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Background: In the current consensus diagnostic criteria, the diagnosis of probable multiple system atrophy (MSA) is based solely on clinical findings, whereas neuroimaging findings are listed as aid for the diagnosis of possible MSA. There are overlapping phenotypes between MSA-parkinsonian type and Parkinson's disease, progressive supranuclear palsy, and dementia with Lewy bodies, and between MSA-cerebellar type and sporadic adult-onset ataxia resulting in a significant diagnostic delay and misdiagnosis of MSA during life., Objectives: In light of an ongoing effort to revise the current consensus criteria for MSA, the Movement Disorders Society Multiple System Atrophy Study Group performed a systematic review of original articles published before August 2019., Methods: We included articles that studied at least 10 patients with MSA as well as participants with another disorder or control group for comparison purposes. MSA was defined by neuropathological confirmation, or as clinically probable, or clinically probable plus possible according to consensus diagnostic criteria., Results: We discuss the pitfalls and benefits of each diagnostic test and provide specific recommendations on how to evaluate patients in whom MSA is suspected., Conclusions: This systematic review of relevant studies indicates that imaging and autonomic function tests significantly contribute to increasing the accuracy of a diagnosis of MSA., (© 2020 International Parkinson and Movement Disorder Society.)

Competing Interests: Competing interests: None declared.

Lower urinary tract (LUT) dysfunction presents early in multiple system atrophy (MSA), usually initially as urinary urgency, frequency and incontinence, and voiding difficulties/urinary retention becomes apparent over time. We have observed a subset of patients who instead presented initially with urinary retention requiring catheterisation. At presentation, these patients had only subtle neurological signs that would not fulfil the diagnostic criteria of MSA; however, the anal sphincter electromyography (EMG) was abnormal and they reported bowel and sexual dysfunction, suggesting localisation at the level of the sacral spinal cord. They subsequently developed classical neurological signs, meeting the diagnostic criteria for probable MSA. One patient was confirmed to have MSA at autopsy. We postulate that in a subset of patients with MSA, the disease begins in the sacral spinal cord and then spreads to other regions resulting in the classical signs of MSA. The transmissibility of alpha-synuclein has been demonstrated in animal models and the spread of pathology from sacral cord to other regions of the central nervous system is therefore plausible. Patients presenting with urinary retention and mild neurological features would be an ideal group for experimental trials evaluating neuroprotection in MSA.

Purpose of Review: This article provides an overview of the clinical presentation, investigations, and treatment options for lower urinary tract and bowel dysfunction in patients with neurologic diseases., Recent Findings: The site of the neurologic lesion influences the pattern of lower urinary tract dysfunction. Antimuscarinic agents are first-line management for urinary incontinence; however, the side effect profile should be considered when prescribing them. β3-Receptor agonists are a promising alternative oral medication. Botulinum toxin injections into the detrusor have revolutionized the management of neurogenic detrusor overactivity.Bowel dysfunction commonly presents as constipation and fecal incontinence. Gastrointestinal emergencies may arise, including intestinal pseudoobstruction, intussusception, volvulus, and stercoral ulcer (ulcer of the colon due to pressure and irritation resulting from severe, prolonged constipation). Bowel function tests in neurologic patients often show a combination of slow transit and anorectal dysfunction. Management for slow transit constipation includes bulking agents, softening agents, yogurt/probiotics, and prokinetic agents. Suppositories, botulinum toxin injections, and transanal irrigation are options for managing anorectal constipation., Summary: Functions of the lower urinary tract and bowel are commonly affected in neurologic disease. Neurologists play an important role in assessing lower urinary tract and bowel symptoms in their patients and planning treatment strategies, often in collaboration with specialist teams.

Purpose: Percutaneous tibial nerve stimulation is a minimally invasive neuromodulation technique for treating overactive bladder symptoms. The aim of this study was to assess safety, efficacy and impact on quality of life of percutaneous tibial nerve stimulation in neurological patients reporting overactive bladder symptoms., Methods: In this retrospective evaluation over 18 months at a tertiary healthcare centre, patients finding first-line treatments for overactive bladder ineffective or intolerable underwent a standard 12-week course of percutaneous tibial nerve stimulation (Urgent PC, Uroplasty). Symptoms were evaluated using standardised International Consultation on Incontinence Questionnaires and bladder diaries., Results: Of 74 patients (52 women, 22 men, mean age 56 years), 49 (66.2%) patients had neurological disorder [19 (25.7%) multiple sclerosis and 30 (40.5%) other neurological conditions] and 25 (33.8%) idiopathic overactive bladder. Overall for the entire cohort significant improvements were recorded after 12 weeks in the following domains: 24-h frequency on bladder diary - 1.67 (- 3.0, 0.33) (p = 0.002), number of incontinent episodes on bladder diary - 0.0 (- 1, 0) (p = 0.01), incontinence severity on bladder diary 0 (- 0.33, 0) (p = 0.007), OAB symptoms - 3 (- 11.5, 5) (p = 0.01), and quality of life - 16 (- 57, 6.5) (p = 0.004). There were no significant differences in outcomes between patients with idiopathic and neurogenic overactive bladder., Conclusions: Percutaneous tibial nerve stimulation appears to be a possible promising alternative for patients with neurological disorder reporting overactive bladder symptoms who find first-line treatments either ineffective or intolerable. However, a properly designed study is required to address safety and efficacy.

Purpose of Review: Multiple sclerosis (MS) is the most frequent neuroinflammatory disease of the central nervous system and is commonly associated with lower urinary tract (LUT) dysfunction. As a consequence, health-related quality of life is often impaired and the upper urinary tract might be at risk for damage. The aim of this review is to give an overview of current treatment options for LUT dysfunction in patients with MS., Recent Findings: The treatment is tailored to the type of dysfunction-storage or voiding dysfunction-beginning with conservative treatment options and ending with invasive therapies and surgery. Additionally, alternative options, e.g., different intravesical therapies or cannabinoids, have been evaluated in recent years with promising results. Current available therapies offer different possible treatments for LUT dysfunction in patients with MS. They address either voiding or storage dysfunction and therefore ameliorate LUT symptoms improve quality of life and protect the upper urinary tract.

Purpose of Review: Lower urinary tract and sexual dysfunction are commonly reported sequelae of neurologic disease and have a significant impact on quality of life. This article presents an up-to-date summary of the clinical presentation, investigations, and treatment options for urogenital dysfunction in patients with neurologic disease., Recent Findings: The pattern of lower urinary tract dysfunction is influenced by the site of the neurologic lesion. The risk for developing upper urinary tract damage is considerably less in patients with slowly progressive nontraumatic neurologic disorders, as compared to those with spinal cord injury or spina bifida. Investigations such as urinalysis, ultrasonography, and urodynamics provide further information about the cause and nature of lower urinary tract dysfunction. Antimuscarinic agents are the first-line management of urinary incontinence; however, side effect profile and anticholinergic burden should be considered when prescribing medications. β3-Adrenergic receptor agonists are a promising alternative oral medication. Tibial and sacral neuromodulation have been shown to be effective for managing incontinence, as well as urinary retention due to Fowler syndrome. Intradetrusor injections of onabotulinumtoxinA have revolutionized the management of neurogenic detrusor overactivity., Summary: Neurologic patients reporting urogenital symptoms require a comprehensive evaluation for planning a patient-tailored approach to management. Neurologists are increasingly inquiring about lower urinary tract and sexual functions and becoming involved in the management of these disorders.

The lower urinary tract (LUT) in health is regulated by coordinated multi-level neurological inputs which require an intact central and peripheral nervous system. Lower urinary tract dysfunction is, therefore, a common sequelae of neurological disease and the patterns of bladder storage and voiding dysfunction depend upon the level of neurological lesion. Evaluation includes history taking, bladder diary, urological examination when relevant, ultrasonography and urodynamic testing when indicated. Antimuscarinic agents are the first line treatment for patients with storage dysfunction. Alternative treatments include intradetrusor injection of onabotulinumtoxinA, which has been shown to be of benefit in patients with neurogenic detrusor overactivity (NDO), and neuromodulation. Intermittent catheterization remains the option of choice in patients with significant voiding dysfunction resulting in high post-void residual volumes.

Opinion Statement: Urogenital dysfunction is commonly reported in Parkinson's disease (PD), and history taking and a bladder diary form the cornerstone of evaluation. The assessment of lower urinary tract (LUT) symptoms include urinalysis, ultrasonography, and urodynamic studies and help to evaluate concomitant urological pathologies such as benign prostate enlargement. Antimuscarinic medications are the first line treatment for overactive bladder (OAB) symptoms and solifenacin has been specifically studied in PD. Antimuscarininc drugs may exacerbate PD-related constipation and xerostomia, and caution is advised when using these medications in individuals where cognitive impairment is suspected. Desmopressin is effective for the management of nocturnal polyuria which has been reported to be common in PD. Intradetrusor injections of botulinum toxin have been shown to be effective for detrusor overactivity, however, are associated with the risk of urinary retention. Neuromodulation is a promising, minimally invasive treatment for PD-related OAB symptoms. Erectile dysfunction is commonly reported and first line treatments include phosphodiesterase-5 inhibitors. A patient-tailored approach is required for the optimal management of urogenital dysfunction in PD., Competing Interests: Amit Batla and Natalie Tayim each declare no potential conflicts of interest. Mahreen Pakzad has been a speaker for Astellas. Jalesh N. Panicker has received royalties from Cambridge University Press, has been involved in trials supported by FirstKind Ltd, Allergan and Ipsen and has received speaker honoraria from Wellspect, Astellas and Allergan. Human and Animal Rights and Informed Consent This article does not contain any studies with human or animal subjects performed by any of the authors.

Background: Urinary tract infections (UTIs) are commonly reported by people with multiple sclerosis (PwMS) and significantly impact quality of life., Objective: To provide an overview of the problem of UTIs in PwMS and offer a practical approach for the diagnosis and management., Methods: A review of the literature through a Pubmed search up to October 2015 was performed using the following keywords: multiple sclerosis, neurogenic bladder, urinary tract infections, relapse, dipsticks, culture, recurrent and prevention., Results: Noteworthy topics include the definition of a confirmed symptomatic UTI as a positive urine culture defined by >10(5) colony-forming units (CFU)/mL or >10(4) CFU/mL if a urethral catheter urine sample is taken, or any count of bacteria in a suprapubic bladder puncture specimen, both in addition to symptoms including fever, pain, changes in lower urinary tract symptoms or neurological status. Urinalysis is useful to exclude a UTI; however, on its own is insufficient to confirm a UTI, for which urine culture is required. Experts advise asymptomatic UTIs should not be treated except in the context of an acute relapse. From international guidelines, there is no validated strategy to prevent recurrent UTIs in PwMS., Conclusion: This review provides an overview of the diagnosis, treatment and prevention of UTIs in the setting of multiple sclerosis (MS)., (© The Author(s), 2016.)

Objectives: To assess the efficacy (defined as improvements in maximum urinary flow rate [Qmax ] of ≥50%, post-void residual urine volume [PVR] and scores on the International Prostate Symptom Score [IPSS] questionnaire) and safety of urethral sphincter injections of onabotulinumtoxinA in women with a primary disorder of urethral sphincter relaxation, characterised by an elevated urethral pressure profile (UPP) and specific findings at urethral sphincter electromyography (EMG), i.e. Fowler's syndrome., Patients and Methods: In this open-label pilot Institutional Review Board-approved study, 10 women with a primary disorder of urethral sphincter relaxation (elevated UPP, sphincter volume, and abnormal EMG) presenting with obstructed voiding (five) or in complete urinary retention (five) were recruited from a single tertiary referral centre. Baseline symptoms were assessed using the IPSS, and Qmax and PVR were measured. After 2% lidocaine injection, 100 U of onabotulinumtoxinA was injected into the striated urethral sphincter, divided on either side, under EMG guidance. Patients were reviewed at 1, 4 and 10 weeks after injection, and assessed using the IPSS, Qmax and PVR measurements. The UPP was repeated at week 4., Results: The mean (range) patient age was 40 (25-65) years, and the mean symptom scores on the IPSS improved from 25.6 to 14.1, and the mean 'bother' score reduced from 6.1 to 3.5 at week 10. As compared with a baseline mean Qmax of 8.12 mL/s in the women who could void, the Qmax improved to 15.8 mL/s at week 10. Four of the five women in complete retention could void spontaneously, with a mean Qmax of 14.3 mL/s at week 10. The mean PVR decreased from 260 to 89 mL and the mean static UPP improved from 113 cmH2 O at baseline to 90 cmH2 O. No serious side-effects were reported. Three women with a history of recurrent urinary tract infections developed a urinary tract infection. There were no reports of stress urinary incontinence. Seven of the 10 women opted to return for repeat injections., Conclusions: This pilot study shows an improvement in patient-reported lower urinary tract symptoms, and the objective parameters of Qmax , PVR and UPP, at 10 weeks after urethral sphincter injections of onabotulinumtoxinA. No serious side-effects were reported. This treatment could represent a safe outpatient treatment for young women in retention due to a primary disorder of urethral sphincter relaxation. However, a larger study is required to confirm the findings of this pilot study., (© 2015 The Authors BJU International © 2015 BJU International Published by John Wiley & Sons Ltd.)

Lower urinary tract (LUT) dysfunction is common in patients with multiple sclerosis and is a major negative influence on the quality of life of these patients. The most commonly reported symptoms are those of the storage phase, of which detrusor overactivity is the most frequently reported urodynamic abnormality. The clinical evaluation of patients' LUT symptoms should include a bladder diary, uroflowmetry followed by measurement of post-void residual urine volume, urinalysis, ultrasonography, assessment of renal function, quality-of-life assessments and sometimes urodynamic investigations and/or cystoscopy. The management of these patients requires a multidisciplinary approach. Intermittent self-catheterization is the preferred option for management of incomplete bladder emptying and urinary retention. Antimuscarinics are the first-line treatment for patients with storage symptoms. If antimuscarinics are ineffective, or poorly tolerated, a range of other approaches, such as intradetrusor botulinum toxin A injections, tibial nerve stimulation and sacral neuromodulation are available, with varying levels of evidence in patients with multiple sclerosis. Surgical procedures should be performed only after careful selection of patients. Stress urinary incontinence owing to sphincter deficiency remains a therapeutic challenge, and is only managed surgically if conservative measures have failed. Multiple sclerosis has a progressive course, therefore, patients' LUT symptoms require regular, long-term follow-up monitoring.

Lower urinary tract (LUT) dysfunction is common in multiple sclerosis (MS), and has a considerable impact on quality of life. It most often results from involvement of the spinal cord, which results in detrusor overactivity and detrusor sphincter dyssynergia. LUT symptoms may change with time, paralleling the dynamic course of MS, and therefore the need for regular follow-up assessments is essential. A formal evaluation includes history taking, measurement of the postvoid residual volume (PVR), testing for urinary tract infections and urodynamic studies in select cases. If the PVR is elevated, incomplete bladder emptying is best managed by intermittent self-catheterization. Several options exist for managing the overactive bladder, including antimuscarinics, desmopressin, tibial nerve stimulation, and botulinum toxin A. A stepwise approach is adopted for managing LUT dysfunction in MS., (© 2015 Elsevier B.V. All rights reserved.)

Objective: To assess the morbidity, mortality, and impact on quality of life and renal function after laparoscopic cystectomy and ileal conduit in patients with multiple sclerosis with lower urinary tract symptom refractory to conservative management., Materials and Methods: A prospective study was conducted between February 2004 and December 2010 on 44 consecutive patients with multiple sclerosis who underwent laparoscopic cystectomy and ileal conduit for lower urinary tract symptom. Median Expanded Disability Status Scale score was 8 (6.5-8.5). Mean duration of multiple sclerosis was 19.3 ± 7.9 years. The quality of life was determined using the validated Qualiveen questionnaire preoperatively and at minimum 6 months after the surgery., Results: No conversion to open surgery was required. Postoperative morbidity rate was 18.2%; minor (Clavien ≤ 2) and major (Clavien ≥ 3) complications occurred in 13.6% and 6.8%, respectively. Mean follow-up was 44.5 ± 20.6 months. Complications noted were asymptomatic ureteroileal stenosis (n = 6) and pyelonephritis (n = 3). Neurological status and Expanded Disability Status Scale score remained stable throughout. Renal function remained unchanged. Limitations, constraints, and specific urinary impact index subscores of the Qualiveen were significantly improved at 6 months time., Conclusion: Laparoscopic cystectomy and ileal conduit for lower urinary tract symptom in advanced multiple sclerosis is a safe procedure with low complications. Neurological status and renal function remain stable and quality of life improves and continues to remain improved during long-term follow-up, suggesting this to be an attractive option in patients with advanced multiple sclerosis with lower urinary tract symptom refractory to conservative treatment., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Purpose: Urinary retention in women often presents a diagnostic difficulty, and the etiology may remain unidentified even after excluding structural and neurological causes. We evaluated a group of women referred to a specialist center with unexplained urinary retention., Materials and Methods: A total of 61 consecutive women with complete urinary retention were evaluated. Urological and neurological investigations locally had failed to identify a cause. Urethral pressure profile, sphincter volume measurement and in some cases urethral sphincter electromyography were performed to diagnose a primary disorder of sphincter relaxation (Fowler's syndrome)., Results: Mean patient age was 39 years (range 18 to 88). Following investigations, a probable etiology was identified in 25 (41%) women, the most common being Fowler's syndrome. Of the women 24 (39%) were being treated with opiates for various pain syndromes and in 13 no other cause of retention was identified. Opiates could be discontinued in only 2 patients, and both demonstrated improved sensations and voiding., Conclusions: The cause of urinary retention may remain unknown in spite of extensive investigations. Young women regularly using prescription opiates for various undiagnosed pain syndromes present a challenging clinical problem and this study suggests that iatrogenic causes should be considered if voiding difficulties emerge. An association between opiate use and constipation is well-known and, although urinary retention is a listed adverse event, it appears to be often overlooked in clinical practice. It is hypothesized that Fowler's syndrome is due to an up-regulation of spinal cord enkephalins and that exogenous opiates may compound any functional abnormalities predisposing young women to urinary retention., (Copyright © 2012 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

This article reviews the neurologic conditions associated with a high prevalence of bladder dysfunction and about which significant advances in understanding have occurred in recent years. The importance of the frontal lobes for bladder control has been confirmed through functional brain imaging, and recent findings in the elderly with incontinence suggest the problem may result from disconnection of important frontal areas caused by white matter disease. The very different urologic profile of the two sometimes-confused conditions, multiple system atrophy and Parkinson's disease, is clarified. The advances in treatments for multiple sclerosis in recent years have been remarkable and are briefly described., (Copyright © 2010 Elsevier Inc. All rights reserved.)