Neuromuscular Dysfunction

of the Lower Urinary Tract

abnormalities of the micturition cycle produced by different
types of neuromuscular disease, injury, or dysfunction. The source
material for the central and peripheral factors involved in the
physiology and pharmacology of lower urinary tract function. The
specific types of voiding dysfunction that occur secondary to the
most common categories of neuromuscular disease, injury, or
dysfunction. The expected states of the following parameters should
be described :detrusor activity, detrusor compliance, smooth
sphincter activity, striated sphincter activity, sensation.
GENERAL PATTERNS OF NEUROPATHIC LOWER URINARY TRACT
DYSFUNCTION
Discrete neurologic lesions generally affect the filling
and
emptying phases of lower urinary tract function in relatively
consistent. This lesion is dependent on the area of the nervous
system affected, the physiologic function and the location of the
area affected, and whether the lesion or process is destructive,
inflammatory, or irritative.
Plasticity
The plasticity of the nervous system refers to the inherent
capacity to undergo structural and functional modification. the
neurologic hanges can then be reflected on a number of structural,
metabolic and neurologic. In addition, neurologic can changes by
the number of morphologic, neurochemical, electrical and
organizational. plasticity may account for the persistence of clinical
symptoms after the initial stimulus for dysfunction has been
removed or corrected.
DISEASE AT OR ABOVE THE BRAINSTEM
Cerebrovascular Disease
1. Cerebrovascular Accident (Stroke)
Cerebrovascular accident (CVA) is a common cause of death
and one of the most common causes of disability. Thrombosis,
occlusion, and hemorrhage are the most common causes of stroke,
leading to ischemia and infarction of variably sized areas in the
brain, usually around the internal capsule.
After the initial acute episode, urinary retention from detrusor
areflexia may occur. After a variable degree of recovery from the
neurologic lesion, a fixed deficit may become apparent over a few
weeks or months. The most common long-term expression of lower
urinary tract dysfunction after CVA is phasic detrusor overactivity.
Sensation is variable but is classically described as generally intact,
and thus the patient has urgency and frequency with detrusor

overactivity. The appropriate response is to try to inhibit the

involuntary bladder contraction by forceful voluntary contraction of
the striated sphincter.
Tsuchida and colleagues (1983) and Khan and colleagues
(1990) reported that patients with lesions in only the basal ganglia
or thalamus have normal sphincter function. The majority of
patients with involvement of the cerebral cortex or internal capsule
or both were unable to forcefully contract the striated
Sphincter. Griffiths (1998), studying the results of True urgency
incontinence with reduced bladder sensation was associated with
global underperfusion of the cerebral cortex, especially the frontal
areas, especially on the right.
There are two possible mechanisms for the incontinence
associated with involuntary bladder contractions in patients who
have sustained a CVA: (1) impaired striated sphincter control and (2)
lack of appreciation of bladder filling and impending bladder
contraction.
2. Brainstem stroke
Sakakibara and colleagues (1996b) studied 39 patients with
brainstem stroke. Nineteen of these had voiding symptoms. The
major problems were nocturnal frequency and voiding difficulty in
six, urinary retention in eight, and urinary incontinence in three. The
authors concluded that lesions of the dorsolateral pons involving the
pontine reticular nucleus, reticular formation, and the locus
coeruleus were mainly responsible for the micturition disturbances
in patients with brainstem lesions.
3. Dementia
Dementia is a poorly understood disease complex involving
atrophy and the loss of gray and white matter of the brain,
particularly of the frontal lobes. Problems result with memory and
the performance of tasks requiring intellectual mentation. When
voiding dysfunction occurs like incontinence, it is difficult to make it
diference between patophysiology and consideration are similar to
those in the stroke patient whether the inconetinence reflects a
situation in which the individual has simply lost the awareness of
voluntary urinary control.
4. Traumatic Brain Injury
Traumatic brain injury has been cited as the most common
form of severe neurologic impairment as a result of trauma. In
patients who have more isolated brainstem injuries with
involvement below the pontine micturition center, detrusor striated
sphincter dyssynergia may occur in addition. Urinary incontinence
was associated with poor functional status and bilateral lesions.
5. Brain Tumor
Both primary and metastatic brain tumors have been reported

to be associated with disturbances of bladder function. When

dysfunction results, it is related to the localized area involved rather
than to the tumor type. 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 overactivity and urinary incontinence. Urinary
retention has also been described in patients with space-occupying
lesions of the frontal cortex.

6. Cerebellar ataxia
This group of diseases involves pathologic degeneration of the
nervous system generally involving the cerebellum, but with a
possible extension to the brainstem, spinal cord, and dorsal nerve
roots. Poor coordination, depressed deep tendon reflexes,
dysarthria, dysmetria, and choreiform movements result because of
the cerebellar involvement. Voiding dysfunction is generally
manifested by incontinence, usually associated with detrusor
overactivity and sphincter synergia. Retention or high residual urine
volume may occur as well. Poor emptying, when present, is most
commonly caused by detrusor areflexia, but it may be associated
with detrusorstriated sphincter dyssynergia, presumably secondary
to spinal cord involvement.
7. Normal-pressure hydrocephalus
This is a disease of progressive dementia and ataxia
occurring in patients with normal cerebrospinal fluid pressure and
distended cerebral ventricles but with no passage of air over the
cerebral convexities by pneumoencephalography. When voiding
dysfunction occurs, it is generally incontinence secondary to
detrusor overactivity with sphincter synergia.
8. Cerebral Palsy
Cerebral palsy (CP) is the name applied to a nonprogressive
injury of the brain in the prenatal or perinatal or postnatal period.
That produces neuromuscular disability and/or specific symptom
complexes of cerebral dysfunction. The etiology is generally
infection or a period of hypoxia. Affected children exhibit delayed
gross motor development, abnormal motor performance, altered
muscle tone, abnormal posture, and exaggerated reflexes. Most
children and adults with only CP have urinary control and what
seems to be normal filling/storage and normal emptying.
9. Parkinson Disease

Parkinson disease (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. Dopamine deficiency in the nigrostriatal pathway
accounts for most of the classic clinical motor features of PD.
Voiding dysfunction occurs in 35% to 70% of patients with PD. When
voiding dysfunction occurs, symptoms generally (50% to 75%)
consist of urgency, frequency, nocturia, and urge incontinence. The
most common urodynamic finding is detrusor overactivity. The
pathophysiology of detrusor overactivity 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. Whether the
dopamine D1 or D2 receptor (or both) are primarily responsible does
not seem to have been settled as yet. It has been suggested that
loss of inhibitory D1-like receptors causes detrusor overactivity,
allowing D2 receptors to facilitate micturition.
10. Multiple System Atrophy
MSA is a progressive neurodegenerative disease of unknown
etiology. The neurologic lesions of MSA consist of cell loss and gliosis
in widespread areas, much more so than with PD, and this more
diffuse nature of cell loss probably explains why bladder symptoms
may occur earlier than in PD and be more severe and why erection
may be affected as well. The initial urinary symptoms of MSA are
urgency, frequency, and urgency incontinence, occurring up to 4
years before the diagnosis is made, as does erectile failure.
Detrusor overactivity is frequently found, as one would expect
from the central nervous system areas affected, but decreased
compliance may occur, reflecting distal spinal involvement of the
locations of the cell bodies of autonomic neurons innervating the
lower urinary tract. As the disease progresses, difficulty in initiating
and maintaining voiding may occur, probably from pontine and
sacral cord lesions, and this is generally associated with a poor
prognosis.
Drug treatment for sphincteric incontinence in this patient
may further worsen emptying problems. Generally, the goal in these
patients is to facilitate storage.
DISEASES PRIMARILY INVOLVING THE SPINAL CORD
1. Multiple Sclerosis
Multiple sclerosis (MS) is primarily a disease of young and
middleaged adults with a twofold predilection for women. The
disease is believed to be immune mediated and is characterized by
neural demyelination, generally characterized by axonal sparing, in
the brain and spinal cord. The demyelinating process most
commonly involves the lateral corticospinal (pyramidal) and
reticulospinal columns of the cervical spinal cord, and it is thus not

surprising that voiding dysfunction and sphincter dysfunction are so

common. Pseudodyssynergia from true striated sphincter
dyssynergia can be find in this patient.
The most important parameters predisposing patients with MS
to significant urologic complications: (1) striated sphincter
dyssynergia in men; (2) high detrusor filling pressure (>40 cm H2O);
and (3) an indwelling catheter.
2. Spinal Cord Injury
SCI may occur as a consequence of acts of violence, fracture,
or dislocation of the spinal column secondary to motor vehicle,
diving accidents or falls, vascular injuries or repair, infection, disk
prolapse, or sudden or severe hyperextension from other causes.
Altered lower urinary tract and sexual function frequently occur
secondary to SCI and significantly affect quality of life.
Impaired mobility is commonly noted in the spinal cordinjured
patient and may substantially affect urinary habit and incontinence.
Urologic phenomena also figure prominently in chronic SCI. Within
10 years after SCI, approximately 7% of patients with spinal cord will
develop an initial kidney stone with the greatest risk occurring
during the first 3 months after injury. Ninety-eight percent of these
stones will be apatite or struvite in composition.
a. Spinal Shock
After a significant SCI, a period of decreased excitability of
spinal cord segments at and below the level of the lesion occurs,
referred to as spinal shock. There is absent somatic reflex activity
and flaccid muscle paralysis below this level. In this patient can be
found that 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 (striated sphincter response during filling) is
absent and there is no voluntary control.
b. Suprasacral Spinal Cord Injury
In this patient we can find from Neurologic examination that shows
spasticity of skeletal muscle distal to the lesion, hyperreflexic deep
tendon reflexes, and abnormal plantar responses. There is
impairment of superficial and deep sensation
The characteristic pattern in this patient is when a patient has
a complete lesion above the sacral spinal cord is detrusor
overactivity, smooth sphincter synergia (with lesions below the
sympathetic outflow), and striated sphincter dyssynergia.
c. Sacral Spinal Cord Injury
After the patient recovers from spinal shock, there is generally
a depression of deep tendon reflexes below the level of complete
lesion with varying degrees of flaccid paralysis. Sensation is

generally absent below the lesion level. Detrusor areflexia with high
or normal compliance is the common initial result, but decreased
compliance may develop.
d. Neurologic and Urodynamic Correlation
Bladder function differs according to the level of injury. It is
important o define the neurological lesion to appreciate the voiding
dysfunction and thereby to develop an appropriate management
plan for long-term urologic care. Neurologic examination may be
incorrect because of the superimposed complxity of multiple injury
levels. Despite consistent data regarding classic voiding dysfunction with
completeinjuries,multiplicityofinjurymaycontribute
tocomplicatedurodynamicfindings.Therefore,urodynamicevaluationiscrucial
tocorrectlyidentifythetypeofvoidingdysfunctionandtooptimizelongterm
management.

e. AutonomicHyperreflexia
autonomichyperreflexiaisasyndromeofexaggeratedsympatheticactivity
inresponsetostimulibelowthelevelofthelesion.Thesymptomsarepounding
headache,hypertension,andflushingofthefaceandbodyabovethelevelofthe
lesionwithsweating.Bradycardiaisausualaccompaniment,althoughtachycardia
orarrhythmiamaybepresent.Hypertensionmaybeofvaryingseverity.
Autonomic hyperreflexia represents an acute massive disordered
autonomic (primarily sympathetic) response to specific stimuli in
patients with SCI above the cord level of T6 to T8 (the sympathetic
outflow)
Thestimuliforthisexaggeratedresponsecommonlyarisefromthebladder
orrectumandgenerallyinvolvedistentionalthoughotherstimulifromtheseareas
canbeprecipitating.Precipitationmaybetheresultofsimplelowerurinarytract
instrumentation,tubechange,catheterobstruction,orclotretentionand,insuch
cases,thesymptomsresolvequicklyifthestimulusiswithdrawn.Othercausesor
exacerbatingfactorsmayincludeotherupperorlowerurinarytractpathology.
(e.g.,calculi),gastrointestinalpathology,longbonefracture,sexualactivity,
electrocoagulation,anddecubiti.Striatedsphincterdyssynergiainvariablyoccurs,
andsmoothsphincterdyssynergiaisgenerallyapartofthesyndromeaswell,at
leastinmales.Thepathophysiologyisthatofanociceptivestimulationvia
afferentimpulsesthatascendthroughthecordandelicitreflexmotoroutflow,
causingarteriolar,pilomotor,andpelvicvisceralspasmandsweating.
f. VesicoureteralReflux
LongtimesurvivalofSpinalcordinjuryusuallyhavepersistentreflux
thatcanleadtochronicrenaldamage.Thebestinitialtreatmentforrefluxina
patientwithvoidingdysfunctionsecondarytoneurologicdiseaseorinjuryisto
normalizelowerurinarytracturodynamicsasmuchaspossible.Dependingonthe
clinicalcircumstances,thismaybebypharmacotherapy,urethraldilatation(inthe
myelomeningocelepatient),neuromodulation,deafferentiation,augmentation
cystoplasty,sphincterotomy.

g. UrinaryTractInfection
Urinarytractinfection(UTI)isrelativelycommoninpatientswithSCI.
FiftysevenpercentofpatientswithSCIexperienceUTIorbacteriuriainthefirst
yearafterinitialhospitalization.Recurrentinfectionsmaybeamanifestationof
upperorlowertractcalculi,symptomaticorsilentpyelonephritis,orlowerurinary
tractdysfunctioncausingpersistentresidualurine.UTIcanleadtohighmorbidity,
poorqualityoflife,anddecreasedlifeexpectancyinpatientswithSCI.
TotreatUTI,BieringSorensenandcolleagues(2001)recommendedto:
(1)treatbacteriuriaonlyifsymptomatic;(2)useantimicrobialagents,ifpossible,
with little or no impact on normal flora; (3) treat at least 5 days; those with
reinfectionorrelapse,treat7to14days;(4)repairstructuralandfunctionalrisk
factors;(5)useprophylaxisonlyinthosewithrecurrentUTIwhennounderlying
causecanbefoundandespeciallyiftheuppertractsaredilated;(6)donotuse
antibioticstopreventUTIinpatientswithanindwellingcatheter.
h. SpinalCordInjuryinWomen
Therearemanyaspectsofmanagementofthelowerurinarytractaffected
bySCIthatarespecifictowomen.Thesymptomsofmenopause(e.g.,hotflashes)
maybedifficulttodistinguishfromthoseofautonomicdysreflexia.Incontinence
and UTI become worse with age in women in the general population and
particularlyinthosewithSCI.
AfewquadriplegicwomencanbetrainedtoselfCIC, for the majority
there is no practical alternative to indwelling catheterization, but female SCI
patientwithCICwithlongtermmanagementhavecomplication:reflexvoiding
and incontinence padding and indwellingcatheter. The authors notedalso that
55%ofthewomenwithpermanentcathetershadbladdercalculi.
i. SpinalCordInjuryandBladderCancer
Thedevelopmentofcarcinomaofthebladderin6of59patientswith
SCIs who had longterm indwelling catheters was reported by Kaufman and
colleagues.Allweresquamouscelllesions.Fourofthesepatientshadnoobvious
tumorsvisibleatendoscopy,andthediagnosiswasmadebybladderbiopsy.
Urodynamic evaluation was recommended by the APS at the same
intervals as upper and lower tract screening. Cystoscopy was recommended
annuallyinthosewithanindwellingcatheter.
3. CervicalMyelopathy
Cervicalmyelopathyisgenerallycausedbycompression,secondaryto
eitherspondylosis,ossificationoftheposteriorlongitudinalligament,orcervical
diskherniation.Sakakibaraandcolleagues(1995a)studied128affectedpatients,
of whom 95 had voiding symptoms, 61 had irritative symptoms, 71 had
obstructive symptoms, and 25 had urinary incontinence. Urodynamic studies
revealed involuntary bladder contractions in 61 patients and detrusor sphincter
dyssynergia.

4. AcuteTransverseMyelitis
Acute transverse myelitis is a rapidly developing condition
with motor, sensory, and sphincter abnormalities, generally with a
well defined upper sensory limit and no signs of spinal cord
compression or other neurologic disease. It may result from a
variety of mechanisms from parainfectious, autoimmune, vascular,
or demyelinating.
5. Neurospinal Dysraphism
Spinal dysraphism refers to the malformation of the
vertebral arches and, commonly, malformation of the neural tube.
he term includes spina bifida occulta, which involves only a bony
(vertebral) arch defect, and spina bifida cystica (aperta), which
involves a bony defect and a neural tube (spinal cord) defect. The
two
primary
subclasses
of
spina
bifida
cystica
are
myelomeningocele (the nerve roots or portions of the spinal cord
have evaginated beyond the vertebral bodies) and meningoceles
(contain only a herniated meningeal sac with no neural elements).
Urologic dysfunction often becomes a problem of the
adolescent or adult with this disease. The typical myelodysplastic
patient shows an areflexic bladder with an open bladder neck. The
bladder generally fills until the resting residual fixed external
sphincter pressure is reached, and then leakage occurs. Stress
incontinence occurs also, related to changes in intra-abdominal
pressure.
In adult females, the treatment strategy is generally to
increase urethral sphincter efficiency without causing a major
enough increase in urethral closing pressure that will result in a
change in bladder compliance. Periurethral injection therapy to
achieve continence may replace the pubovaginal sling and artificial
sphincter in this circumstance. continence in adult male
myelodysplastic individuals follows the same general rules as in
females, and injectable materials may give good results in this
group as well. When the urethra is widely dilated and somewhat
rigid, and neither procedure alone will provide sufficient coaptation,
it may be possible to combine a prostatic sling with periurethral
collagen injection. Dry individuals, of course, will be on intermittent
self-catheterization.
6. Tabes Dorsalis, Pernicious Anemia
Although syphilitic myelopathy 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 be a cause of
sensory neurogenic. Hattori and colleagues (1990) reported on
some patients with only tabes as an obvious cause of their voiding
dysfunction who had low compliance or detrusor overactivity.
6. Poliomyelitis

Voiding dysfunction in polio is that of a typical motor neurogenic

bladder with urinary retention detrusor areflexia, and intact
sensation. The reported incidenc of voiding dysfunction in patients
with polio was describe as ranging from 4% to 42%
7. Disease Distal to the Spinal Cord
a. Disk Disease
Disk prolapse anywhere in the lumbar spine could interfere
with the parasympathetic and somatic innervation of the lower
urinary tract, striated sphincter and other pelvic floor musculature,
and afferent activity from the bladder and affected somatic
segments to the spinal cord. Some patients with detrusor areflexia
reported difficulty voiding with straining. Patients with voiding
dysfunction generally presented with these symptoms or in urinary
retention. The most consistent urodynamic finding was that of a
normally compliant areflexic bladder associated with normal
innervation or findings of incomplete denervation of the perineal
floor musculature
The detrusor areflexia associated with lumbar disk
protrusion shows lower incidence of decreased compliance than in
the voiding dysfunction caused by myelomeningocele. There is
Cauda equina syndrome is a term applied to the clinical picture of
perineal sensory loss with loss of voluntary control of both anal and
urethral sphincter and of sexual responsiveness. This can occur not
only secondary to disk disease (severe central posterior disk
protrusion) but to other spinal canal pathologic processes as well.
Laminectomy may not improve bladder function, and
prelaminectomy urodynamic evaluation is therefore desirable
because it may be difficult postoperatively in these cases to
separate causation of voiding dysfunction owing to the disk
sequelae from changes secondary to the surgery.
b. Spinal Stenosis
Spinal stenosis is a term applied to any narrowing of the
spinal canal, nerve root canals, or intervertebral foramina. It may
be congenital, developmental, or acquired. Compression of the
nerve roots or cord by such a problem may lead to neuronal
damage, ischemia or edema. Spinal stenosis may occur without
disk prolapse. Symptoms may range from those consequent to
cervical spinal cord compression to a cauda equina syndrome, with
corresponding urodynamic findings. The urodynamic findings are
dependent on the level and the amount of spinal cord or nerve root
damage.
c. Radical Pelvic Surgery
Voiding dysfunction after pelvic plexus injury occurs most
commonly
after abdominoperineal resection and radical hysterectomy. The
incidence has been estimated to range from 20% to 68% of patients
after abdominoperineal resection, 16% to 80% after radical

hysterectomy, 20% to 25% after anterior resection, and 10% to 20%

after proctocolectomy. The injury may occur consequent to
denervation or neurologic decentralization, tethering
of the nerves or encasement in scar, direct bladder or urethral
trauma, or bladder devascularization.
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.
d. Herpesvirus Infections
Invasion of the sacral dorsal root ganglia and posterior nerve
roots with herpes zoster virus may produce urinary retention and
detrusor areflexia days to weeks after the other primary viral
manifestations. Urinary incontinence secondary to detrusor
overactivity may also occur, but the pathophysiology is uncertain. It
may be related to nerve root irritation, inflammation of the
meninges or spinal cord, or zoster cystitis.
Herpetic cystitis may present with dysuria, frequency,
retention, pyuria, hematuria. Neuritis associated with affecting the
sacral motor neurons and presented in urinary retention and with a
flaccid bladder. Urinary retention has also been reported to occur in
association with anogenital herpes simplex virus infection.
e. Diabetes Melitus
In DM patient have classic typical of urodinamic include
impaired bladder sensation, increased cystometric capacity,
decreased bladder contractility, impaired uroflow and increased
residual urine volume. Hyperglycemia is lead to microvascular and
neurologic complications, the neurologic sequelae ultimately
resulting in a loss of myelinated and unmyelinated fibers, wallerian
degeneration, and blunted nerve fiber reproduction and function.
Hyperglicemia increased accumulation of polyols (sorbitol)
from glucose through the aldolase-reductase pathway, inhibiting
both glomerular and neural synthesis of myoinositol. The decrease
in myoinositol synthesis depresses phosphoinositide metabolism,
decreasing Na+,K+-ATPase activity. Hyperglycemia also leads to the

formation of advanced glycosylation end products, inhibition of the

formation of which in animals has been shown to improve response
to functional and structural abnormalities of peripheral nerves.

f. Guillain-Barr Syndrome
Guillain-Barr syndrome (GBS) is an inflammatory
demyelinating disorder of the peripheral somatic and autonomic
nervous system that may be life threatening. It is described as a
recognizable clinical entity characterized by rapidly evolving
symmetrical limb weakness, loss of tendon reflexes, absent or mild
sensory signs, and variable autonomic dysfunctions. It is triggered
by a preceding bacterial or viral infection, with the immune
responses directed toward the infecting organisms crossreacting
with neural tissues. The immune reactions against Schwann cell
surface membrane or myelin result in acute inflammatory
demyelinating neuropathy and cause acute motor-sensory axonal
neuropathy.
The prevalence of lower urinary tract dysfunction has been
reported as ranging from 25% to more than 80% like, voiding
dysfunction, urinary retention, urgency, nocturia and urge
incontinence, stress incontinence and voiding dificulty.
8. Miscellaneous Neurologic Diseases Causing Voiding
Dysfunction
a. Lyme disease
Lyme disease have three syndromes : Encephalopathy,
polyneuropathy and leukoencephalitis. Chancellor and colleagues
(1993) described seven patients who also had lower urinary tract
dysfunction like detrusor overactivity and detrusor areflexia and
urinary retention and subjective symptoms noted were urgency,
frequency, nocturia and urge incontinence.
b. Hereditary Spastic Paraplegia
Hereditary spastic paraplegia is a genetically transmitted
disorder, generally autosomal dominant, less commonly autosomal
recessive, and rarely sex linked. There is a pattern of central
demyelination with axon loss and progressive lower extremity
spasticity generally with muscle weakness. Urinary urgency and
frequency were the dominant complaints. The authors proposed that
the lower urinary tract symptoms (and bowel and sexual
dysfunction) in patients with this disorder are caused by a
combination of somatic and autonomic nervous system
involvement, supporting a multisystem involvement.
c. Tropical Spastic Paraparesis
Tropical spastic paraparesis is primarily a spinal cord
myelopathy caused by a retrovirus (human T-cell leukemia virus 1

[HTLV-1]) similar to HIV. Progressive lower limb weakness and back

pain are typically the primary complaints, but voiding dysfunction
occurs in up to 60% of those affected. The type of voiding
dysfunction depends on whether the damage is primarily to the
descending spinal tracts, to the sacral nuclei, or to the sacral
outflow.
d. Acquired Immunodeficiency Syndrome
Infection with HIV can affect both the central and
peripheral nervous systems. Clinical symptomps that
occure form HIV infection included frequency, urgency and
incontinence.

e. Syringomyelia
Syringomyelia is a chronic disorder of the spinal cord
characterized by dissociated sensory loss and brachial amyotrophy.
Voiding dysfunction has been reported in 9% to 25% of patients.
Syringomyelia patient had urinary symptoms like difficulty voiding,
retention, nocturnal and daytime frequency and also incontience,
urgency and enuresis. The urinary symptoms appeared from 2
months to 13 years after the initial neurologic symptoms.
e. Schistosomal Myelopathy
Schistosomiasis can rarely cause spinal cord involvement,
either as a granulomatous intrathecal mass or as an acute
transverse myelitis. This patient presented with urinary
incontinence. They had detrusor overactivity like dyssynergia and
with minimal motor weakness and striated sphincter dyssynergia.
f. Systemic Lupus Erythematosus
Systemic lupus erythematosus (SLE) is a disease in which
there is widespread inflammatory change in the connective tissues
and small vessels of the skin and systemic organs, probably
autoimmune in origin. This patient presented voiding dysfunction,
voiding difficulty, urinary incontinenece.
9. Miscellaneous Conditions Definitely, Probably, or Possibly
Related To Neuromuscular Dysfunction
a. Detrusor Sphincter Dyssynergia
Sphincter dyssynergia refers to an involuntary
contraction or lack of relaxation of either the striated sphincter (the
striated muscle surrounding the proximal urethra and the striated
muscle that forms a part of the urethra for a variable distance from
the urogenital diaphragm to the bladder neck) or the smooth
sphincter. True DESD should exist only in patients who have an
abnormality in pathways between the sacral spinal cord and the

brainstem pontine micturition center, generally caused by

neurologic injury or disease.
Without proper treatment, more than 50% of men with
DESD will develop significant complications such as VUR, upper tract
deterioration, urolithiasis, urosepsis, and ureterovesical obstruction.
Therapy for DESD is designed to either eliminate or
significantly lessen the abnormal sphincter activity or to circumvent
it. Oral medical therapy directed toward the striated sphincter has
not enjoyed wide success, and the most common current
approaches are (1) CIC (usually combined with therapy to control
detrusor overactivity), (2) sphincterotomy, (3) stent placement
across the sphincter, (4) injection of botulinum toxin into the
sphincter, (5) continuous catheterization, and (6) urinary diversion.
b. Dysfunctional Voiding
This syndrome, also described by various authors as
non-neurogenic neurogenic bladder, occult voiding dysfunction,
occult neuropathic bladder, learned voiding dysfunction, and the
Hinman syndrome, presents the unusual circumstance of what
appears urodynamically to be involuntary obstruction at the striated
sphincter level existing in the absence of demonstrable neurologic
disease .
c. Bladder Neck Dysfunction
Bladder neck dysfunction is defined here as an
incomplete
opening of the bladder neck during voluntary or involuntary voiding.
It has also been referred to as smooth sphincter dyssynergia,
proximal urethral obstruction, primary bladder neck obstruction, and
dysfunctional bladder neck. The term smooth sphincter dyssynergia
or proximal sphincter dyssynergia generally used when referring to
this urodynamic finding in an individual with autonomic
hyperreflexia.
The dysfunction is found almost exclusively in young and
middle-aged men, and characteristically they complain of longstanding
voiding/emptying
(obstructive)
and
filling/storage
(irritative) symptoms.
d. Bladder Outlet Obstruction in Woman
Bladder outlet obstruction in women in general is
uncommon. Nitti and colleagues (1999) evaluated the videourodynamic studies of 261 of 331 women who underwent
multichannel studies for nonneurogenic voiding dysfunction. They
defined bladder outlet obstruction as radiographic evidence of
obstruction between the bladder neck and the distal urethra in the
presence of a sustained detrusor contraction of any magnitude,
which is usually associated with reduced or delayed urinary flow
rate. Obstruction at the level of the bladder neck was diagnosed
when the bladder neck was closed or narrowed during voiding.

Obstruction of the urethra was diagnosed as a discrete area of

narrowing, associated with proximal dilatation. Most authors would
agree that surgical treatment of this problem in women should be
approached with caution because sphincteric incontinence is a
significant risk.
e. Low-Pressure/Low-Flow Voiding in Younger Men: Bashful
Bladder
pressure/low-flow voiding can be the result of a number
of causes, most notably a decompensating detrusor (generally from
bladder outlet obstruction or as a part of the syndrome known as
detrusor hyperactivity with impaired contractility. When this occurs
in a young man, it is generally characterized by frequency,
hesitancy, and a poor stream.
f. Urinary Retention; the Fowler Syndrome in Young
Women
The Fowler syndrome is 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 of age who
has found herself unable to void for a day or more with no urinary
urgency but increasing lower abdominal discomfort. A bladder
capacity of more than 1 L with no sensation of urgency is necessary
for the diagnosis. There are no neurologic or laboratory features to
support a diagnosis of any neurologic disease. MRI of the brain and
the entire spinal cord is 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.
g. Poseoperative Urinary Retention
Postoperative urinary retention is a well-recognized but
poorly understood event. It occurs more frequently after lower
urinary tract, perineal, gynecologic, and anorectal surgery. There are
contributing factors, which are not mutually exclusive, include the
following eight factors:
1. Traumatic instrumentation
2. Bladder overdistention
3. Diminished awareness of bladder sensation
4. Decreased bladder contractility
5. Increased outlet resistance
6. Decreased micturition reflex activity
7. Nociceptive inhibitory reflex
8. Preexistent outlet pathology (e.g., BPH)
-Adrenergic blockade with phenoxybenzamine historically has
seemed effective prophylactically in decreasing the incidence of
postoperative retention.

h. Hyperthyroidism
Patients with thyrotoxicosis often present with
symptoms caused by sympathetic overactivity and autonomic
nervous system imbalance. The authors hypothesize that increased
-adrenergic activity in thyrotoxicosis is responsible for a reduced
flow rate and increased bladder capacity because of the inhibitory adrenergic activity on detrusor muscle contractility. The voiding
dysfunction and urodynamic abnormalities resolved after resolution
of the hyperthyroidism.
i. Gastroparesis
Gastroparesis is a condition characterized by
symptoms from impaired transit of intraluminal gastric
contents into the duodenum but in the absence of mechanical
obstruction. It may becaused by diabetes, occur after gastric
surgery, or be idiopathic. patients with gastroparesis have
voiding syndrome like abnormal detrusor contraction, delayed
sensation, poor detrusor function.
j. 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 after even 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 to weakness in a variety of
striated muscle groups. The incidence of incontinence after
prostatectomy is indeed greatly increased in patients with this
disease.
voiding dysfunction in patients with myasthenia gravis
(one woman with intrinsic sphincter deficiency, poor pelvic muscle
contractility, and detrusor hyperreflexia; one male with detrusor
hyporeflexia complaining of urgency and incontinence; and one
young woman with an acontractile bladder) and added a personal
report of a fourth patient with urinary retention from detrusor
areflexia.
k. Isaacs Syndrome
Isaacs syndrome is a rare neurologic disorder
characterized by continuous muscle contraction, fasciculations,
myokymia, excessive sweating, and elevated creatinine kinase level.
It is shown to be secondary to antibodies possibly directed against
potassium channels on peripheral nerves and is associated with
peripheral neuropathy, autoimmune diseases, malignancies, and
endocrine disorders. This patient have painful urinary, it because by
spasm of the periurethral striated sphincter. This condition was
treated with plasmapheresis and phamacologic agent to relax
skeletal muscle.

l. Wernicke Encephalopathy
Wernicke encephalopathy is a rare but well-documente
condition
caused by a deficiency in thiamine (vitamin B1). Pathologic lesions
are characteristically distributed periventricularly at the levels of the
third and fourth ventricles including the mammillary body, medial
thalamic nucleus, hypothalamus, superior cerebellar vermis,
periaqueductal gray matter, and midbrain tegmentum. The two
major clinical manifestations of thiamine deficiency involve the
cardiovascular and neurologic systems.
Sakakibara and colleagues (1997b) report a case of a
pregnant woman with multiple neurologic manifestations of central
and peripheral neuropathy and with urge incontinence, manifested
urodynamically by involuntary bladder contractions and a decreased
bladder volume. Resolution of the urinary symptoms occurred after
thiamine replacement.
m. Systemic Sclerosis (Scleroderma)
Scleroderma is a disease of the connective tissue
characterized by thickening and fibrosis of the skin, abnormalities of
the small arteries, and involvement of the gastrointestinal tract,
heart, lung, and kidneys. The pathogenesis is unknown but thought
to be caused by overexpression of the collagen gene DNA,
contributing to excessive production of collagen in these patients.
The authors speculate that voiding dysfunction, when it
occurs, could be caused by the fibrotic replacement of bladder
smooth muscle, but they did not exclude some degree of autonomic
dysfunction as well.
10.

Aging
Lower urinary tract symptoms and disorders are
prevalent and bothersome in the elderly population. When
considering the effects of aging on the lower urinary tract, one
cannot separate the effects of chronologic age itself from the
various
anatomic,
neuromorphologic,
neurophysiologic,
neuropharmacologic, metabolic, and hormonal changes that coexist
with aging, along with the effects of other coexisting disease
processes. Additionally, neurologic phenomena may masquerade as
lower urinary tract symptoms associated with bladder outlet
obstruction. These phenomena may include multiple cerebral
infarctions, cervical spondylosis, and lumbar spondylosis.

11.

Other Conditions

a. Parturition, Caesarean Section,

and Simple Hysterectomy.

No clear consensus yet exists as to the independent
effects of child birth and hysterectomy on lower urinary tract
function. A variety of explanations have been put forth regarding
whether or not, in fact, lower urinary tract dysfunction is induced by
these events and, if so, what the underlying pathophysiology may
be.
12. TREATMENT OF NEUROGENIC LOWER URINARY TRACT
DYSFUNCTION
The results of treatment of voiding dysfunction are
rarely perfect, and they do not have to be. The goals are :
1. Voiding Dysfunction: Goals of Management
Upper urinary tract preservation or improvement
Absence or control of infection
Adequate storage at low intravesical pressure
Adequate emptying at low intravesical pressure
Adequate control
No catheter or stoma
Social acceptability and adaptability
Vocational acceptability and adaptability
2. Reasons to Change or Augment a Given Regimen
Upper urinary tract deterioration
Recurrent sepsis or fever of urinary tract origin
Lower urinary tract deterioration
Inadequate storage
Inadequate emptying
Inadequate control
Unacceptable side effects
Skin changes secondary to incontinence or collecting device
3. Patient Factors to Consider in Choosing Therapy
Prognosis of underlying disease, especially if progressive or
malignant
General health
Limiting factors: inability to perform certain tasks (e.g., hand
dexterity, ability to transfer, body habitus)
Mental status
Motivation
Desire to remain catheter or appliance free
Desire to avoid surgery
Sexual activity status
Reliability
Educability
Psychosocial environment, interest, reliability, and cooperation of
family
Economic resources