Functional significance of upper and lower motor neuron impairment in amyotrophic lateral sclerosis

The objective of this study was to examine the contribution of lower motor neuron (LMN) and upper motor neuron (UMN) dysfunction to weakness and impaired motor control in 27 patients with amyotrophic lateral sclerosis (ALS). Isometric strength was measured by dorsiflexor maximum voluntary contraction force (MVC). LMN function was measured by tetanic force, twitch force, and the amplitude of the compound muscle action potential. UMN function was measured by the speed of rapid foot taps, the maximum rate of rise of voluntary force, and the central activation ratio [CAR = MVC/(MVC + superimposed tetanic force)]. The results suggest that (1) LMN loss appears to be the primary cause of progressive weakness in ALS; while (2) UMN impairment in ALS leads to slowing of contraction speed and rapid movements, and modest decreases in central activation; and (3) during 6 months of progression, LMN changes were greater than UMN changes.     

Urinary bladder incontinence after prostatectomy. Urodynamic evaluation and results of therapy

Forty-seven males referred due to postprostatectomy urinary incontinence (34 after transurethral resection of prostatic adenoma and 13 after open suprapubic adenomectomy) were retrospectively studied. Urodynamic evaluation identified 19 (40.4%) men with incontinence due solely to sphincter incompetence, and 19 (40.4%) men, in addition to sphincter incompetence, had urinary bladder dysfunction (unstable detrusor and/or reduced bladder compliance). Seven (14.8%) men had pure bladder dysfunction as the only cause of urinary incontinence. Two patients had normal urodynamic findings (N = 2; 4.2%). Men with urinary incontinence due only to sphincter incompetence were treated by insertion of artificial sphincter devices or condom catheter drainage (lack of artificial sphincters), while others were treated pharmacologically (imipramine, propantheline, oxybutynin or their combinations ... N = 25), or by augmentation cystoplasty using ileum after unsuccessful pharmacological treatment (N = 3). Out of 25 patients with pharmacological treatment, 21 were available for the final assessment of the treatment efficacy. Eleven (52.3%) patients were "socially continent" after the treatment. It is concluded that in the assessment of the cause of postprostatectomy urinary incontinence urodynamic evaluation is mandatory, and that the treatment should be based on the results of such studies. The role of bladder dysfunction as a cause of postsurgical urinary incontinence is again strongly emphasized.     

Tethered cord syndrome in occult spinal dysraphism: timing and outcome of surgical release

OBJECTIVE: To investigate the influence of neurosurgical intervention on the appearance of upper motor neuron (UMN) signs in newborns diagnosed with occult spinal dysraphism and tethered cord (TC) during the first month of life. METHODS: A prospective study (1990 to 1996) of 22 consecutive newborns with occult spinal dysraphism monitored for the appearance of UMN signs. Untethering was performed when neurologic or urodynamic investigation indicated the presence of UMN dysfunction. RESULTS: Of 22 patients, 10 remained free of UMN symptoms during follow-up (mean, 67+/-22 months). Untethering was performed in 12 of 22 patients because of the presence of UMN symptoms. In 7 of these 12 patients, there was a documented asymptomatic period of 13+/-11 months before the onset of UMN symptoms. Untethering at a mean age of 18+/-17 months restored normal neurologic and urinary function in all patients (mean postoperative follow-up, 25+/-16 months). Of the 12 children, 5 presented with UMN signs at birth. In these children, untethering was performed at a mean age of 9+/-5 months. In two of these five patients, UMN symptoms did not resolve after surgery, and ongoing conservative bladder treatment was required (mean follow-up, 37+/-14 months). In none of the 12 operated children did signs of retethering occur. CONCLUSIONS: A significant number (10/22) of children born with occult spinal dysraphism and TC did not develop UMN symptoms during follow-up; neurosurgical correction after the appearance of an UMN sign restored normal neurologic and urinary function in all children; and untethering in children presenting at birth with UMN symptoms resulted in poorer outcome.     

Anal and urinary incontinence after obstetric anal sphincter rupture]

The aim of the study was to assess the long term impact of obstetric anal sphincter rupture on the frequency of anal and urinary incontinence, and identify factors to predict patients at risk. In 94 consecutive women who had sustained an obstetric anal sphincter rupture, anal manometry, anal sphincter electromyography and pudendal nerve terminal motor latency at three months post partum was performed. A questionnaire regarding incontinence was sent between two to four years post partum. Forty-two percent of responders had anal incontinence, 32% had urinary and anal incontinence. Overall, 56% of the women had incontinence symptoms. The occurrence of anal incontinence was associated with pudendal nerve terminal motor latencies > 2.0 milliseconds and the occurrence of urinary incontinence was associated with the degree of rupture, the use of vacuum extraction and previous presence of urinary incontinence. Thirty-eight percent of the women with incontinence wanted treatment, but only a few had sought medical advice.     

Clinical features of transurethral anterior sphincterotomy and urological management of patients with cervical spinal cord injury

BACKGROUND: The ideal urological management for the patients with cervical spinal cord injury (CSCI) is to obtain catheter free urination and to prevent urinary tract complications. We have evaluated cases that had undergone transurethral anterior sphincterotomy from the view-point of the operative indications and the efficacy. METHODS: We carried out sphincterotomy 166 times on 133 male patients with CSCI in our Center. Before the operations were performed, all patients suffered from urinary incontinence, and they were unable to catheterize themselves for low level activity of daily life. Before and after the operation, their detrusor functions with sphincter reactions were assessed by urodynamic study. In principle we have followed up these cases by cystogram combined with cystometry, cystogram and excretory-pyelography or abdominal ultrasonography. RESULTS: In post-operative evaluations, more than 80% of cases attained hypotonic detrusor contractions and residual urine was significantly decreased. In long term follow-up, 96% of patients had obtained catheter free urination and about 85% of patients had no urinary tract complications, such as bladder deformity, vesicoureteral reflux, or hydronephrosis, with the exception of common unavoidable urinary infections. About 20% of cases had to be re-operated upon, and some cases showed hypertonic detrusor contractions or detrusor-sphincter-dyssynnergia during follow-up. CONCLUSION: The operative indications of sphincterotomy should be decided when the CSCI patients is unable to perform self-catheterization, and when due to the dysfunction of the urethral sphincter, these patients suffered from voiding difficulties or autonomic dysreflexia, or when the urinary tract complications might occur. In the majority of cases the aims of the sphincterotomy were achieved but some cases underwent another operation or had recurrent dysfunction of the urethral sphincter, indicating the need for careful follow-up.     

Our experience with pubovaginal slings in patients with stress urinary incontinence

PURPOSE: Pubovaginal slings successfully treat stress urinary incontinence in women with intrinsic sphincter deficiency. Because of its durability, it has been an attractive procedure in select patients with urethral hypermobility. We examine our experience with pubovaginal sling. MATERIALS AND METHODS: A total of 150 patients were evaluated for pelvic prolapse and urinary incontinence. An abdominal leak point pressure was determined in all patients. Of patients with type II stress urinary incontinence, 36 patients (80%) underwent additional gynecological procedures at the time of the pubovaginal sling, compared to 29% with intrinsic sphincter deficiency and 33% with coexisting urethral hypermobility and intrinsic sphincter deficiency. RESULTS: The overall cure rate was 93% with a mean followup of 22 months. At 1 week postoperatively spontaneous voiding was accomplished by 56% of the patients with urethral hypermobility and 57% with intrinsic sphincter deficiency. Only 2.8% of patients required surgical therapy for prolonged urinary retention. De novo urgency/urge incontinence occurred in 19% of women with a 3% incidence of persistent urge incontinence. CONCLUSIONS: Pubovaginal slings are effective and durable. Voiding dysfunction is uncommon and is temporary in most patients.     

Motor neuron syndromes in cancer patients

Previous reports indicate that motor neuron disease (MND) may rarely be associated with systemic cancer. We have encountered 14 patients with MND and cancer who formed three distinct groups. Group 1: Three patients developed a rapidly progressive MND, less prominent symptoms of involvement of other areas of the nervous system, and anti-Hu antibodies. Group 2: Five women developed signs of upper motor neuron (UMN) disease, initially resembling primary lateral sclerosis (PLS), and breast cancer. In 4, symptoms of UMN occurred within 3 months of cancer diagnosis or tumor recurrence. They had no metastases or spinal cord compression. Serum anti-neuronal antibodies were negative. Three patients are alive (follow-up of 156, 15, and 12 months), and 2 remain without lower motor neuron signs. Group 3: Six patients developed MND resembling amyotrophic lateral sclerosis between 47 months before and 48 months after their cancer diagnosis. In group 1, the MND associated with the anti-Hu antibody is unequivocally paraneoplastic. In group 2, the proximate onset of MND with the diagnosis of cancer or its recurrence, its pure or long-lasting UMN signs, and its association with breast cancer, suggest that the disorder may be paraneoplastic. Although for most cancer patients who develop MND the occurrence of both disorders is probably coincidental, in some patients with MND a careful search for an underlying cancer is warranted (ie, patients in groups 1 and 2).     

Two-staged pancreatoduodenectomy with external drainage of pancreatic juice and omental graft technique

Massive bilateral vesicoureteral reflux (VUR) in a 7-year-old girl with spinal scoliosis was successfully treated by endoscopic correction. She was admitted due to a febrile urinary tract infection and urinary incontinence. A cystometrogram demonstrated normal detrusor function during storage. The endoscopic subureteric injection of polytetrafluoroethylene (Teflon) was performed, resulting in the disappearance of the VUR. A postoperative cystometrogram demonstrated overactive detrusor function during storage, necessitating anticholinergic medication. She has been free of febrile urinary tract infections and incontinence for 2 years postoperatively, although self-catheterization is necessary. In a case of neurogenic vesical dysfunction with massive reflux, endoscopic subureteric injection is not only a therapeutic tool, but also a useful diagnostic option for detecting occult detrusor overactivity during storage prior to open surgery.     

Value of sphincter electromyography in the diagnosis of multiple system atrophy

It is clinically important, to distinguish between idiopathic Parkinson's disease (IPD) and multiple system atrophy (MSA) not only because of the implications for prognosis but also because urinary incontinence is often an early troublesome feature of MSA and by making the correct neurological diagnosis inappropriate urological surgery may be avoided. Onuf's nucleus in the sacral cord is the location of the anterior horn cells innervating the sphincters, and it is among central nervous system sites affected by neuronal cell loss in MSA but not in IPD. A systematic analysis of motor units recorded from the sphincter looking for changes of chronic reinnervation has therefore been used to distinguish between these conditions. Sphincter electromyography (EMG) was carried out in 126 patients with suspected MSA with review of their case notes up to 2 years later. Of those in whom a diagnosis of MSA was made, 82% had had an abnormal sphincter EMG.     

Management of neurogenic fecal incontinence in myelodysplastic children by a modified continent appendiceal stoma and antegrade colonic enema

OBJECTIVES: Antegrade colonic enemas for neurogenic fecal incontinence via reverse reimplanted appendices (Mitrofanoff principle) have been primarily reported by Malone and coworkers in 1990. We used a modification of the described surgical technique and treated the first 10 patients with neurogenic fecal incontinence due to spina bifida. The surgical procedure and the results are reported. METHODS: Since November 1991, we have used a surgical procedure similar to the appendiceal continence mechanism in urinary diversion to establish a continent colonic cutaneous stoma for antegrade enemas in 10 myelodysplastic patients (4 females, 6 males; median age 13.2 years [range 6 to 26]) with severe neurogenic fecal incontinence. The average follow-up is now 26.4 months (range 12.5 to 50). All patients had neurogenic bladder dysfunction successfully managed by clean intermittent catheterization, anticholinergic drugs, or artificial sphincter implantation. The surgical technique for fecal incontinence included the partial orthotopic submucosal imbedding of the appendix into a cecal tenia and the fixation of the ileocecal region at the inner side of the abdominal wall after creation of an appendicocutaneous catheterizable stoma. RESULTS: All patients reached fecal continence for at least 38 hours (median 45.3) by using antegrade colonic enemas with 1.5% saline solution (n = 9) or GoLYTELY solution (n = 1), 0.5 to 1.5 L every 2 to 3 days. All other therapies (diet, oral medication, rectal purgative, or enema) to reach fecal continence had previously failed. There were only two complications seen at the follow-up. One boy with an artificial urinary sphincter presented with infection of the sphincter system, which led to explantation. Another boy presented 15 months after creation of the colonic appendiceal stoma with saline intoxication possibly due to a homemade saline solution. CONCLUSIONS: We conclude that the antegrade colonic enema via an orthotopic continent appendiceal stoma is a safe and highly effective treatment modality for fecal incontinence in patients with neurogenic bowel dysfunction if nonsurgical management has failed.     

Effect of extradural analgesia on the paradoxical arousal response of the electroencephalogram

Nineteen patients with Duchenne's muscular dystrophy underwent segmental spinal instrumentation and posterior fusion between 1989 and 1994. The indication for surgery was loss of the ability to walk and development of scoliosis with sitting discomfort. Preoperative assessment included evaluation of pulmonary function. Average age at operation was 12.5 years. Instrumentation and fusion extended from upper thoracic levels to L-5 or the sacrum. A Hartshill rectangle was used in all cases, with banked allograft bone. Severe intraoperative blood loss was avoided by use of hypotensive anaesthesia. Peroperatively, systolic blood pressure was maintained between 75 and 85 mm Hg. Average blood loss was 1,246 ml (range, 400-3,100) or 30% of estimated total blood volume. Average transfusion requirements were 3 units of packed cells. Postoperative analgesia was provided by infusion via an epidural catheter. There were no postoperative wound or chest infections. Three patients required catheterisation for urinary retention. Postoperatively patients were fitted with a Neofract jacket to allow early mobilisation and discharge. Mean postoperative length of stay was 16 days. Posterior spinal fusion by using the Hartshill rectangle provided good correction and fixation. Hypotensive anaesthesia permitted surgery to be performed rapidly in a relatively dry field and avoided the complications of severe intraoperative blood loss and massive transfusion.     

The AMS 800 sphincter in urinary incontinence after prostatic surgery. Apropos of 16 patients

OBJECTIVE: To present the results of a series of artificial sphincters implanted for urinary incontinence after prostatic surgery. PATIENTS AND METHODS: From 1987 to 1996, artificial sphincters were implanted for urinary incontinence after prostatic surgery, consisting of one transvesical prostatectomy, 11 prostatic resections and 4 radical prostatectomies. All these patients were evaluate by urine culture, cystourethrography and urethrocystoscopy. Eleven patients underwent urodynamic assessment. The AMS 800 artificial sphincter was used. RESULTS: The mean interval between onset of incontinence and implantation was 18 months. The mean follow-up after implantation of the sphincter was 6 years. An operative wound of the bulbar urethra did not prevent implantation of the sphincter, but required repair and prolonged catheterization (12 days). The mean duration of catheterization was 6 days. The overall functional result was 81% (13 case), and 75% of sphincters were functional at 5 years. Five revisions were necessary to maintain functioning of the sphincter in 2 cases of rupture and 1 displacement of the balloon, 1 case of urethral atrophy and 1 rupture of the pump. Three explanations, 2 total and 1 partial, were performed. Two reimplantations were performed after 12 months and 26 months respectively. CONCLUSION: The AMS 800 artificial sphincter currently represents an effective treatment for urinary incontinence due to sphincter insufficiency after prostatic surgery. However, an old artificial sphincter may require revision to restore function.     

Reflex electrical stimulation for urinary incontinence

Our previous observations have shown that the electrical stimulation of muscles is prevalently reflex. One of the advantages of reflex stimulation is that it activates not only a limited number of motor units, but rather a number of muscles connected by the same reflex from a single stimulation site. Consequently, it is not necessary to place electrodes into the muscle to be activated. They can be put elsewhere provided that the same effect is obtained and that it is more convenient for the patient. Such an opportunity arises when treating urinary incontinence which involves not only the urethral sphincter but also the group of synergistic muscles of the pelvic floor. Our experiments with several patients suffering stress incontinence have shown that indirect stimulation of the levator ani with a vaginal stimulator and especially of the anal sphincter with an anal stimulator affects the urethral sphincter in the same way as direct stimulation. These findings are significant since they enable us to use external instead of implantable stimulators. External stimulation is worth trying in all cases of stress incontinence where conservative measures have failed. In our cases, the results have been very satisfactory.     

Stress urinary incontinence in women caused by severe sphincter hypotonia. Goebbel-Stoeckel intervention as alternative to artificial sphincter. Apropos of 26 cases treated between January 1985 and January 1994

Stress urinary incontinence with low urethral closure pressure and urethral mobility is often treated by artificial urinary sphincter. Our retrospective report in 19 patients evaluates the sling procedure as an alternative to the artificial urinary sphincter (7 patients). All patients had a preoperative clinical and urodynamic evaluation. 13 patients were continent (68.4%) in the sling procedure group and 5 in the sphincter group. Continence remained stable with a mean follow-up of 77 months (range: 39-110 months). 2 patients had urgency and none had dysuria. The sling procedure gave us the same results as sphincter with less morbidity.     

Results of artificial sphincter in severe anal incontinence. Report of 14 consecutive implantations

PURPOSE AND METHODS: Fourteen AMS 800 (American Medical Systems, Minneapolis, MN) urinary artificial sphincters have been consecutively implanted in 13 patients with total incontinence for stool of various causes (traumatic or postoperative, 7; congenital, 3; neurologic, 2; idiopathic, 1). No proximal stoma was constructed but was already present in one patient before implantation. RESULTS: Sepsis occurred in two patients. Removal of sphincter and colostomy was necessary in three patients: one of these two had developed sepsis, one had perineal ulceration before activation of the sphincter in a severely scarred perineum, and one had severe pain in a radiation-injured anorectum. Sphincter-related failure occurred once by rupture of the cuff in a constipated woman after two years of satisfactory function. Reimplantation of a new cuff restored normal continence in this patient. After median follow-up of 20 (range, 4-60) months, nine of ten patients with a functioning sphincter were continent for stool, and five were also continent for gas. Failure occurred in one patient because the cuff was too large to occlude the anal canal. This patient is awaiting reimplantation. Four patients experienced easily controlled difficulties with evacuation of feces. Anal pressure with inflated cuff varied from 43 to 94 (mean, 58 +/- 12) cm H2O. CONCLUSION: These results show that an artificial sphincter has a role in the treatment of severe anal incontinence when local therapies are not applicable or have failed.     

Glutaraldehyde cross-linked collagen in the treatment of faecal incontinence

BACKGROUND: The treatment of faecal incontinence secondary to internal anal sphincter dysfunction is unsatisfactory. The aim of the study was to evaluate the efficacy of anal glutaraldehyde cross-linked (GAX) collagen injections in patients with a surgically incorrectable disorder. METHODS: Seventeen patients were studied: nine had idiopathic faecal incontinence, three had incontinence following haemorrhoidectomy, two following internal sphincterotomy, two following an internal sphincter defect from obstetric injury and one following treatment for fistula in ano. All patients were refractory to conservative treatment and were unsuitable for surgical repair. All had anorectal physiology and endoanal ultrasonography before and after GAX collagen injections. RESULTS: All patients tolerated the injection without side-effects. All patients had an intact external anal sphincter. Following injection, 11 patients showed marked symptomatic improvement. One patient reported symptomatic improvement but remained in clinical grade 3, and two reported minimal improvement. There was no improvement in three patients, but one of these had a repeat injection and showed significant improvement subsequently. CONCLUSION: Injection of GAX collagen in the anal canal is a simple and well tolerated method of treating faecal incontinence due to internal sphincter dysfunction. Early results suggest it provides an easy and reliable alternative to the currently available methods that are often unsuccessful and at best unpredictable.     

Dielectric behaviour of dry synthetic polypeptides

Histological and histochemical techniques were employed in a study of the spinal muscles from 35 adolescent scoliotic patients. Many abnormalities were found and, in general, the changes were indicative of denervation and neuropathy. None of the abnormalities were found at any one site more frequently than at any other, nor were they more often seen on one side or the other. None of the patients examined were normal at all sites of biopsy. These findings provide evidence of a neuropathic process being involved in the aetiology or progression of scoliosis. So-called "idiopathic" scoliosis may, perhaps, be the result of an otherwise sub-clinical lower motor neurone disorder.     

Signs of vagal reinnervation 4 years after heart transplantation in spectra of heart rate variability

OBJECTIVES: Transurethral injection of glutaraldehyde cross-linked bovine collagen has recently been advocated as a potentially useful treatment modality for management of urinary incontinence. The reported clinical experience with urethral collagen injection in adult males has been limited. METHODS: This study summarizes the current literature and reviews the clinical results of collagen injection in a group of 25 men with incontinence after either transurethral or radical prostatectomy. RESULTS: The overall results in this series were disappointing. Only 2 patients (8%) achieved significant improvement with this treatment. Eight patients (32%) experienced minimal improvement in symptoms, and 15 (60%) remained incontinent with no improvement in symptoms after collagen injection. The number of injection procedures and volume of collagen material implanted did not correlate with clinical outcome. Five patients (20%) have subsequently required placement of an artificial urinary sphincter to control their incontinence. CONCLUSIONS: We conclude that transurethral injection of glutaraldehyde cross-linked bovine collagen has a limited role in the management of urinary incontinence in adult men after prostatectomy.     

Responses of natural killer cell activity to acute laboratory stressors in healthy men at different times of day

BACKGROUND: Five renal recipients with neurovesical dysfunction (NVD) were retrospectively reviewed focusing on anatomical and urodynamic abnormalities of the lower urinary tract and their management prior to kidney transplantation. METHODS: The underlying anomalies in these 5 patients were a posterior urethral valve (1 with an imperforate anus; n = 2), meningomyelocele (n = 2) and a congenital short urethra with an imperforate anus (n = 1). Their urinary tracts were evaluated prior to transplantation with voiding cystourethrography, urethrocystoscopy, cystometrography and electromyography of the external urethral sphincter to identify a possible focus of urinary tract infection, urine storage and voiding function. RESULTS: All 5 patients had NVD proven by urodynamic studies or by documentation of urinary retention in the absence of mechanical outlet obstruction. Bilateral high grade vesicoureteral reflux was noted in all patients, requiring ureteroneocystostomy. Clean intermittent catheterization (CIC) was ultimately employed for bladder emptying in all patients. Two patients with poor bladder compliance underwent augmentation cystoplasty before transplantation. The Mitrofanoff procedure was used in 2 patients with structural urethral abnormalities to access the bladder for catheterization. After eradication of possible sources of infection and establishment of a low-pressure urine storage system with bladder emptying by CIC, kidney transplantation was performed. Following kidney transplantation, all of the recipients were asymptomatic for urinary tract infections using CIC. Although 1 patient lost his graft due to chronic rejection, the other 4 other patients have good renal function. CONCLUSION: Kidney transplantation in patients with NVD can be performed provided that their urinary tract problems are properly resolved.     

Selective loss of peripheral blood CD45RO+ T lymphocytes correlates with increased levels of serum cytokines and endothelial cell-derived soluble cell adhesion molecules in patients with chronic idiopathic neutropenia of adults

The cause of urinary or fecal incontinence may be a complex one. A central or peripheral neurogenic lesion is frequently found to be accounting for the problem. The diagnosis should be specified by neurologic examinations and adequate neurophysiologic workup prior to any conservative or surgical treatment. Of particular expressiveness are, neurological history, both neurography of the pudendal nerve and electromyography of the external anal sphincter. Additional measures such as recording of the motor or sensory potentials, determination of reflex latencies and an electromyogram of other striated muscles of the pelvic floor may be taken as needed. Close co-operation of gynecologists, proctologists, and neurophysiologically trained neurologists will help to improve the prospects of treatment and avoid inappropriate therapeutic approaches.