Editing Neurogenic LUT Dysfunction (section)

== Diseases primarily involving the spinal cord ==

* '''Examples: MS, SCI, transverse myelitis, tabes dorsalis, pernicious anemia, spinal dysraphism, poliomyelitis'''

=== Multiple sclerosis ===
* Primarily a disease of adults ages 20-50 years with a 2x predilection for women
* The disease is believed to be immune mediated and is characterized by neural demyelination in the brain and spinal cord; it is characterized, in general, by axonal sparing'''. The demyelinating process most commonly involves the''' lateral corticospinal (pyramidal) and reticulospinal columns of the '''cervical''' '''spinal cord'''
* '''Common symptoms include optic nerve dysfunction, pyramidal tract abnormalities (hyper-reflexia), ataxia, bowel dysfunction, neurogenic bladder, and bowel and sexual dysfunction'''
* 50-90% report voiding symptoms at some time; the prevalence of incontinence is cited as 37-72%
* '''Urodynamic findings'''
** '''Detrusor overactivity is the most common abnormality detected'''
** '''In general, the smooth sphincter is synergic'''
** '''Striated sphincter dyssynergia coexists with overactivity in 30-65% of patients'''
** The prevalence of coexistent impaired detrusor contractility or areflexia ranges from 12-38%, a phenomenon that can considerably complicate treatment efforts
** One must be careful to distinguish urodynamic pseudodyssynergia from true striated sphincter dyssynergia
** Sensation is frequently intact in these patients
* '''The most common functional classification applicable to patients with LUT dysfunction secondary to MS would be storage failure secondary to detrusor overactivity. This is commonly complicated by striated sphincter dyssynergia, with varying sequelae based on the patient’s ability to empty completely at acceptable voiding pressures'''. Other abnormalities, and especially combined deficits, are obviously possible
* Progressive neurologic disease in patients with MS rarely causes upper urinary tract damage, even when severe spasticity and disability exist
* '''Management'''
** At present there is no consensus on optimal bladder management for patients with MS, and management is most commonly predicated on symptomatic and urodynamic findings.
** '''Caution should be exercised in recommending irreversible therapeutic options, because a significant proportion of patients with MS, both with and without new symptoms''', will develop changes in their detrusor compliance and urodynamic pattern.
*** Surgical intervention for MS appears to be diminishing with improved pharmacologic management and the realization of the alternating neurologic picture of lower urinary dysfunction associated with MS

=== Spinal cord injury ===
* '''See [[CUA: Neurogenic Lower Urinary Tract Dysfunction (2019)|2019 CUA NLUTD Guideline Notes]]'''
*'''The majority of SCIs occur at or above the T12 spinal column''' (vertebral) '''level, with injury to one of the 8 cervical segments accounting for the patients with tetraplegia and with patients with paraplegia having injury in the thoracic, lumbar, or sacral regions of the spinal cord.'''
* '''Urologic complications of SCI (8):'''
*# '''UTI'''
*# '''Sepsis'''
*# '''Upper urinary tract and LUT deterioration'''
*# '''Urolithiasis'''
*# '''Sexual and reproductive dysfunction'''
*# '''Autonomic hyperreflexia (dysreflexia)'''
*# '''Skin complications'''
*# '''Depression (which can complicate urologic management)'''
* '''Despite the strong correlation between neurologic and urodynamic findings, it is not perfect, and a neurologic examination is no substitute for a urodynamic evaluation in these patients''' when one is determining risk factors and treatment
* '''Spinal shock'''
** '''A temporary physiologic disorganization of spinal cord that may be expected after a significant SCI'''
** '''Defined as the loss of motor, sensory, reflex, and autonomic neurologic function below the level of SCI'''
** Classically starts within one hour after the neurologic injury.
** '''Bladder is acontractile and areflexic. The bladder neck is usually closed and competent'''
** '''The smooth sphincter mechanism appears to be functional. Some electromyographic activity may be recorded from the striated sphincter'''
** '''The normal guarding reflex (striated sphincter response during filling) is absent and there is no voluntary control'''
** '''Urinary retention is the rule, and catheterization is necessary to circumvent this problem'''
*** Once the patient is medically stable and can regulate fluid intake, the indwelling catheter can be removed and clean intermittent catheterization should be started.
** '''Spinal shock usually lasts 6-12 weeks in complete suprasacral spinal cord lesions''' '''but may last up to 1 or 2 years.'''
*** May last a shorter period of time in incomplete suprasacral lesions and only a few days in some.
**** The exception is sacral SCI which has persistent detrusor areflexia
*** '''Resolution of spinal cord shock classically begins with the initial return of the bulbocavernosus reflex followed by the eventual restoration of the deep tendon reflexes (DTR) below the level of spinal cord injury.'''
** '''Initial urodynamics should be performed within 3 months of SCI onset regardless of spinal shock resolution and then repeated after spinal shock has resolved.'''
*** '''The ideal time to perform initial urodynamics is after spinal shock has resolved,''' which can take up to 3 months in most cases.
**** Nearly two-thirds of patients had unfavorable urodynamic parameters (detrusor overactivity, vesicoureteral reflux and high detrusor filling pressures) within 40 days after SCI.
*** Videourodynamics with fluoroscopy is the gold standard for urodynamic investigation in patients with neurogenic bladder.
*** The Society of Urodynamics, Female Pelvic Medicine and Urogenital Reconstruction best practice policy on antibiotic prophylaxis during urodynamics recommends that patients with SCI receive a single antibiotic dose to avoid symptomatic UTI and bacteremia.
* '''Suprasacral spinal cord injury'''
** The characteristic pattern in a patient with a complete lesion above the sacral spinal cord is detrusor overactivity, smooth sphincter synergia (with lesions below the sympathetic outflow), striated sphincter dyssynergia, and absent sensation below the level of the lesion
*** Lesions at or above the spinal cord level of T7 or T8 (the spinal column level of T6) may result in smooth sphincter dyssynergia as well
*** The striated sphincter dyssynergia causes a functional obstruction with poor emptying and high detrusor pressure.
*** Occasionally, incomplete bladder emptying may result from what seems to be a poorly sustained or absent detrusor contraction.
** Neurologic examination shows spasticity of skeletal muscle distal to the lesion, hyperreflexic DTRs, and abnormal plantar responses. There is impairment of superficial and deep sensation
** The guarding reflex is absent or weak in most patients with a complete suprasacral SCI. In incomplete lesions the reflex is often preserved but quite variable
** From a functional standpoint, the voiding dysfunction most commonly seen in suprasacral SCI represents both a filling or storage and an emptying or voiding failure. Although the urodynamics are “safe” enough in some individuals to allow only periodic stimulation of bladder reflex activity, many will require some form of additional treatment.
* '''Sacral spinal cord injury'''
** Typically, a depression of DTRs below the level of a complete lesion with varying degrees of flaccid paralysis. Sensation is usually absent below the lesion level. Detrusor areflexia with high or normal compliance is the common initial result. However, decreased compliance may also develop
** '''The classic outlet findings are described as a competent but nonrelaxing smooth sphincter and a striated sphincter that retains some fixed tone but is not under voluntary control'''. Closure pressures are decreased in both areas

==== Management ====
* '''Although generally correct, the correlation between somatic neurologic findings and urodynamic findings in suprasacral and sacral SCI patients is not exact''', especially in patients with paraplegia resulting from spinal cord lesions at column level T10 to L2.
* '''If bladder pressures are suitably low or if they can be sufficiently and safely lowered with nonsurgical or surgical management, the problem can be treated primarily as an emptying failure. CIC''' can then be continued as a safe and effective way of satisfying many of the goals of treatment. The role of additive '''antimuscarinic''' administration is supported in this patient population.
** '''2019 AUA Update on SCI: For indwelling catheters a suprapubic catheter is preferred over a urethral catheter as there is less risk of urethral erosion and epididymo-orchitis in men. However, there is no difference between the 2 catheters regarding risk of UTI, bladder stones or urinary incontinence from NDO.'''
** In the absence of bladder outlet obstruction there is currently no role for medication in treating urinary retention in these patients.
* '''Alternatively, sphincterotomy, urethral stenting, or intrasphincteric injection of onabotulinumtoxinA can be used in males to lower the detrusor leak point to an acceptable level and render the patient incontinent, thus converting the dysfunction primarily to a storage failure (incontinence),''' which can be obviated either by timed stimulation or with an external collecting device. In the dexterous SCI patient, the former approach using CIC is becoming predominant.
* '''Electrical stimulation of the anterior sacral roots with some form of deafferentation''' is also now a distinct reality. Although used sparingly, as with all patients with neurologic impairment, a careful initial evaluation and periodic, routine follow-up evaluation must be performed to identify and correct the following risk factors and potential complications: bladder overdistention, high-pressure storage, high detrusor leak point pressure, vesicoureteral reflux (VUR), stone formation (lower and upper tracts), and complicating infection, especially in association with reflux.
* Potential risk factors and complications are those previously described, with particular emphasis on storage pressure, which can result in silent upper tract decompensation and deterioration in the absence of VUR. The treatment of such a patient is usually directed toward producing or maintaining low-pressure storage while circumventing emptying failure with CIC when possible.
* Bowel management
** Approximately 60% of patients will have fecal incontinence and 40% will have constipation.
** The goal of a neurogenic bowel regimen is to accomplish complete evacuation of the rectum on a regular basis, thereby reducing the risk of fecal impaction. This goal is achieved with adequate fluid intake, balanced diet, appropriate physical activity and a regular bowel routine.
** In a systematic review of non-pharmacological therapies for chronic constipation, a transanal irrigation system appeared promising in decreasing constipation and fecal incontinence.
** If further therapy is needed, patients with SCI should be started on medications such as cisapride, prucalopride or neostigmine.

==== Autonomic hyperreflexia (dysreflexia) ====
* '''See [[CUA: Neurogenic Lower Urinary Tract Dysfunction (2019)|2019 CUA NLUTD Guideline Notes]]'''
*'''Potentially fatal emergency unique to the SCI patient'''
* Onset after injury is variable—usually soon after spinal shock, but it may occur up to years after injury, and distal spinal cord viability is a prerequisite.
* '''Represents an acute massive disordered autonomic (primarily sympathetic) response in patients with SCI above the cord level of T6-T8 (the sympathetic outflow) to specific stimuli below the level of the lesion'''
* '''Symptoms (5):'''
*# '''Pounding headache'''
*# '''Hypertension'''
*# '''Flushing and sweating above the level of the lesion'''
*# '''Bradycardia'''
*#* Tachycardia or arrhythmia may be present
*# '''Hypertension'''
*#* May vary in severity from causing a mild headache before voiding to life-threatening cerebral hemorrhage or seizure
* The pathophysiology is that of nociceptive stimulation via afferent impulses that ascend through the cord and elicit reflex motor outflow, causing arteriolar, pilomotor, and pelvic visceral spasm and sweating. Normally, the reflexes would be inhibited by secondary output from the medulla, but because of the SCI this does not occur below the lesion level.
* '''The stimuli for this exaggerated response commonly arise from the bladder or rectum and typically involve distention.''' '''Precipitation may be the result of simple LUT instrumentation, tube change, catheter obstruction, or clot retention, and in such cases the symptoms resolve quickly if the stimulus is withdrawn.'''
* '''Ideally, any endoscopic procedure in susceptible patients should be done using spinal anesthesia or carefully monitored general anesthesia'''.
* '''Acutely, the hemodynamic effects of this syndrome may be managed with β- and/or α-adrenergic blocking agents.'''
** '''Sublingual nifedipine is capable of alleviating this syndrome when given during cystoscopy (10 to 20 mg) and of preventing it when given orally 30 minutes before cystoscopy (10 mg)'''
** No consensus on the acute pharmacologic management of autonomic dysreflexia when necessary;

==== Vesicoureteral reflux ====
* '''Incidence between 17-25% in SCI patients, more common in those with suprasacral SCI.'''
** Persistent reflux can lead to chronic renal damage and may be an important factor in the long-term survival of SCI patients.
* '''Contributing factors include:'''
*# '''Elevated intravesical pressure during filling and emptying'''
*# '''Infection'''
* '''Management'''
** '''The best initial treatment for VUR in a patient with voiding dysfunction secondary to neurologic disease or injury is to normalize LUT urodynamics (i.e., decrease storage pressures and decrease outlet resistance) as much and as quickly as possible.'''
*** Depending on the clinical circumstances, this may be achieved by pharmacotherapy, urethral dilatation (in the myelomeningocele patient), neuromodulation, deafferentation, augmentation cystoplasty, or sphincterotomy

==== Urinary tract infection ====
* Relatively common in patients with SCI.
* '''Bacteriuria should be treated only when the patient has signs or symptoms of a UTI'''
* Risk factors for symptomatic UTIs in patients with SCI:
** Bladder overdistention
** Increased bladder filling pressures
** Vesicoureteral reflux
** Bladder stones

==== Sexual dysfunction in males ====
* After SCI most men have erectile and ejaculatory dysfunction.
** Rates of successful intercourse after SCI in men range from 5% to 75%, and only 10% with SCI can ejaculate.
* Erectile dysfunction in men can be managed using the same options available to the general population.
* '''Only 10% of men with SCI are able to achieve natural conception.''' Infertile men with SCI can achieve fertility with medical intervention that restores ejaculatory function and normal semen quality.

==== Spinal cord injury in women ====
* Management complicated by lack of an appropriate external collecting device
* Surgical intervention for SUI in this population may be beneficial in well-selected patients
* Fertility is not impaired in most women with SCI

==== Spinal cord injury and bladder cancer ====
* Strong association between the development of bladder cancer (squamous cell carcinoma) and long-term indwelling catheterization
** Some patients have no obvious tumors visible at endoscopy, and the diagnosis is made by bladder biopsy

==== Follow-up ====
* '''See [[CUA: Neurogenic Lower Urinary Tract Dysfunction (2019)|2019 CUA NLUTD Guideline Notes]]'''

=== Transverse Myelitis ===
* '''Acute transverse myelitis is a rapidly developing condition with motor, sensory, and sphincter abnormalities, usually with a well-defined upper sensory limit and no signs of spinal cord compression or other neurologic disease'''
* '''May result from a variety of mechanisms: parainfectious, autoimmune, vascular, or demyelinating'''
* '''Presentation'''
** Bladder dysfunction may occur simultaneously with the motor dysfunction or more commonly follows it
**'''Most common urinary symptom is urinary retention'''
***95% of children in the acute phase of the disease will have urinary retention.
** '''Most common urodynamic finding in the acute phase is areflexia or detrusor underactivity'''
** '''After resolution of spinal shock, the urodynamic patterns identifiable are detrusor overactivity''' (59-90%), decreased compliance (47%), DSD (17-80%), and detrusor leak point pressure > 40 cm H2O
** '''The condition usually stabilizes within 2-4 weeks and is not progressive afterward; however, recovery may be variable and some residual neurologic deficits are possible. Although recovery is more variable, and the prognosis, in general, is favorable, the development and nature of voiding dysfunction have been reported to be similar, level by level, to those of SCI'''

=== Neurospinal dysraphism ===

* See Pediatrics: Neuromuscular Disorders of the Lower Urinary Tract Chapter Notes
* '''LUT dysfunction occurs in 90% of patients.'''
* '''LUT dysfunction secondary to occult spinal dysraphism may not manifest in childhood,''' and such patients may be referred as adults for symptoms as commonplace as urinary incontinence or recurrent UTIs.
* '''The level(s) of the lesion correlate(s) poorly with urodynamic findings.'''
* '''The “typical” myelodysplastic patient shows an areflexic bladder with an open bladder neck.'''
** The bladder usually fills until the resting residual fixed external sphincter pressure is reached, and then leakage occurs.
** Stress incontinence may also occur owing to changes in intra-abdominal pressure.
** A small percentage (10-15%) of patients demonstrate DSD, but these individuals show normal bladder neck function that, if detrusor reflex activity is controlled, may be associated with urinary continence.
* The neurologic exam does not predict urodynamic behaviour
* '''Management'''
** Regardless of the pattern of LUT dysfunction in the adult, the main goal of therapy is the avoidance of high storage pressures
** The urologic rehabilitation of patients with spinal dysraphism relies primarily on medical management and intravesical injection of onabotulinumtoxinA, with the selective use of augmentation enterocystoplasty or urinary diversion if failure occurs.
** '''The treatment strategy in women is to increase urethral sphincter efficiency without causing an increase in urethral closing pressure significant enough to result in a change in bladder compliance.'''
*** Periurethral injection therapy may be a safer option than the pubovaginal sling and artificial urethral sphincter in this case.
** SUI in men with myelodysplasia may follow similar general rules as in women, and bulking agents may give good results in this group as well.
*** When the urethra is very widely dilated and somewhat rigid, and neither procedure alone will provide sufficient coaptation, it may be possible to combine a “prostatic sling” with periurethral bulking.
*** Continent individuals will remain on CIC.
* '''Tethered cord syndrome'''
** Defined as a stretch-induced functional disorder of the spinal cord with its caudal part anchored by inelastic structures and restricting vertical movement.
** Children often develop symptoms of tethered cord after growth spurts; in adults the presenting symptoms often follow activities that stretch the spine, such as sports or motor vehicle accidents
** '''Cord tethering can affect both bowel and leg function, as well as bladder function. Usually, there is no bladder dysfunction, and treatment must be based on urodynamic evaluation.'''
*** Despite efforts at improved radiographic visualization of the spinal cord, imaging does not correlate with physical findings or connote overall responsiveness to surgical intervention because detethering remains a critical aspect of management and control of tethered cord.
*** Urodynamics is improved by detethering, and this parallels functional improvement in those individuals who have undergone the surgical procedure
* Follow-up
** Includes annual surveillance for early identification of urinary tract deterioration.
*** These assessments should include renal and bladder ultrasonography and urodynamics when indicated (by symptomatic change or clinical physical examination finding).
*** In addition, serum creatinine and renal scintigraphy may be performed when upper tract changes are suspected.

=== Tabes dorasalis (late neurosyphilis) ===

* Syphilitic myelopathy is rapidly disappearing as a major neurologic problem
* Involvement of the spinal cord dorsal columns and posterior sacral roots '''can result in a loss of bladder sensation and large postvoid residual urine volumes''' and therefore can be a cause of “sensory neurogenic bladder”

=== Pernicious anemia ===

* Disease caused by impaired uptake of vitamin B12 resulting from the lack of intrinsic factor in the gastric mucosa.
* Now uncommon
* A spinal cord cause of the classic “sensory bladder” caused by subacute combined degeneration of the dorsolateral columns of the spinal cord

=== Poliomyelitis ===

* Although not always present, when voiding dysfunction is seen in patients with polio it is that of a typical “motor neurogenic bladder”, with urinary retention, detrusor areflexia, and intact sensation.