INCONTINENCE IN NEUROPATHIC PATIENTS
EXAMINATION IN MULTIPLE SCLEROSIS
VIII. GUILLAIN-BARRE SYNDROME
3. DISEASE SPECIFIC THERAPY
Emergency surgical decompression has been reported to be important to increase the chance of satisfactory neurological recovery in patients with cauda equina syndrome due to central lumbar disc prolapse [4,18,19]. In a meta-analysis of surgical outcomes, Ahn et al (2000)  reported that a significant improvement in sensory and motor deficits as well as urinary and rectal function occurred in patients who underwent the surgery within 48 hours compared with those who had the surgery more than 48 hours after the onset of the cauda equina syndrome. Although there is still a controversy , most of other reports support the concept that decompression performed within 48 hours of onset of this syndrome resulted in improved functional outcomes [3,8,20]. However, acontractile detrusor is usually irreversible even after
patients can empty their bladder postoperatively, but only by straining or changing their voiding postures [10,22]. In contrast to bladder dysfunction, urethral function shows a better recovery after surgery [10,13].
Please refer to the chapter on children. We have looked in the scarce literature on adult patients.
1. URINARY INCONTINENCE
a) Epidemiology and prevalence
Myelomeningocele (spina bifida) is one of the most common birth defects of the spine and brain. It occurs in 1-2 births per 1000, involving all levels of the spinal column (lumbar 26%, lumbosacral 47%, sacral 20%, thoracic 5% and cervical spine 2%). Associated Arnold- Chiari malformation is seen in 85% of children, often requiring ventriculo-peritoneal shunting of cerebros- pinal fluid. Ingestion of folic acid prior to conception and during the first trimester of pregnancy has significantly reduced the incidence of this problem and other associated neural tube defects. The neurologic defect produced is quite variable and cannot be totally predicted by the vertebral level of the lesion. Additionally the fibrosis associated with myomeningocele closure, may tether the cord. Subsequent growth of the infant or child will produce further neurologic problems, manifesting as changes in bladder, bowel and lower extremity function. The incidence of urethrovesical dysfunction in myelomeningocele is not absolutely known, but most studies suggest it is very high (>90%). Similarly, anorectal dysfuction is very common, but its exact incidence has not been reported. Congenital neurologic bladder dysfunction with spina bifida and sacral dysgenesis that manifested itself only at middle age in a 48-year-old male is reported by Kaneoya et al (LOE4). Yamamura et al reviewed the literature of tethered cord of adult onset and found 56 cases published.(LOE 3) .
b) Pathology and disease specific LUT problems
The two major consequences of the vesicourethral dysfunction are urinary incontinence and hydrouretero- nephrosis which can occur early or later in life . There are many studies documenting the urodynamic characteristics of the vesicourethral unit in myelomeningocele patients but almost all in children. Almodhen and colleagues examined myelomenigocele patients in postpubertal age and correlated these findings with upper urinary tract changes  (LOE 3). Of the 26 patients with urinary incontinence before puberty 12 achieved continence following puberty. Hydronephrosis remained stable in 4 patients,
coureteral reflux persisted in 1 patient, resolved in 4 and was new onset in 1. Regarding the uro-dynamic findings in patients achieving urinary continence following puberty total cystometric bladder capacity increased significantly and maximum detrusor pressure and detrusor leak point pressure showed insignificant changes. This findigs demonstrate that conservative treatment is a viable option for some myelo- meningocoele patients and that with current treatment modalities, including intermittent catheterization no significant upper tract deterioration occurs after reaching the pospubertal age.
In the past much attention has been directed at the significance of dyssynergia between the external sphincter and the bladder, and the associated deterioration of the upper renal tracts in these patients. With the increasing reliance of clean intermittent catheterization in the management of these patients, more emphasis has been placed on the pressure the bladder is able to generate prior to leaking, as a prognostic factor in predicting upper tract deterioration. c) Disease specific diagnosis and treatment Urodynamics is the cornerstone in the diagnosis and management of vesicourethral dysfunction in myelomeningocele. As previously stated, urodynamic findings may predict the patients at risk of upper tract deterioration. Controversy continues as when to initiate these studies, either as soon as possible after back closure, at the first sign of upper tract changes or before considering management of incontinence. Studies supporting each position have been reported, although the preponderence of evidence suggests earlier diagnosis of hostile factors is advisable.Taskinen et al  examined 30 patients with anorectal anomalies mainly because of fecal or urinary incontinence. All patients underwent spinal magnetic resonance imaging and urodynamic investigation. Major lumbosacral abnormalities were detected in 57% of patients, including 13, 4 and 3 with a tethered cord, syrin- gomyelia and caudal regression, respectively. Significant dysfunction of the LUT in 57% of the cases involved an overactive detrusor in 11, detrusor- sphincter dyssynergia in 4, distended bladder in 4 and lazy bladder in 1. When the spinal cord was normal, 54% of the patients had abnormal urodynamic findings but when the spinal cord was abnormal, 59% had abnormal urodynamics. When the bony spine was normal, 33% of the patients had an abnormal spinal cord but when the bony spine was abnormal, 69% had an abnormal spinal cord. (LOE3).
As hydronephrosis and vesico-ureteric reflux are a consequence of detrusor dysfunction, synchronous fluoroscopic evaluation of the urinary tract is advisable at the time of urodynamics. Similarly, renal ultrasound has become an invaluable routine serial evaluation in these patients, assessing renal growth, development of scarring and, most importantly, hydrourete- ronephrosis. Studies suggest a role for repeat
urodynamics and ultrasound in this patient population, however, the timing and frequency of these studies still needs to be elucidated.
Although, renal scans are routinely used, especially in the myelomeningocele patient with hydronephrosis, the exact role of this study in these patients is not clear.
Urologic treatment depends on the age of the patient and the nature of the vesico urethral dysfunction as characterized by urodynamics. In a retrospective study urinary sepsis accounted for the majority of admissions (62%), while 38 of 62 patients required 60 surgical procedures. Targeting the primary urological abnormality (the dysfunctional and usually poorly compliant bladder) allowed implementation of effective treatments, including regular intermittent bladder catherisation (52%) in order to preserve upper renal tract function. Associated postural abnormalities complicated both conservative and interventional therapies.
The mainstay of treatment is clean intermittent catheterization and antimuscarinic medication. As continence is not at issue in the neonate and infant, treatment may be postponed, unless upper tract changes are present. Some evidence exists pointing to the fact that early initiation of treatment may prevent subsequent deleterious bladder changes. Recently botulinum toxin was suggested as a possibility to avoid invasive surgery in these patients .
Bruschini et al evaluated 104 patients who were not managed and followed-up adequately during their childhood  (LOE 3). Reflux and urinary tract damage were found in 30 patients, 6 patients presented signs of upper tract damage without reflux. The cystometry showed detrusor overactivity in 48% of patients, poor compliance in 49% of patients, increased bladder capacity in 2% and normal cystometry in 1%. Detrusor leak point pressure over 40 cm H(2)O was associated with upper urinary tract damage. Patients with a decrease of functional bladder capacity over 33% had more renal scars than their counterparts. Overall, 26 % of urological untreated myelomeningocele patients have kidney damage.
On the other hand there is a work by Olsson and colleagues, evaluating 175 Swedish myelomenin- gocele patients in adult age  (LOE 3). Clean intermittent catheterisation for bladder emptying was used by 85%, and 59% used enemas on a regular basis because of the neurogenic bowel dysfunction. Renal dysfunction was than seen only in 1.7% of the adolescents.
Management of incontinence and/or upper tract deterioration mirrors the treatment of neurologic bladder. Variations in this algorithm include the use of vesicostomy in the younger child who has failed conservative measures and has evidence of deteriorating upper tracts. External sphincterotomy
has no place in the management of these patients and the use of the appendico vesicostomy in continent LUT reconstruction(Mitrofanoff) has become very popular. Most studies on surgical management of the myelomeningocele bladder are descriptive (LOE 4) at best. Data from adult and paediatric surveys show renal damage to be the single most prevalent cause of morbidity and mortality; even in children, 30-40% exhibit evidence of renal damage.
Additional factors such as chronic infection and stone formation will then render the kidney more vulnerable to progressive loss of renal mass and subsequent chronic renal failure. Renal transplantation is now considered the optimal treatment for end-stage renal disease in all age groups. Although more prone to complications, recent data on patients with meningomyelocele or severely abnormal LUTs demonstrate excellent patient and graft outcomes.  (LOE 3)
d) Guidelines for further research
Further clarification of the role of fetal surgery to repair the neural tube defect is required. Similarly the role of early intervention, conservative or surgical is required. The timing of surgical intervention needs further study as well as better quality of life assessments and risk/ benefit analyses of LUT reconstructive procedures. The development of a tissue-engineered substitute for cystoplasy is being studied. Finally, the fate of the adult myelomeningocele patient, especially those who have undergone reconstruction needs to be documented.
2. FAECAL INCONTINENCE AND BOWEL