Urinary Dysfunction in Myasthenic Syndromes: A Scoping Review of Clinical Features and Treatment-Related Associations.

IF 2.9 3区 医学 Q2 CLINICAL NEUROLOGY
Muscle & Nerve Pub Date : 2026-09-01 Epub Date: 2026-07-07 DOI:10.1002/mus.70337
Julia M Augustin, Lillith Horstkorte, Hellen Schmidt, Blanca G Sánchez Navarro, Avan Kader, Inga Koneczny, Holly M Nelson, Pratishtha P Wadnerkar, Rafael Meleka Hanna, Ayşe N Özdağ Acarlı, Piraye Oflazer, Atay Vural, Franz Blaes, Miriam L Fichtner
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引用次数: 0

Abstract

Urinary dysfunction has been reported in association with myasthenic syndromes, including myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), and congenital myasthenic syndromes (CMS), but evidence regarding its prevalence, clinical impact, pathophysiology, and management remains limited. This scoping review synthesizes the available evidence on urinary symptoms, diagnostic approaches, pathophysiological features, treatment-related associations, and sex-specific findings in these disorders. A literature search was conducted in PubMed, LIVIVO, Epistemonikos, and the Cochrane Library in accordance with PRISMA guidelines. Of 774 records identified, eight studies met the inclusion criteria, comprising two case reports, three case-control studies, one prospective study, and two retrospective observational studies. Seven studies addressed MG and one addressed LEMS; no eligible studies were identified for CMS. Reported urinary symptoms were predominantly storage lower urinary tract symptoms (LUTS), including urinary incontinence, urgency, nocturia, and increased frequency, whereas voiding and bladder-emptying abnormalities were described less consistently. Diagnostic approaches were heterogeneous and included questionnaires, clinical examination, urodynamic testing, imaging, and autonomic assessments. Proposed mechanisms remain uncertain, with limited evidence supporting contributions from pelvic floor weakness, autonomic dysfunction, and cholinesterase inhibitor exposure. Treatment-related associations were largely observational and most consistently implicated pyridostigmine in symptom worsening, while evidence for specific treatment of urinary symptoms was sparse. Sex-specific analyses were limited and did not identify consistent sex-related patterns. Current evidence indicates that urinary symptoms have been reported in myasthenic syndromes and have been associated with reduced quality of life in the included studies, but the available data remain insufficient to define prevalence, mechanisms, or optimal management.

肌无力综合征的尿功能障碍:临床特征和治疗相关关联的范围综述。
泌尿功能障碍已被报道与重症肌无力综合征相关,包括重症肌无力(MG)、Lambert-Eaton肌无力综合征(LEMS)和先天性肌无力综合征(CMS),但有关其患病率、临床影响、病理生理学和治疗的证据仍然有限。本综述综合了这些疾病中泌尿系统症状、诊断方法、病理生理特征、治疗相关关联和性别特异性发现的现有证据。根据PRISMA指南,在PubMed、LIVIVO、Epistemonikos和Cochrane Library中进行文献检索。在确定的774项记录中,8项研究符合纳入标准,包括2项病例报告、3项病例对照研究、1项前瞻性研究和2项回顾性观察性研究。七项研究涉及MG,一项研究涉及LEMS;没有合适的研究被确定为CMS。报告的泌尿系统症状主要是下尿路症状(LUTS),包括尿失禁、尿急、夜尿症和尿频增加,而排尿和膀胱排空异常的描述不太一致。诊断方法不同,包括问卷调查、临床检查、尿动力学测试、影像学和自主神经评估。提出的机制仍然不确定,有限的证据支持盆底无力、自主神经功能障碍和胆碱酯酶抑制剂暴露。治疗相关的关联在很大程度上是观察性的,并且最一致地表明吡哆斯的明与症状恶化有关,而关于泌尿系统症状的特异性治疗的证据很少。性别特异性分析是有限的,并没有确定一致的性别相关模式。目前的证据表明,在纳入的研究中,已报道了肌无力综合征的泌尿系统症状,并与生活质量下降有关,但现有的数据仍不足以确定患病率、机制或最佳管理。
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来源期刊
Muscle & Nerve
Muscle & Nerve 医学-临床神经学
CiteScore
6.40
自引率
5.90%
发文量
287
审稿时长
3-6 weeks
期刊介绍: Muscle & Nerve is an international and interdisciplinary publication of original contributions, in both health and disease, concerning studies of the muscle, the neuromuscular junction, the peripheral motor, sensory and autonomic neurons, and the central nervous system where the behavior of the peripheral nervous system is clarified. Appearing monthly, Muscle & Nerve publishes clinical studies and clinically relevant research reports in the fields of anatomy, biochemistry, cell biology, electrophysiology and electrodiagnosis, epidemiology, genetics, immunology, pathology, pharmacology, physiology, toxicology, and virology. The Journal welcomes articles and reports on basic clinical electrophysiology and electrodiagnosis. We expedite some papers dealing with timely topics to keep up with the fast-moving pace of science, based on the referees'' recommendation.
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