adulthood and older age. Critical for stability of function is recognition that the spinal cord may be tethered. Regarding the tethered spinal cord:

a.the level of tethering as demonstrated by MRI is predictive of findings on urodynamics.

b.urodynamics often shows improvement after detethering.

c.tethered cord syndrome is not usually associated with bowel or leg dysfunction.

d.there are no predictors for social continence after detethering.

e.the symptomatic tethered cord, associated with worsening ambulation, can be managed nonoperatively.

.Cauda equina syndrome often has effects on lower urinary tract function. Cauda equina syndrome:

a.occurs in the posterior lateral disk direction and is associated in a high degree of dysfunction in patients with cauda equina syndrome.

b.presents with most common neurologic findings of L3/L4 neurologic dysfunction.

c.is caused by disk protrusions occurring at the L5 S1 levels in the majority of cases.

d.is not usually associated with other etiologies other than disk disease.

e.is universally improved after laminectomy in regards to bladder function.

.Guillain-Barré syndrome is a demyelinating disorder. It is:

a.not usually identified as a clinical entity having sporadic clinical manifestation.

b.not caused by an acute inflammatory disorder.

c.not usually associated with an antecedent acute infectious illness.

d.associated with lower urinary tract function, usually in less than 5% of cases.

e.associated with voiding dysfunction, which is usually reversible.

Answers

1.b. Involuntary bladder contractions, smooth sphincter synergy, striated sphincter synergy. Neurologic lesions above the level of the brainstem that affect micturition generally result in involuntary bladder contractions with smooth and striated sphincter synergy. Sensation and voluntary striated sphincter function are generally preserved. Areflexia may

occur either initially or as a permanent dysfunction.

2.c. Involuntary bladder contractions, smooth sphincter synergy, striated sphincter dyssynergia. Patients with complete lesions of the spinal cord between spinal cord levels T6 and S2, after they recover from spinal shock, generally exhibit involuntary bladder contractions without sensation of the contraction, smooth sphincter synergy, but striated sphincter dyssynergia. Those with lesions above T6 may experience, in addition, smooth sphincter dyssynergia and autonomic hyperreflexia.

3.e. Detrusor overactivity. The most common long-term expression of lower urinary tract dysfunction after a CVA is detrusor hyperreflexia.

Sensation is variable but is classically described as generally intact, and thus the patient has urgency and frequency with hyperreflexia.

4.a. Internal capsule. Previous descriptions of voiding dysfunction after a CVA have all cited the preponderance of detrusor hyperreflexia with coordinated sphincter activity. It is difficult to reconcile this with the relatively high incontinence rate that occurs, even considering the probability that a percentage of these patients had an incontinence problem before the CVA. Tsuchida and colleagues (1983)* and Khan and colleagues (1990) made early significant contributions in this area by correlating the urodynamic and computed tomography (CT) pictures after CVA. They reported that patients with lesions in only the basal ganglia or thalamus have normal sphincter function. This means that when an impending involuntary contraction or its onset was sensed, these patients could voluntarily contract the striated sphincter and abort or considerably lessen the effect of an abnormal micturition reflex. The majority of patients with involvement of the cerebral cortex and/or internal capsule were unable to forcefully contract the striated sphincter under these circumstances.

5.d. Pseudodyssynergia. Some authors have described striated sphincter dyssynergia in 5% to 21% of patients with brain disease and voiding dysfunction. This is incompatible with accepted neural circuitry. The authors agree with those who believe that true detrusor-striated sphincter dyssynergia does not occur in this situation. Pseudodyssynergia may indeed occur during urodynamic testing of these patients. This refers to an EMG sphincter "flare" during filling cystometry, which is secondary to attempted inhibition of an involuntary bladder contraction by voluntary contraction of the striated sphincter.

6.e. Full urodynamic evaluation. Poor flow rates and high residual urine

volumes in a male with pre-CVA symptoms of prostatism generally indicate prostatic obstruction, but a full urodynamic evaluation is advisable before committing a patient to mechanical outlet reduction primarily to exclude detrusor hyperactivity with impaired contractility as a cause of symptoms.

7.c. Posterior fossa. The areas that are most frequently involved with associated micturition dysfunction are the superior aspects of the frontal lobe. When voiding dysfunction occurs, it generally consists of detrusor hyperreflexia and urinary incontinence. These individuals may have a markedly diminished awareness of all lower urinary tract events and, if so, are totally unable to even attempt suppression of the micturition reflex. Smooth and striated sphincter activity is generally synergic. Pseudodyssynergia may occur during urodynamic testing. Fowler (1999) reviewed the literature on frontal lobe lesions and bladder control. She cited instances of resection of a tumor relieving the micturition symptoms for a period of time, raising the question of whether the phenomenon of tumorassociated bladder hyperreflexia was a positive one (activating some system) rather than a negative one (releasing a system from control). Urinary retention has also been described in patients with space-occupying lesions of the frontal cortex, in the absence of other associated remarkable neurologic deficits. Posterior fossa tumors may be associated with voiding dysfunction (32% to 70%, based on references cited by Fowler). Retention or difficulty voiding is the rule, with incontinence rarely reported.

8.b. Normal filling/storage; normal emptying. Most children and adults with only CP have urinary control and what seems to be normal filling/storage and normal emptying. The actual incidence of voiding dysfunction is somewhat vague because the few available series report findings predominantly in those who present with voiding symptoms. One study estimated that a third or more of children with CP are so affected. When an adult with CP presents with an acute or subacute change in voiding status, however, it is most likely unrelated to CP.

9.e. Detrusor overactivity, coordinated sphincters. In those individuals with CP who exhibit significant dysfunction, the type of damage that one would suspect from the most common urodynamic abnormalities seems to be localized above the brainstem. This is commonly reflected by detrusor overactivity and coordinated sphincters. Spinal cord damage can occur, however, and probably accounts for those individuals with CP who seem to have evidence of striated sphincter dyssynergia.

.a. Dopamine. PD is a neurodegenerative disorder of unknown cause that affects primarily the dopaminergic neurons of the substantia nigra but also heterogeneous populations of neurons elsewhere. The most important site of pathology is the substantia nigra pars compacta, the origin of the dopaminergic nigrostriatal tract to the caudate nucleus and putamen. Dopamine deficiency in the nigrostriatal pathway accounts for most of the

classic clinical motor features of PD.

.d. Detrusor overactivity. The most common urodynamic finding is detrusor overactivity. The pathophysiology of detrusor overactivity most widely proposed is that the basal ganglia normally have an inhibitory effect on the

micturition reflex, which is abolished by the cell loss in the substantia nigra.

.e. Disease diagnosis preceding voiding and erectile symptoms. One study compared the clinical features of 52 patients with probable MSA and 41 patients with PD. Of patients with MSA, 60% had their urinary symptoms precede or present with their symptoms of parkinsonism. Of patients with PD, 94% had been diagnosed for several years before the onset of urinary symptoms. In patients with MSA, urinary incontinence was a significant complaint in 73%, whereas 19% had only frequency and urgency without incontinence. Sixty-six percent of the patients with MSA had a significant postvoid residual volume (100 to 450 mL). In patients with PD, frequency and urgency were the predominant symptoms in 85% and incontinence was the primary complaint in 15%. It was significant in only 5 of 32 patients with PD in whom residual urine volume was measured. Ninetythree percent of the men with MSA who were questioned about erectile function reported erectile failure, and in 13 of 27 the erectile dysfunction preceded the diagnosis of MSA. Seven of the 21 men with PD had erectile failure, but in all of these men the diagnosis of erectile dysfunction followed the diagnosis of PD by 1 to 4 years. The initial urinary symptoms of MSA are urgency, frequency, and urge incontinence, which occur as long as 4 years before the diagnosis is made, as does erectile failure. Cystourethrography or video-urodynamic studies generally reveal an open bladder neck (intrinsic sphincter deficiency), and many patients exhibit evidence of striated sphincter denervation on motor unit electromyography. The smooth and striated sphincter abnormalities predispose women to sphincteric incontinence and

make prostatectomy hazardous in men.

.c. Cervical spinal cord. The demyelinating process most commonly involves the lateral corticospinal (pyramidal) and reticulospinal columns of the cervical

spinal cord.

.e. Smooth sphincter dyssynergia. Detrusor overactivity is the most common urodynamic abnormality detected, occurring in 34% to 99% of cases in reported series. Of the patients with overactivity, 30% to 65% have coexistent striated sphincter dyssynergia. As many as 60% of those with overactivity may have impaired detrusor contractility, a phenomenon that can considerably complicate treatment efforts. Bladder areflexia may also occur; reports of its frequency vary but generally average from 5% to 20%. Generally, the smooth

sphincter is synergic.

.d. Spinal cord injury (SCI). Progressive neurologic diseases cause upper tract damage much less commonly than SCI, even when associated with severe disability and spasticity (Wyndaele et al, 2005).

.a. 15% or less. How common are voiding problems overall in patients with HIV infection and AIDS? One study prospectively investigated voiding function in 77 men and 4 women with HIV infection or AIDS consecutively attending an outpatient clinic. Eight patients (10%) had moderate subjective voiding problems, whereas two (2%) had severe problems. The authors thought that the nature of the disturbance warranted urodynamic examination in only 4% of patients and concluded that urinary voiding symptoms are only a modest problem; overall in an HIV/AIDS population, neuropathic bladder dysfunction is rare and mostly occurs in the late stages of the disease (Gyrtrup et al, 1995).

.c. L2. Spinal column (bone) segments are numbered by the vertebral level, and these have a different relationship to the spinal cord segmental level at different locations. The sacral spinal cord begins at approximately spinal column level T12-L1. The spinal cord terminates in the cauda equina at approximately the spinal column level of L2.

.c. Open bladder neck. Spinal shock includes a suppression of autonomic activity and somatic activity, and the bladder is acontractile and areflexic.

Radiologically, the bladder has a smooth contour with no evidence of trabeculation. The bladder neck is generally closed and competent unless there has been prior surgery or, in some cases, thoracolumbar and presumably sympathetic injury. The smooth sphincter mechanism seems to be functional. Some EMG activity may be recorded from the striated sphincter, and the maximum urethral closure pressure is lower than normal but still maintained at the level of the external sphincter zone; however, the normal guarding reflex is absent and there is no voluntary control.

.d. High abdominal leak-point pressure. As with all patients with neurologic impairment, a careful initial evaluation and periodic 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, stone formation (lower and upper tracts), and complicating infection, especially in association with reflux.

.a. Pons and the sacral spinal cord. A diagnosis of striated sphincter dyssynergia implies a neurologic lesion that interrupts the neural axis

between the pontine-mesencephalic reticular formation and the sacral spinal cord.

.a. Cervical. Autonomic hyperreflexia represents an acute massive disordered autonomic (primarily sympathetic) response to specific stimuli in patients

with SCI above the level of T6 to T8 (the upper level of the sympathetic outflow). It is more common with cervical (60%) than thoracic (20%) injuries.

.d. Tachycardia. Symptomatically, autonomic hyperreflexia is a syndrome of exaggerated sympathetic activity in response to stimuli below the level of the lesion. The symptoms are pounding headache, hypertension, and flushing of the face and body above the level of the lesion with sweating. Bradycardia is a usual accompaniment, and an arrhythmia may be present.

.b. Decreased compliance. In autonomic hyperreflexia, the urodynamic picture is that of a suprasacral SCI. Smooth sphincter dyssynergia is generally found as well, at least in men.

.b. β-Adrenergic blockade. Acutely the hemodynamic effects may be managed with parenteral ganglionic or α-adrenergic blockade. Any endoscopic procedure in susceptible patients ideally should be done with the patient under spinal or carefully monitored general anesthesia.

.a. Ureteral reimplantation. The best initial treatment for reflux in a patient with voiding dysfunction secondary to neurologic disease or injury is to normalize lower urinary tract urodynamics as much as possible. Depending on the clinical circumstances, this may be by pharmacotherapy, urethral dilatation (in the myelomeningocele patient), neuromodulation, deafferentation, augmentation cystoplasty, or sphincterotomy. If this fails, the question of whether to operate on such patients for correction of the reflux or to correct the reflux while performing another procedure (e.g., augmentation cystoplasty) is not an easy one because correction of reflux in an often thickened bladder may not be an easy task.

.e. Neurologic evaluation initially and yearly for an indefinite period. All but e were specific recommendations (Linsenmeyer and Culkin, 1999).

.e. Maximum bladder capacity decreased. One must remember the potential artifact that significant reflux can introduce into urodynamic studies.

Measured bladder capacity may be more, and measured pressures at given inflow volumes may be less, than those after reflux correction. The

apparent significance of detrusor overactivity may thus be underestimated.

.e. Growth spurt. One study pointed out that although 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.

.d. Pernicious anemia. Although syphilitic myopathy is 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 residual urine volumes and therefore can be a cause of sensory neurogenic bladder. Another spinal cord cause of the classic sensory bladder is the now uncommon pernicious anemia. The most common cause is diabetes.

.d. Difficulty voiding; normal bladder compliance. A study reported on findings in 114 patients with lumbar disk protrusion who were prospectively studied. The authors found detrusor areflexia in 31 (27.2%) and normal detrusor activity in the remaining 83. All 31 patients with detrusor areflexia reported difficulty voiding with straining. Patients with voiding dysfunction generally present with these symptoms or in urinary retention. The most consistent urodynamic finding is that of a normally compliant areflexic bladder associated with normal innervation or findings of incomplete denervation of the perineal floor musculature.

.a. Exertional (or stress) incontinence; detrusor areflexia. When permanent voiding dysfunction occurs after radical pelvic surgery, the pattern is generally one of a failure of voluntary bladder contraction, or impaired bladder contractility, with obstruction by what seems urodynamically to be residual fixed striated sphincter tone, which is not subject to voluntarily induced relaxation. Often, the smooth sphincter area is open and nonfunctional. Decreased compliance is common in these patients, and this, with the "obstruction" caused by fixed residual striated sphincter tone, results in both storage and emptying failure. These patients often experience leaking across the distal sphincter area and, in addition, are unable to empty the bladder because although intravesical pressure may be

increased, there is nothing that approximates a true bladder contraction. The patient often presents with urinary incontinence that is characteristically most manifest with increases in intra-abdominal pressure. This is usually most obvious in females because the prostatic bulk in males often masks an equivalent deficit in urethral closure function. Alternatively, patients may present with variable degrees of urinary retention.

.b. Clean intermittent catheterization. The temptation to perform a prostatectomy should be avoided unless a clear demonstration of outlet obstruction at this level is possible. Otherwise, prostatectomy simply decreases urethral sphincter function and thereby may result in the occurrence or worsening of sphincteric urinary incontinence. Most of these dysfunctions will be transient, and the temptation to "do something" other than perform

clean intermittent catheterization initially after surgery in these patients, especially in those with little or no preexisting history of voiding dysfunction, cannot be too strongly discouraged.

.e. Detrusor pressure. Detrusor contractility is classically described as being decreased in the end-stage diabetic bladder. Current evidence points to both sensory and motor neuropathy as being involved in the pathogenesis, the motor aspect per se contributing to the impaired detrusor contractility. The typically described classic urodynamic findings include impaired bladder sensation, increased cystometric capacity, decreased bladder contractility, impaired uroflow, and, later, increased residual urine volume. The main differential diagnosis, at least in men, is generally bladder outlet obstruction because both conditions commonly produce a low flow rate. Pressure/flow urodynamic studies easily differentiate the two.

.c. Stroke. True detrusor sphincter dyssynergia should exist only in patients who have an abnormality in pathways between the sacral spinal cord and the

brainstem pontine micturition center, generally due to neurologic injury or disease.

.e. Video-urodynamic study. Objective evidence of outlet obstruction in these patients is easily obtainable by urodynamic study. Once obstruction is diagnosed, it can be localized at the level of the bladder neck by videourodynamic study, cystourethrography during a bladder contraction, or micturitional urethral profilometry.

.c. Bladder capacity of less than 1 L. The criteria (Swinn and Fowler, 2001; Fowler, 2003) include a bladder capacity of more than 1 L with no sensation of urgency.

.a. Striated sphincter needle EMG recording. Fowler syndrome refers particularly to a syndrome of urinary retention in young women in the absence of overt neurologic disease. The typical history is that of a woman younger than 30 years, who has found herself unable to void during the preceding day but with no sensation of urgency. MRI studies of the brain and the entire spinal cord are normal. On concentric needle electrode examination of the striated muscle of the urethral sphincter, however, Fowler and colleagues described a unique EMG abnormality. This abnormal activity, localized to the urethral sphincter, consists of a type of activity that would be expected to cause inappropriate contraction of the muscle. Sphincter activity consists of

two components: complex repetitive discharges and decelerating bursts. This abnormal activity impairs sphincter relaxation.

.e. Neuromodulation. Fowler reports that efforts to treat this condition by hormonal manipulation, pharmacologic therapy, or injections of botulinum toxin have been unsuccessful. This condition is highly responsive to neuromodulation, even in women who have had retention for many months or years.

.b. Myasthenia gravis. Any neuromuscular disease that affects the tone of the smooth or striated muscle of the distal sphincter mechanism can predispose an individual patient to a greater chance of urinary incontinence even after a well-performed transurethral or open prostatectomy. Myasthenia gravis is an autoimmune disease caused by autoantibodies to acetylcholine nicotinic receptors. This leads to neuromuscular blockade and hence weakness in a variety of striated muscle groups. The incidence of incontinence after prostatectomy is indeed greatly increased in patients with this disease.

.e. All of the above. Traumatic brain injury has now risen to the most common etiologic factor for neurologic voiding dysfunction. Depending on the level of

the injury, there may be detrusor sphincter synergia or dyssynergia. In recent studies, urinary incontinence, on a chronic basis associated with this injury, is associated with poor functional status and bilaterality of lesion. Patients who are in chronic retention are more commonly noted to have diabetes mellitus or bowel-related issues as comorbidities. Rehabilitation results in some improvement for patients, but it depends on the magnitude of injury.

.b. Symptoms such as tremor, skeletal rigidity, and bradykinesia occur.

The symptom complex of PD is classic for its presentation, and these symptoms are often followed longitudinally for benefit of therapy. The diagnosis of PD is classically made by neuropathologic examination;

however, there is a constellation of symptoms that is consistent with the syndrome and leads to therapeutic intervention during life. Positron emission tomography (PET) scanning shows brain responses with bladder filling. These responses are found most prominently with detrusor overactivity. The cerebellum seems to be actively involved in these increased responses. Dopaminergic agonists are used therapeutically for the management of this disorder.

.b. Urodynamics often show improvement after detethering. 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. Cord tethering affects both bowel and leg function, as

well as bladder function. Urodynamics is improved by detethering, and this parallels functional improvement in those individuals who have undergone the surgical procedure. The successful management of the tethered cord remains a critical aspect of long-term care in the myelomeningocele population.

.c. Is caused by disk protrusions occurring at the L5 S1 levels in the majority of cases. Cauda equina is most commonly associated with low lumbar, high sacral disk lesions that compress the nerve roots while they ramify from the spinal cord. It can be caused by a variety of etiologies other than disk disease, which may or may not affect long-term function after laminectomy. Although urologic findings associated with cauda equina are often indications for surgery, laminectomy may not improve overall function. Posterolateral disk disease does not usually result in cauda equina syndrome.

.e. Associated with voiding dysfunction, which is usually reversible.

Guillain-Barré syndrome is an acute inflammatory disorder thought to be due to some inflammatory process because most patients do have evidence of acute inflammatory conditions before development of the syndrome. The syndrome is characterized by a distinct presenting scenario (ascending paralysis). Lower urinary tract dysfunction has been reported in as few as 25% to as many as 80% of cases and is usually reversible with resolution of the disorder.

Chapter review

1.Neurologic lesions above the brainstem generally result in involuntary bladder contractions and a coordinated sphincter.

2.Spinal cord lesions between T6 and S2 result in involuntary bladder

contractions, smooth sphincter synergy, and striated sphincter dyssynergia; spinal cord lesions above T7 or T8 may have smooth sphincter dyssynergia as well.

3.Lesions below S2 result in areflexia.

4.Traumatic brain injury results in an initial period of detrusor areflexia.

5.Parkinson disease commonly results in detrusor overactivity with possible impaired detrusor contractility.

6.For a true Parkinson disease patient, a TURP is not contraindicated. However, in multisystem atrophy with Parkinson-like symptoms, smooth and striated sphincter abnormalities may result in incontinence after a TURP.

7.Spinal shock includes a suppression of autonomic activity and somatic activity, and the bladder is acontractile and areflexic. The bladder neck is generally closed.

8.Autonomic hyperreflexia is primarily a sympathetic response and occurs in patients with SCI above the level of T6; it is a syndrome of exaggerated sympathetic activity in response to stimuli below the level of the lesion. The symptoms are pounding headache, hypertension, and flushing of the face and body above the level of the lesion with sweating. Bradycardia is a usual accompaniment, and an arrhythmia may be present. Drugs commonly used to treat the manifestations include nifedipine, nitrates, and captopril.

9.In patients with SCI, bacteriuria should only be treated when there are signs or symptoms.

10.In SCI patients the incidence of bladder cancer is comparable with the general population; however, 60% of those affected present with T2 or greater disease versus 20% in the general population.

11.Tethered cord syndrome results from fixation of the spinal cord caudally with stretch as the individual grows. Presenting symptoms are back pain, leg weakness, foot deformities, scoliosis, sensory loss, and bowel and lower urinary tract dysfunction.

12.Radical pelvic surgery may result in failure of voluntary bladder contraction and residual fixed striated sphincter tone, which is not subject to voluntary control.

13.Diabetic cystopathy is a result of peripheral and autonomic neuropathy that affects both sensory and motor nerves. It is primarily a problem of overdistention, and therefore timed voidings may be helpful.

14.True detrusor sphincter dyssynergia occurs when pathways between the sacral spinal cord and the brainstem are interrupted. Therefore traumatic SCI, multiple sclerosis, and transverse myelitis are the usual causes. In patients without a neurologic lesion, the diagnosis should be suspect.

15.Radiation results in decreased cystometric capacity, decreased compliance, and a reduction in the volume at which the first urge to void is noted.

16.In patients with multiple sclerosis or voiding dysfunction secondary to radical pelvic surgery, conservative measures are preferred and irreversible treatments should be avoided.

17.Urinary retention may be secondary to herpes zoster and herpes simplex.

18.Syphilitic myopathy, pernicious anemia, and diabetes may cause the classic sensory neurogenic bladder; poliomyelitis may cause the classic motor neurogenic bladder.

19.The most common long-term expression of lower urinary tract dysfunction after a CVA is detrusor hyperreflexia.

20.Pseudodyssynergia refers to an EMG sphincter "flare" during filling cystometry, which is secondary to attempted inhibition of an involuntary bladder contraction by voluntary contraction of the striated sphincter.

21.Most children and adults with only cerebral palsy have urinary control and what seems to be normal filling/storage and normal emptying.

22.The sacral spinal cord begins at approximately spinal column level T12L1. The spinal cord terminates in the cauda equina at approximately the spinal column level of L2.

23.Measured bladder capacity may be more, and measured pressures at given inflow volumes may be less than those after reflux correction.

* Sources referenced can be found in Campbell-Walsh Urology, 11th Edition, on the Expert Consult website.