Holtom Garrett Program in Neuroimmunology and Multiple Sclerosis Center, Department of Neurology, University of Michigan, Ann Arbor, Michigan 48109, USA.
Curr Opin Neurol. 2013 Jun;26(3):307-13. doi: 10.1097/WCO.0b013e3283608459.
The aims of this article are to discuss the epidemiology, pathophysiology and clinical phenomenology of neurosarcoidosis, as well as current approaches to diagnosis and treatment. This review focuses on central nervous system (CNS) complications of sarcoidosis.
Neurosarcoidosis is a rare disorder with diverse clinical manifestations and outcomes. It is often difficult to diagnose and even more difficult to treat. New diagnostic approaches include the use of [¹⁸F]-fluorodeoxyglucose PET to identify potential biopsy sites. Success has been reported in the treatment of steroid refractory cases with disease-modifying therapies that were originally designed to manage other chronic inflammatory conditions by neutralizing key cytokines or depleting leukocyte subsets.
The diagnosis and management of neurosarcoidosis can be challenging. Currently, the disorder is treated with corticosteroids in combination with global immunosuppressant agents and/or immunomodulatory monoclonal antibodies, such as infliximab. The development of novel CNS penetrant drugs that are particularly effective at inhibiting granuloma formation would represent a significant therapeutic advance. Future progress will be informed by a deeper understanding of the pathways underlying the granulomatous inflammation characteristic of sarcoidosis and by an increased appreciation of how sarcoid lesions evolve in the CNS microenvironment.
本文旨在讨论神经结节病的流行病学、病理生理学和临床现象学，以及目前的诊断和治疗方法。本综述重点讨论结节病的中枢神经系统（CNS）并发症。
神经结节病是一种罕见的疾病，具有多种临床表现和结果。它通常很难诊断，更难治疗。新的诊断方法包括使用 [¹⁸F]-氟脱氧葡萄糖 PET 来识别潜在的活检部位。通过使用最初用于治疗其他慢性炎症性疾病的疾病修饰疗法，即用中和关键细胞因子或耗尽白细胞亚群的方法，成功治疗了类固醇难治性病例。
神经结节病的诊断和治疗具有挑战性。目前，该病采用皮质类固醇联合全身免疫抑制剂和/或免疫调节单克隆抗体（如英夫利昔单抗）治疗。开发新型 CNS 穿透性药物，特别有效地抑制肉芽肿形成，将代表着重大的治疗进展。对结节病特征性肉芽肿炎症的潜在途径的深入了解，以及对 CNS 微环境中结节病病变如何演变的认识的提高，将为未来的进展提供信息。
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