Institute for Neuroscience and Muscle Research, T.Y. Nelson Department of Neurology and Neurosurgery, Children's Hospital at Westmead, Clinical School, University of Sydney, Westmead, New South Wales, Australia.
Curr Opin Pediatr. 2010 Dec;22(6):718-25. doi: 10.1097/MOP.0b013e3283402b71.
Inflammatory and auto-immune disorders of the central nervous system are a heterogeneous group of disorders. Many of these disorders are potentially treatable with immune therapies that can reduce disability or prevent death. We review the clinical value of biomarkers which can aid in the diagnosis of paediatric inflammatory and auto-immune central nervous system (CNS) disorders.
This review will first describe the clinical usefulness of nonspecific biomarkers of CNS inflammation such as cerebrospinal fluid neopterin and oligoclonal bands. Neopterin is produced by immune and neuronal cells after stimulation by interferon species and is increased in a broad range of inflammatory and auto-immune CNS disorders. Oligoclonal bands represent clonal production of immunoglobulin G in the CNS and are present in demyelinating, auto-immune, and infectious CNS disorders. In addition, we will review new advances in the immunogenetic investigation of familial auto-inflammatory disorders such as Aicardi-Goutières syndrome and Chronic Infantile Neurologic Cutaneous Articular syndrome. Finally, we will review the clinical utility of auto-antibodies in CNS disorders, with specific focus on auto-antibodies that bind to cell surface proteins such as N-methyl-D-asparate receptor, voltage-gated potassium channels, myelin oligodendrocyte glycoprotein, and aquaporin-4.
These biomarkers are increasingly important in the recognition and treatment of inflammatory and auto-immune CNS disorders. Like many biomarkers in paediatric practice, it is essential to interpret the findings in the context of the patient history and examination.
中枢神经系统的炎症和自身免疫性疾病是一组异质性疾病。许多此类疾病可通过免疫疗法进行治疗,从而减轻残疾或预防死亡。我们回顾了有助于诊断儿科炎症和自身免疫性中枢神经系统(CNS)疾病的生物标志物的临床价值。
本篇综述首先将描述 CNS 炎症的非特异性生物标志物的临床应用,如脑脊液中新蝶呤和寡克隆带。新蝶呤是在干扰素种类刺激下由免疫和神经元细胞产生的,在广泛的炎症和自身免疫性 CNS 疾病中增加。寡克隆带代表 CNS 中免疫球蛋白 G 的克隆产生,存在于脱髓鞘、自身免疫和感染性 CNS 疾病中。此外,我们将综述家族性自身炎症性疾病(如 Aicardi-Goutières 综合征和慢性婴儿神经皮肤关节综合征)的免疫遗传学研究中的新进展。最后,我们将综述 CNS 疾病中自身抗体的临床应用,重点关注与细胞表面蛋白结合的自身抗体,如 N-甲基-D-天冬氨酸受体、电压门控钾通道、髓鞘少突胶质细胞糖蛋白和水通道蛋白-4。
这些生物标志物在识别和治疗炎症和自身免疫性 CNS 疾病方面越来越重要。与儿科实践中的许多生物标志物一样,必须根据患者的病史和检查结果来解释这些发现。