Frost Nicole, Barclay S Jade
Flex-Ability Physio, Wollongong, NSW, Australia.
Connected Health Alliance, Wollongong, NSW, Australia.
Front Neurol. 2024 Dec 10;15:1479545. doi: 10.3389/fneur.2024.1479545. eCollection 2024.
Individuals with joint hypermobility and the Ehlers-Danlos Syndromes (EDS) are disproportionately affected by neuraxial dysfunction and Central Nervous System (CNS) disorders: such as Spontaneous Intracranial Hypotension (SIH) due to spinal cerebrospinal fluid (CSF) leaks, Upper Cervical Instability (UCI; including craniocervical or atlantoaxial instability (CCI/AAI)), Occult Tethered Cord Syndrome (TCS), Chiari Malformation (CM) and Idiopathic Intracranial Hypertension (IIH). The neuraxis comprises the parts of the nervous system (brain, nerves, spinal cord) along the craniospinal axis of the body. Neuraxial tissue includes all tissue structures that comprise, support, sheath, and connect along the neuraxis and peripheral nerves. Altered mechanical loading or vascular supply of neural structures can adversely impact neural health and conductivity, with local and remote effects on inflammation, venous congestion, and muscle control. With EDS characterized by altered structure of the connective tissues found throughout the body including the neural system, altered mechanical properties of the central nervous system (CNS) and its surrounding tissue structures are important considerations in the development and diagnostics of these CNS disorders, as well as response to therapeutic interventions. Experts have identified a need for neuraxial curriculum in medical education and hypermobility-adapted treatment approaches in pain management, neurosurgery, anesthesiology, hematology, gastrointestinal surgery, dermatology, cardiology, dentistry, gastroenterology, allergy/immunology, physical therapy, primary care, radiology and emergency medicine. This paper reviews the interactions between neuraxial biomechanics and pathology related to CNS disorders seen commonly with EDS. First, we provide a concise synthesis of the literature on neuraxial kinematics and fluid dynamics. We then discuss the interplay of these biomechanics and their involvement in clinically-relevant diagnoses and overlapping symptom presentations, modeling physiological reasoning to highlight knowledge gaps, support clinical decision-making, improve multidisciplinary management of hypermobility-associated complexity, and add weight to the call for medical education reform.
关节活动过度和埃勒斯-当洛综合征（EDS）患者受神经轴功能障碍和中枢神经系统（CNS）疾病的影响尤为严重，这些疾病包括：因脊髓脑脊液（CSF）漏导致的自发性颅内低压（SIH）、上颈椎不稳（UCI；包括颅颈或寰枢椎不稳（CCI/AAI））、隐匿性脊髓拴系综合征（TCS）、 Chiari畸形（CM）和特发性颅内高压（IIH）。神经轴包括沿身体颅脊柱轴的神经系统（脑、神经、脊髓）部分。神经轴组织包括沿神经轴和周围神经构成、支持、包裹和连接的所有组织结构。神经结构的机械负荷或血管供应改变会对神经健康和传导产生不利影响，并对炎症、静脉充血和肌肉控制产生局部和远处影响。EDS的特征是包括神经系统在内的全身结缔组织结构改变，中枢神经系统（CNS）及其周围组织结构的机械性能改变是这些CNS疾病发生、诊断以及对治疗干预反应的重要考虑因素。专家们已经认识到医学教育中需要神经轴课程，以及在疼痛管理、神经外科、麻醉学、血液学、胃肠外科、皮肤科、心脏病学、牙科、胃肠病学、过敏/免疫学、物理治疗、初级保健、放射学和急诊医学中采用适应活动过度的治疗方法。本文综述了与EDS常见的CNS疾病相关的神经轴生物力学与病理学之间的相互作用。首先，我们对神经轴运动学和流体动力学的文献进行了简要综述。然后，我们讨论这些生物力学的相互作用及其在临床相关诊断和重叠症状表现中的作用，建立生理推理模型以突出知识差距、支持临床决策、改善对活动过度相关复杂性的多学科管理，并强调医学教育改革的必要性。
