High Prevalence of Psychological Comorbidities and Functional Neurological Symptoms in Women With Urinary Retention.

PURPOSE: Chronic idiopathic urinary retention (CIUR) in young women is poorly understood and a probable etiology is established only in around 40%, most commonly a primary disorder of external urethral sphincter relaxation, sometimes referred to as Fowler's syndrome. A high prevalence of psychological and functional comorbidities is reported, however these have been poorly characterized. MATERIALS AND METHODS: Women consecutively referred for the assessment and management of CIUR were evaluated cross-sectionally for 13 psychological/behavioral domains using a structured clinical interview: depression, anxiety, post-traumatic stress disorder (PTSD), other psychiatric history, functional neurological disorder, other functional syndromes, childhood and adult trauma, personality disorder, and self-harm (ever/current). RESULTS: A total of 91 women (mean age [SD]: 34 [11] years) were evaluated. Women with Fowler's syndrome (n = 69) were younger (mean age [SD]: 32 [9] vs 40 [13] years) than women without Fowler's syndrome and reported shorter mean duration of urinary symptoms (mean [SD]: 5 [6] vs 10 [9]). A high prevalence of psychiatric and psychological comorbidities was reported (97%) including current depression (77%), current anxiety (78%), and PTSD (32%). A high prevalence of functional neurological disorder (56%) and other functional symptoms (65%) was also reported. Self-harm was reported in (14%) and personality disorder in 16%. Childhood trauma was reported in 35% of women. CONCLUSIONS: Young women with CIUR report a high burden of psychiatric disorders, affective symptoms, trauma, PTSD, self-harm, and functional neurological disorder, particularly in those with Fowler's syndrome. These factors can undermine the engagement with health care professionals and affect management and should therefore be addressed during the urological assessment.

The impact of bladder problems on well-being in multiple sclerosis - A cross-sectional study.

BACKGROUND: Persons with multiple sclerosis (pwMS) frequently suffer from bladder problems that are not identified and managed optimally, reducing quality of life and increasing risk of health consequences. OBJECTIVE: This study aimed to investigate associations between bladder problems and well-being of pwMS. METHODS: The study included 1872 pwMS from France, Germany, Italy, and the UK self-reporting on demographics, MS status, bladder problems and management, and well-being. Logistic regression analyses were performed to investigate associations with self-reported well-being. RESULTS: Population mean age was 51 years and 79 % were women. Among pwMS, 55 % reported bladder problems indicating overactive bladder (OAB), and 40 % reported self-experienced urinary retention. Self-management of bladder problems was reported by 32 %, 33 % were yet to have problem resolution, and 45 % reported a wish to receive bladder management help. Additionally, 35 % of pwMS reported that bladder problem onset preceded awareness of a link to MS. OAB, problem self-management, and urinary complications were significantly associated with lower well-being. CONCLUSION: Bladder problems affect pwMS across disease subtypes and many attempt self-management. Consequently, pwMS with bladder problems are more likely to experience lower well-being, suggesting an unmet need. Raising awareness of the link between bladder problems and well-being could benefit pwMS living with bladder problems.

Hypersexuality in neurological disorders: A systematic review.

BACKGROUND: Hypersexuality (HS) accompanying neurological conditions remains poorly characterized despite profound psychosocial impacts. Objective We aimed to systematically review the literature on HS in patients with neurological disorders. Study selection and analysis We conducted a systematic review to identify studies that reported HS in neurological disorders. HS was defined as a condition characterized by excessive and persistent preoccupation with sexual thoughts, urges, and behaviors that cause significant distress or impairment in personal, social, or occupational functioning. Data on demographics, assessment techniques, associated elements, phenotypic manifestations, and management strategies were also extracted. Findings The final analysis included 79 studies on HS, encompassing 32 662 patients across 81 cohorts with neurological disorders. Parkinson's disease was the most frequently studied condition (55.6%), followed by various types of dementia (12.7%). Questionnaires were the most common assessment approach for evaluating HS, although the techniques varied substantially. Alterations in the dopaminergic pathways have emerged as contributing mechanisms based on the effects of medication cessation. However, standardized treatment protocols still need to be improved, with significant heterogeneity in documented approaches. Critical deficiencies include risks of selection bias in participant sampling, uncontrolled residual confounding factors, and lack of blinded evaluations of reported outcomes. Conclusions and clinical implications Despite growth in the last decade, research on HS remains limited across neurological conditions, with lingering quality and methodological standardization deficits. Key priorities include advancing assessment tools, elucidating the underlying neurobiology, and formulating management guidelines. PROSPERO REGISTRATION NUMBER: CRD42017036478.

Detrusor Sphincter Dyssynergia.

Detrusor sphincter dyssynergia (DSD) is defined as a detrusor contraction concurrent with an involuntary contraction of the urethral and/or periurethral striated muscles typically occurring in a patient with a spinal cord lesion above the sacral cord. Consequently, high urethral closure pressures during the detrusor contraction leads to high intravesical voiding pressure and large postvoid residuals, which can lead to significant complications in up to 50% of patients if DSD is not treated and followed-up regularly. DSD treatment options are centered around symptomatic management rather that addressing the underlying causative mechanisms.

MS bladder check tool: Development and validation of a patient awareness tool to facilitate timely management of lower urinary tract dysfunction due to multiple sclerosis.

BACKGROUND: Lower urinary tract dysfunction impacts quality of life of people with MS; often, symptoms are 'lived with' or deprioritised by healthcare providers (HCPs). Consequently, patients must be given the skills they need to become confident in managing their illness and enhance their involvement in the process. OBJECTIVE: To develop and validate a self-assessment tool to help people with MS become more aware of their bladder symptoms and prompt contact with their HCP to facilitate timely management and specialist referral, if required. METHODS: The 'MS bladder check tool' was developed by a multidisciplinary panel of specialist advisors. Consensus meetings and pilot testing were conducted to design and evolve the tool into a series of nine questions, using population-appropriate language, and covering all aspects of bladder dysfunction in MS. The tool was then validated by an international, multidisciplinary team of experts. RESULTS: Validity was rated 'excellent' for all questions indicating that the MS bladder check tool is an appropriate method of highlighting bladder problems in people with MS. CONCLUSION: The MS bladder check tool is simple, easy-to-use, and empowers patients to take charge of their urinary tract health, aiming to improve the management of MS and, ultimately, patient quality of life.

Sexual dysfunction and commonly used drugs in neurology.

Sexual dysfunction is common in men and women with neurological diseases. Medications used in neurology can cause sexual dysfunction independently of the disease process and this may adversely affect patients' quality of life. This review focuses on medications commonly prescribed to neurological patients that may contribute to altered sexual function, and discusses how they may differ in men and women.

Abnormal dopamine transporter imaging in pure autonomic failure: a potential biomarker of central nervous system involvement.

BACKGROUND AND PURPOSE: Pure autonomic failure (PAF) is a rare progressive neurodegenerative disease characterized by neurogenic orthostatic hypotension at presentation, without other neurological abnormalities. Some patients may develop other central neurological features indicative of multiple system atrophy or a Lewy body disorder. There are currently no biomarkers to assess possible central nervous system involvement in probable PAF at an early stage. A possibility is to evaluate the nigrostriatal dopaminergic degeneration by imaging of dopamine transporter with DaTscan brain imaging. The objective was to evaluate subclinical central nervous system involvement using DaTscan in PAF. METHODS: We retreospectively reviewed pure autonomic failure patients who were evaluated at the Autonomic Unit between January 2015 and August 2021 and underwent comprehensive autonomic assessment, neurological examination, brain magnetic resonance imaging and DaTscan imaging. DaTscan imaging was performed if patients presented with atypical features which did not meet the criteria for Parkinson's disease or multiple system atrophy or other atypical parkinsonism. RESULTS: In this cohort, the median age was 49.5 years at disease onset, 57.5 years at presentation, and the median disease duration was 7.5 years. Five of 10 patients had an abnormal DaTscan without neurological features meeting the criteria of an alternative diagnosis. Patients with abnormal DaTscan were predominantly males, had shorter disease duration and had more severe genitourinary symptoms. DISCUSSION: Degeneration of nigrostriatal dopaminergic neurons measured using DaTscan imaging can present in patients with PAF without concurrent signs indicating progression to widespread α-synucleinopathy. It is advocated that DaTscan imaging should be considered as part of the workup of patients with emerging autonomic failure who are considered to have PAF.

The Impact of Polypharmacy on Management of Lower Urinary Tract Symptoms in Parkinson's Disease.

Lower urinary tract (LUT) symptoms are a common presentation of autonomic dysfunction in Parkinson's disease (PD). Symptoms significantly impact quality of life and are associated with worsening of motor symptoms and increased risk for falls. Different medical co-morbidities can often contribute to LUT symptoms, and a thorough evaluation therefore becomes essential. The effects of medications used for Parkinson's disease and other co-existing medical co-morbidities on LUT symptoms is often underestimated. Treatment options include behavioural therapy, oral agents such as antimuscarinic and beta-3 receptor agonist agents, botulinum toxin and neuromodulation. The first-line oral agents cause adverse effects that may exacerbate pre-existing Parkinson's disease-related symptoms. Furthermore, these oral agents can interact with other medications used in Parkinson's disease, and the challenges posed by interactions on pharmacological effects and metabolism are discussed. Knowledge about drug interactions can help in effective management of such patients and mitigate the risks for developing adverse effects.

Sacral Tarlov cysts: Neurophysiology abnormalities and correlation with pelvic sensory and visceral symptoms.

BACKGROUND AND PURPOSE: Recent studies suggest a possible association between Tarlov cysts (TCs), usually considered as incidental radiological findings, and neurological symptoms such as pain, numbness and urogenital complaints. The aim was to explore the relationship between TCs and sacral nerve root functions using pelvic neurophysiology tests, and to correlate changes with clinical symptoms and magnetic resonance imaging (MRI) findings. METHODS: Consecutive patients with sacral TCs, referred for pelvic neurophysiology testing and presenting with at least one symptom related to the pelvic area, participated in a cross-sectional review of symptoms using validated questionnaires. Findings of pelvic neurophysiology (pudendal sensory evoked potentials, sacral dermatomal sensory evoked potentials, external anal sphincter electromyography) and urodynamics testing were collected retrospectively. The relationship between neurophysiology, MRI findings and patients' symptoms was assessed using Fisher and ANOVA tests. RESULTS: Sixty-five females were included (mean age 51.2 ± 12.1 years). The commonest symptom was pain (92%). Urinary (91%), bowel (71%) and sexual (80%) symptoms were also frequently reported. Thirty-seven patients (57%) had abnormal neurophysiology findings reflecting sacral root dysfunction. No association was seen between MRI findings (size, location of the cysts, severity of compression) and neurophysiology. A negative association was observed between neurophysiology abnormalities and occurrence of urgency urinary incontinence (p = 0.03), detrusor overactivity (p < 0.01) and stress urinary incontinence (p = 0.04); however, there was no association with voiding difficulties. CONCLUSIONS: Contrary to current understanding, TCs are associated with injury to the sacral somatic innervation in the majority of patients with presumed symptomatic cysts. However, urinary incontinence is unlikely to be related to TC-induced nerve damage.

Systematic review exploring the relationship between sexual abuse and lower urinary tract symptoms.

INTRODUCTION AND HYPOTHESIS: Patients presenting with lower urinary tract symptoms (LUTS) may report a history of sexual abuse (SA), and survivors of SA may report LUTS; however, the nature of the relationship is poorly understood. The aim of this review is to systematically evaluate studies that explore LUT dysfunction in survivors of SA. METHODS: A systematic literature search of six databases, Cochrane Database of Systematic Reviews, MEDLINE, EMBASE, CINAHL, AMED, and PsycINFO, was performed. The last search date was June 2021 (PROSPERO CRD42019122080). Studies reporting the prevalence and symptoms of LUTS in patients who have experienced SA were included. The literature was appraised according to the PRISMA statement. The quality of the studies was assessed. RESULTS: Out of 272 papers retrieved, 18 publications met the inclusion criteria: studies exploring LUTS in SA survivors (n=2), SA in patients attending clinics for their LUTs (n=8), and cross-sectional studies (n=8). SA prevalence ranged between 1.3% and 49.6%. A history of SA was associated with psychosocial stressors, depression, and anxiety. LUTS included urinary storage symptoms, voiding difficulties, voluntary holding of urine and urinary tract infections. Most studies were of moderate quality. Assessment of SA and LUTS lacked standardisation. CONCLUSIONS: The review highlights the need for a holistic assessment of patients presenting with LUTS. Although most of the studies were rated as being of 'moderate' quality, the evidence suggests the need to provide a "safe space" in clinic for patients to share sensitive information about trauma. Any such disclosure should be followed up with further assessment.

Urogenital dysfunction following neurotrauma.

PURPOSE OF REVIEW: Trauma affecting the nervous system can have widespread consequences, depending on the location and severity of injury. The sensorimotor and cognitive deficits due to neurotrauma have been studied extensively. Although the overall mortality due to neurotrauma has reduced, the disability and morbidity due to neurotrauma has not significantly reduced and focus on urogenital disturbances in these patients has been limited. This article reviews the limited evidence and scientific literature on this topic. RECENT FINDINGS: Lower urinary tract symptoms are commonly reported in patients with neurotrauma, both acutely and over the long term. Multiple medical and surgical approaches are available for managing bladder dysfunction in these patients. Sexual dysfunction and changes in sexual behaviour are reported by patients with neurotrauma and are usually multifactorial. Treatment options for sexual dysfunction are available and their management requires a holistic approach. SUMMARY: Urogenital dysfunction contributes significantly to the overall functional outcome and impaired quality of life in patients with neurotrauma. A better understanding of the mechanisms behind the urogenital abnormalities is needed for efficient management and treatment.

Diagnosing Premotor Multiple System Atrophy: Natural History and Autonomic Testing in an Autopsy-Confirmed Cohort.

BACKGROUND AND OBJECTIVES: Nonmotor features precede motor symptoms in many patients with multiple system atrophy (MSA). However, little is known about differences between the natural history, progression, and prognostic factors for survival in patients with MSA with nonmotor vs motor presentations. We aimed to compare initial symptoms, disease progression, and clinical features at final evaluation and investigate differences in survival and natural history between patients with MSA with motor and nonmotor presentations. METHODS: Medical records of autopsy-confirmed MSA cases at Queen Square Brain Bank who underwent both clinical examination and cardiovascular autonomic testing were identified. Clinical features, age at onset, sex, time from onset to diagnosis, disease duration, autonomic function tests, and plasma noradrenaline levels were evaluated. RESULTS: Forty-seven patients with autopsy-confirmed MSA (age 60 ± 8 years; 28 men) were identified. Time from symptom onset to first autonomic evaluation was 4 ± 2 years, and the disease duration was 7.7 ± 2.2 years. Fifteen (32%) patients presented with nonmotor features including genitourinary dysfunction, orthostatic hypotension, or REM sleep behavior disorder before developing motor involvement (median delay 1-6 years). A third (5/15) were initially diagnosed with pure autonomic failure (PAF) before evolving into MSA. All these patients had normal supine plasma noradrenaline levels (332.0 ± 120.3 pg/mL) with no rise on head-up tilt (0.1 ± 0.3 pg/mL). Patients with MSA with early cardiovascular autonomic dysfunction (within 3 years of symptom onset) had shorter survival compared with those with later onset of cardiovascular autonomic impairment (6.8 years [5.6-7.9] vs 8.5 years [7.9-9.2]; p = 0.026). Patients with early urinary catheterization had shorter survival than those requiring catheterization later (6.2 years [4.6-7.8] vs 8.5 years [7.6-9.4]; p = 0.02). The survival of patients with MSA presenting with motor and nonmotor symptoms did not differ (p > 0.05). DISCUSSION: Almost one-third of patients with MSA presented with nonmotor features, which could predate motor symptoms by up to 6 years. Cardiovascular autonomic failure and early urinary catheterization were predictors of poorer outcomes. A normal supine plasma noradrenaline level in patients presenting with PAF phenotype is a possible autonomic biomarker indicating later conversion to MSA.

Stimulation of the tibial nerve-a randomised trial for urinary problems associated with Parkinson's-the STARTUP trial.

BACKGROUND: non-motor symptoms such as bladder dysfunction are common (80%) in people with Parkinson's increasing the risk for falls with a negative impact on health-related costs and quality of life.We undertook STARTUP to evaluate the clinical and cost-effectiveness of using an adhesive electrode to stimulate the transcutaneous tibial nerve stimulation (TTNS) to treat bladder dysfunction in people with Parkinson's disease (PD).Study design, materials and methods: STARTUP was a parallel two-arm, multi-centre, pragmatic, double-blind, randomised controlled trial. Each participant attended one clinic visit to complete consent, be randomised using a computer-generated system and to be shown how to use the device.The trial had two co-primary outcome measures: International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form and the International Prostate Symptom Score (IPSS). These were completed at baseline, 6 and 12 weeks. A bladder frequency chart and resource questionnaire were also completed. RESULTS: two hundred forty two participants were randomised. About 59% of participants were male, the mean age was 69 years and mean time since diagnosis was 6 years. Questionnaire return rate was between 79 and 90%.There was a statistically significantly lower score in the active group at 6 weeks in the IPSS questionnaire (mean difference (Standard deviation, SD) 12.5 (6.5) vs 10.9 (5.5), effect size -1.49, 95% CI -2.72, -0.25). There was no statistically significant change in any other outcome. CONCLUSION: TTNS was demonstrated to be safe with a high level of compliance. There was a significant change in one of the co-primary outcome measures at the end of the treatment period (i.e. 6 weeks), which could indicate a benefit. Further fully powered RCTs are required to determine effective treatments.

Female Urinary Retention Progressing to Possible Multiple System Atrophy-cerebellar Form after 12 Years.

We herein report a 73-year-old Japanese woman with possible multiple system atrophy-cerebellar form (MSA-C) who suffered from urinary retention (sacral autonomic disorder) for 12 years before exhibiting cerebellar ataxia. A peculiar combination of findings on urodynamics and sphincter electromyography (EMG), e.g. detrusor hyperactivity with impaired contraction (DHIC), detrusor-sphincter dyssynergia (DSD) and neurogenic sphincter EMG (upper and lower neuron-type autonomic dysfunction), seems to have been predictive of future development of MSA.

The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy.

BACKGROUND: The second consensus criteria for the diagnosis of multiple system atrophy (MSA) are widely recognized as the reference standard for clinical research, but lack sensitivity to diagnose the disease at early stages. OBJECTIVE: To develop novel Movement Disorder Society (MDS) criteria for MSA diagnosis using an evidence-based and consensus-based methodology. METHODS: We identified shortcomings of the second consensus criteria for MSA diagnosis and conducted a systematic literature review to answer predefined questions on clinical presentation and diagnostic tools relevant for MSA diagnosis. The criteria were developed and later optimized using two Delphi rounds within the MSA Criteria Revision Task Force, a survey for MDS membership, and a virtual Consensus Conference. RESULTS: The criteria for neuropathologically established MSA remain unchanged. For a clinical MSA diagnosis a new category of clinically established MSA is introduced, aiming for maximum specificity with acceptable sensitivity. A category of clinically probable MSA is defined to enhance sensitivity while maintaining specificity. A research category of possible prodromal MSA is designed to capture patients in the earliest stages when symptoms and signs are present, but do not meet the threshold for clinically established or clinically probable MSA. Brain magnetic resonance imaging markers suggestive of MSA are required for the diagnosis of clinically established MSA. The number of research biomarkers that support all clinical diagnostic categories will likely grow. CONCLUSIONS: This set of MDS MSA diagnostic criteria aims at improving the diagnostic accuracy, particularly in early disease stages. It requires validation in a prospective clinical and a clinicopathological study. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Approach and management to patients with neurological disorders reporting sexual dysfunction.

Sexual difficulties are common in patients with neurological disorders, and different domains of sexual function-desire, arousal, orgasm, and ejaculation-can be affected. Advances in the past 7 years in structural and functional neuroimaging have contributed to a greater understanding of the neural pathways involved in the regulation of sexual functions in health and disease, and this increased knowledge might help with development of future therapeutic strategies. A comprehensive assessment of patients includes history taking-covering the different domains of dysfunction, and primary, secondary, and tertiary contributory factors-as well as clinical examination in select patients (ie, patients for whom an associated non-neurological cause for sexual dysfunction is suspected). Investigations, such as assessment of associated cardiovascular risk factors, might also be indicated in specific situations. PDE5A inhibitors and intracavernosal injections of the prostaglandin alprostadil are effective for treating erectile dysfunction; however, options for managing other domains of sexual dysfunction in men and women remain poor. Research into different domains of sexual dysfunction is likely to lead to additional therapeutic strategies in the future.

Diagnostic criteria for enduring sexual dysfunction after treatment with antidepressants, finasteride and isotretinoin.

BACKGROUND: A set of enduring conditions have been reported in the literature involving persistent sexual dysfunction after discontinuation of serotonin reuptake inhibiting antidepressants, 5 alpha-reductase inhibitors and isotretinoin. OBJECTIVE: To develop diagnostic criteria for post-SSRI sexual dysfunction (PSSD), persistent genital arousal disorder (PGAD) following serotonin reuptake inhibitors, post-finasteride syndrome (PFS) and post-retinoid sexual dysfunction (PRSD). METHODS: The original draft was designed using data from two published case series (Hogan et al., 2014 and Healy et al., 2018), which represent the largest public collections of data on these enduring conditions. It was further developed with the involvement of a multidisciplinary panel of experts. RESULTS: A set of criteria were agreed upon for each of the above conditions. Features of PSSD, PFS and PRSD commonly include decreased genital and orgasmic sensation, decreased sexual desire and erectile dysfunction. Ancillary non-sexual symptoms vary depending on the specific condition but can include emotional blunting and cognitive impairment. PGAD presents with an almost mirror image of unwanted sensations of genital arousal or irritability in the absence of sexual desire. A new term, post-SSRI asexuality, is introduced to describe a dampening of sexual interest and pleasure resulting from a pre-natal or pre-teen exposure to a serotonin reuptake inhibitor. CONCLUSIONS: These criteria will help in both clinical and research settings. As with all criteria, they will likely need modification in the light of developments.

Urinary catheter management: what neurologists need to know.

Patients with neurological disorders often have lower urinary tract dysfunction, manifesting as urinary retention or urinary incontinence, and so commonly use catheters. Neurologists should therefore be aware of the different types of catheters and appliances and their risks, benefits and complications. Clean intermittent self-catheterisation is preferable to an indwelling catheter; however, if this is not possible, then a suprapubic indwelling catheter is preferable to a urethral catheter for long-term management. We review the decision-making process when selecting catheters for neurological patients, the evidence base regarding the different options and how neurologists can recognise and address complications. We also discuss alternatives to catheterisation, such as non-invasive containment products and surgical treatments, and the indications for urological referral.

Urodynamic Evaluation in Multiple System Atrophy: A Retrospective Cohort Study.

BACKGROUND: Urological dysfunction in patients with multiple system atrophy (MSA) is one of the main manifestations of autonomic failure. Urodynamic examination is clinically relevant since underlying pathophysiology of lower urinary tract (LUT) dysfunction can be variable. OBJECTIVE: Evaluation of the pathophysiology of urological symptoms and exploration of differences in urodynamic patterns of LUT dysfunction between MSA-P and MSA-C. METHODS: Retrospective study of patients with possible and probable MSA who were referred for urodynamic studies between 2004 and 2019. Demographic data, medical history, physical examination and urodynamic studies assessing storage and voiding dysfunction were obtained. RESULTS: Seventy-four patients were included in this study (MSA-P 64.9% n = 48; median age 62.5 (IQR 56.8-70) years). Detrusor overactivity during filling phase was noted in 58.1% (n = 43) of the patients. In the voiding phase, detrusor sphincter dyssynergia and detrusor underactivity were observed in 24.6% (n = 17) and in 62.1% (n = 41) of the patients, respectively. A postmicturition residual volume of over 100 ml was present in 71.4% (n = 50) of the patients. Comparison of MSA subtypes showed weaker detrusor contractility in MSA-P compared to MSA-C [pdetQmax 26.2 vs. 34.4 cmH20, P = 0.04]. In 56.2% (n = 41) of patients pathophysiology of LUT dysfunction was deemed to be neurogenic and consistent with the diagnosis of MSA. In 35.6% (n = 26) urodynamic pattern suggested other urological co-morbidities. CONCLUSION: Urodynamic evaluation is an important tool to analyze the pattern of LUT dysfunction in MSA. Impaired detrusor contractility was seen more in MSA-P which needs to be investigated in further studies.