excitation phase consists of vaginal lubrication. The thoracolumbal sympathetic center has a major role for psychogenic lubrication, and the
sacral parasympathetic center for reflex lubrication. In most SCI women the lubrication is not sufficient for penetration. SCI women appear to need longer stimulation before achieving orgasms. Diverse problems such as incontinence, spasticity, autonomic dysreflexia may substantially interfere with sexual performance as well.
Pregnancy in SCI women is possible, but must be considered as a high risk and requires rigorous management.
Cauda equina disorders
The spinal cord tapers to its end, the conus medullaris, usually at the lower edge of
the first lumbar vertebra. The ventral and dorsal lumbar and sacral nerve roots that arise from the conus medullaris and form a bundle are called cauda equina. The main destination
for these roots are the lumbar and sacral plexuses. Nerves from these plexuses provide motor and sensory innervation of the lower limbs and pelvic organs. Disorders affecting cauda
equina are characterized by weakness and sensory loss in the lower limbs, buttocks and perineum, usually with important abnormalities of bladder, bowel, and sexual functions. The causes of cauda equina damage can be acute or chronic.
Causes of acute cauda equina damage are: central disk herniation; vertebral collapse
due to metastic infiltration; spinal subarachnoid hemorrhage: acute extradural hematoma; trauma.
Causes of chronic cauda equina damage are: extrinsic tumors; primary tumors
arising from the cauda; spinal stenosis (degenerative spondylosis, achondroplasia, fluorosis); chronic central disk herniation; abscess; tuberculoma.
Central disk herniation. Disk herniations usually occur in a dorsolateral direction. Central disk herniations comprise less than 3% of all disk herniations. The disk usually involved is L4-L5, but herniations at other levels can occur. The symptoms and signs vary depending on the rate and extent of herniation, the size of the spinal canal, and the number of nerve roots involved.
The acute central disk herniation produces a serious syndrome of bilateral sacral,
buttock, perianal and posterior leg pain and numbness, weakness in the legs, and sphincter dysfunction. The patulous anal sphincter, loss of anal wink, and bulbocavernosus reflexes are present.
Chronic central disk herniations mimic tumors of the conus medularis or cauda equina.
The presenting complaints are often perianal pain or paresthesias, urinary dysfunction and erectile dysfunction in men.
Primary tumors of cauda equina. The most frequent primary tumors of cauda equina
are ependymomas and neurofibromas; rarer types include meningeomas, lipomas, dermoid tumors, schwanomas, hemangioblastomas and paragangliomas. The main symptom is pain, symptoms of nerve root compression usually develop later. Bladder disturbances are very often, while bowel and erectile dysfunction are less common. Occasionally, sphincter disturbances are the first and only symptom and no neurological signs are present.
Vertebral mestases, primary bone tumors, multiple myeloma can cause compression of
the nerve root, conus medularis or cauda equina. Compression of the conus medullaris or cauda equina can produce the classic picture of saddle anesthesia and loss of sphincter control.
Congenital malformations of the distal spinal cord often involve cauda equina. The
tethered cord syndrome (TCS) can present also in patients without known dysraphism. Those
patients have pain localized to the anal, perianal and gluteal areas, but the bladder
dysfunction is a prominent symptom; this usually consists of urinary urgency due to a hyperreflexic bladder, sometimes in combination with sphincter weakness contributing to incontinence.
Cauda equina claudication is the hallmark symptom of spinal stenosis. It consists of various combinations of low back, buttock and leg pain or paresthesia exacerbated by walking or standing. Bladder dysfunction is not common, but possible. The bladder and bowel dysfunction is the rule in patients with lumbar spinal stenosis as a result of ankylosing
Spinal arachnoiditis. The lumbosacral region is most commonly affected. Causes of spinal arachnoiditis are: Intrathecal drugs or chemical agents: radiological contrast agents,
local anesthetic drugs, amphotericin B, methotrexate. Spinal and epidural anesthesia.
Infections: tuberculosis, cryptococcosis, syphilis, viral infections. Trauma: spinal surgery,
vertebral injuries, lumbar disk herniation. Spinal subarachnoid hemorrhage. Idiopathic.
Viral cauda equina syndromes. The cytomegalovirus cauda equina syndrome is a
serious infectious disorder occurring in patients with acquired immune deficiency syndrome (AIDS), characterized by low back pain, asymmetric leg weakness, sensory loss, bladder and bowel incontinence.
Genital herpes simplex infections may cause a neurological syndrome consisting of
urinary retention, constipation, sacral pain or numbness. Spontaneous recovery usually occurs.
The peripheral nervous system is divided into the somatic and the autonomic nervous system. The somatic motor nerve fibers control skeletal muscle function, the sensory fibers mediate cutaneous and some deep sensations. The autonomic sensory and motor nerve fibers control most aspects of cardiovascular, gastrointestinal bladder and sexual functions. Disorders of peripheral nerves - peripheral neuropathies may involve somatic nerves,
autonomic nerves, or both.
Polyneuropathies include involvement of the autonomic nerves, focal neuropathies of the pelvic nerves produce bladder, bowel, and sexual dysfuncion. Examples of such polyneuropathies are: diabetes mellitus, amyloidosis, porphyria, Guillain-Barré syndome and some other chronic neuropathies.
Diabetes mellitus. Diabetes mellitus is the commonest cause of polyneuropathy, and the commonest polyneuropathy associated with bladder, bowel and sexual dysfunction. The severity and manifestations of diabetic polyneoropathy are highly variable from being unnoticed by the patient to a severe disabling neuropathy with marked weakness.
Diabetic autonomic neuropathy (DAN) is a frequent accompaniment of severe diabetic
polyneuropathy. Diabetic autonomic neuropathy can be restricted to certain organs or functions, especially to bladder and bowel, or may be widespread, involving most or all of the peripheral ANS.
Bladder dysfunction. Bladder dysfunction in diabetics – “diabetic cystopathy” at first
is usually insidious. Initially, there is reduced sensation of bladder fullness, and decreased frequency of voiding. This is followed by slowing of the urinary stream and difficulty in voiding. The impaired bladder emptying and urinary retention predispose to urinary tract infections.
Bowel dysfunction. There are three types of bowel dysfunction in diabetics:
constipation, diarrhea, and anorectal incontinence. Constipation is probably the commonest, and can be severe.
Diabetic diarrhea can also cause a greta deal of trouble and can be an isolated symptom of restricted autonomic dysfunction. It can be chronic, but is often intermittent, and may alternate with constipation or with normal bowel movements.
Diabetics may have dysfunctions of the more proximal gastrointestinal tract. Dysphagia, because of abnormal oesophageal motility can be present. Gastric atony may cause abdominal distension, nausea and vomiting.
Sexual dysfunction. This is common in diabetic males showing prevalence of 30-60%.
Usually , erectile dysfunction (ED) is present, but ejaculatory difficulties are also possible. Erectile dysfuncion in diabetic men begins with a progressive decline in erection rigidity and duration to the point where penetration and intercourse become impossible. Despite of lack of erection, ejaculation and orgasm often occur. It is thought that the high prevalence of ED my be due to a metabolic effect which blocks the vasodilatator action of released nitric oxide in corporeal tissue.
Amyloid neuropathy. There are various types of amyloidosis but those manifesting as somatic and autonomic neuropathy are: immunoglobulin amyloidosis and familial amyloidosis.
The dysautonomia often accompanies, but may precede the manifestation of a somatic polyneuropahy. Abnormalities of gastrointestenal motility can produce pseudoobstruction, diarrhea, constipation, or each of these alternating with the other. Bladder dysfunction and erectile dysfunction in men are common. Prominent are painful paresthesiasis.
Guillain–Barré syndrome. Autonomic dysfunctions, hyperactivity or hypoactivity of
sympathetic and parasympathetic functions, occurs in about 80% of patients. Major fluctuations in blood pressure, cardiac arrhythmias, anhidrosis can occur. Bladder dysfunctions occurs in 20% of patients. Recovery sometimes takes months. Constipation is common and paralytic ileus sometimes occurs.
Porphyria. Patients with porphyria can have acute attacks of motor neuropathy. Constipation, intestinal stasis, micturition difficulties, orthostatic hypotension, paroxismal hypertension and tachycardia may be a part of clinical syndrome.
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Kamm M. Bowel dysfunction in spinal cord injury patients. Lancet 1996; 347: 1651-3 3. Karlsson AK. Autonomic dysreflexia. Spinal cord 1999; 37:383-391 4. Malcolm A, Camilleri M. Treatment ndof diabetic gastroparesis and diarrhea. In: Dyck PJ, Thomas PK, editors. Diabetic neuropathy. 2 ed.
Philadelphia: Saunders; 1999. p 530-40. 5. Yang CC, Bradly WE. Treatment of diabetic sexual nddysfunction and cystopathy. In: Dyck PJ, Thomas PK, editors. Diabetic neuropathy, 2, ed.
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Slava Podobnik Šarkanji, MD, PhD, Professor of Neurology, Neurology Department, University Hospital “Sestre milosrdnice”, Vinogradska 29, HR-10000 Zagreb, Croatia