Andrusiów Szymon, Dziadkowiak Edyta, Koszewicz Magdalena
Clinical Department of Neurology, University Centre of Neurology and Neurosurgery, Wroclaw Medical University, Wroclaw, Poland.
Clinical Neurophysiology Laboratory, University Centre of Neurology and Neurosurgery, Faculty of Medicine, Wroclaw Medical University, Wroclaw, Poland.
Front Immunol. 2025 Jun 4;16:1572507. doi: 10.3389/fimmu.2025.1572507. eCollection 2025.
The classification of combined central and peripheral demyelination (CCPD) is challenging due to unclear pathomechanisms and a lack of diagnostic and therapeutic criteria. Existing clinical data are limited to case reports or small series, with few attempts to define CCPD using radiological or molecular markers. Differential diagnosis depends on excluding well-characterized demyelinating diseases of the central and peripheral nervous systems. No systematic review has yet summarized the clinical, radiological, electrophysiological, molecular, and therapeutic evidence for CCPD.
This review follows PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, uses the JBI critical appraisal tool for case series and is registered at PROSPERO (CRD42025640575). A systematic search of Embase, MEDLINE, Web of Science, and Google Scholar was conducted for studies available up to December 2024. Inclusion criteria focused on adult patients with electrophysiological and imaging findings. Exclusion criteria included CCPD associated with infections, rheumatological conditions, or anti-MOG/anti-AQP4 antibodies.
Most patients exhibited hemiparesis assessed by MMT and MRC scales, with tetraparesis often asymmetrical. Imaging revealed either diffuse CNS involvement (cerebral hemispheres, brainstem, spinal cord) or lesions limited to one or two sites. Nerve conduction studies showed primarily demyelinating features. Treatment frequently involved combination therapies.
This review underscores the dearth of high-quality data on CCPD, with extant studies frequently exhibiting a paucity of methodology for definitive analysis. The presence of elevated protein concentrations in CSF and the presence of antibodies, specifically anti-LacCer and anti-NF, has been identified as potential biomarkers of the disease. Furthermore, GCS in high doses might be one of the most effective treatment options.
https://www.crd.york.ac.uk/PROSPERO/view/CRD42025640575, identifier CRD42025640575.
由于发病机制不明以及缺乏诊断和治疗标准，中枢和周围联合脱髓鞘（CCPD）的分类具有挑战性。现有的临床数据仅限于病例报告或小系列研究，很少有人尝试使用放射学或分子标记来定义CCPD。鉴别诊断取决于排除中枢和周围神经系统特征明确的脱髓鞘疾病。尚无系统综述总结CCPD的临床、放射学、电生理学、分子学和治疗证据。
本综述遵循PRISMA（系统评价和Meta分析的首选报告项目）指南，使用JBI病例系列关键评估工具，并在PROSPERO（CRD42025640575）注册。对Embase、MEDLINE、科学网和谷歌学术进行了系统检索，以查找截至2024年12月的可用研究。纳入标准侧重于有电生理学和影像学结果的成年患者。排除标准包括与感染、风湿性疾病或抗MOG/抗AQP4抗体相关的CCPD。
大多数患者通过MMT和MRC量表评估表现为偏瘫，四肢瘫通常不对称。影像学显示中枢神经系统弥漫性受累（大脑半球、脑干、脊髓）或病变局限于一两个部位。神经传导研究主要显示脱髓鞘特征。治疗通常涉及联合治疗。
本综述强调了CCPD高质量数据的匮乏，现有研究往往缺乏进行确定性分析的方法。脑脊液中蛋白质浓度升高以及抗体（特别是抗LacCer和抗NF）的存在已被确定为该疾病的潜在生物标志物。此外，高剂量的糖皮质激素可能是最有效的治疗选择之一。
https://www.crd.york.ac.uk/PROSPERO/view/CRD42025640575，标识符CRD42025~
