"C.N.S. Alliance" Research Group, Athens, Greece.
Department of Neurosurgery, School of Medicine, Democritus University of Thrace, University General Hospital of Alexandroupolis, Alexandroupolis, Greece.
Childs Nerv Syst. 2024 Dec;40(12):4107-4114. doi: 10.1007/s00381-024-06649-5. Epub 2024 Oct 10.
Myelomeningocele (MMC), a congenital neural tube defect, is the most common developmental anomaly of the central nervous system (CNS). Spasticity is among the main disabling factors in these patients, affecting up to 1/5 of children. The purpose of this article is to review the current knowledge regarding spasticity in MMC patients, mainly focusing on its manifestations and management.
A literature search was conducted using the PubMed database for the terms "myelomeningocele" and "spasticity." Data were collected by 5 independent investigators and then synthesized in a scoping format.
Causes of spasticity in these patients include hydrocephalus, tethered cord syndrome (TCS), syringomyelia, CNS infection, and associated congenital brain and spine anomalies. Clinical manifestations include limb spasticity and neurogenic bladder. Spasticity primarily affects muscles below the neurological level and contributes to gait impairment. Besides walking ability, spasticity also severely affects functional mobility in general, self-care, and quality of life. The majority of MMC patients experience neurogenic bladder. Treatment of spasticity can be symptomatic or target its cause. Medical and surgical options are available for both limb and bladder spasticity. Regular physiotherapy and orthotics are used to prevent contractures and the early introduction of orthoses is the cornerstone of a gait training program. Neurosurgical treatment options for spasticity include nonablative and ablative procedures such as selective dorsal rhizototmy. The urodynamic pattern guides treatment strategies, with intermittent catheterization being the best treatment option for patients with underactive detrusor and overactive sphincter muscles.
Given the particularities of spasticity in MMC patients, a multidisciplinary team approach and early rehabilitation programs are keys for their optimal management.
脊髓脊膜膨出（MMC）是一种先天性神经管缺陷，是中枢神经系统（CNS）最常见的发育异常。痉挛是这些患者的主要致残因素之一，高达 1/5的患儿受其影响。本文旨在综述 MMC 患者痉挛的现有知识，主要集中在其表现和管理方面。
使用 PubMed 数据库，对“脊髓脊膜膨出”和“痉挛”这两个术语进行文献检索。由 5 名独立研究人员收集数据，并以综述形式综合分析。
这些患者痉挛的原因包括脑积水、脊髓栓系综合征（TCS）、脊髓空洞症、中枢神经系统感染以及相关的先天性脑和脊柱异常。临床表现包括肢体痉挛和神经性膀胱。痉挛主要影响神经水平以下的肌肉，导致步态受损。除了行走能力外，痉挛还严重影响一般的功能性移动、自理能力和生活质量。大多数 MMC 患者存在神经性膀胱。痉挛的治疗可以是对症的，也可以针对其病因。有针对肢体和膀胱痉挛的药物和手术治疗选择。定期进行物理治疗和矫形器有助于预防挛缩，早期引入矫形器是步态训练计划的基石。痉挛的神经外科治疗选择包括非消融和消融性手术，如选择性脊神经根切断术。尿动力学模式指导治疗策略，间歇性导尿是逼尿肌活动低下伴尿道外括约肌过度活动患者的最佳治疗选择。
鉴于 MMC 患者痉挛的特殊性，多学科团队方法和早期康复计划是其最佳管理的关键。
