Kristjánsdóttir R, Uvebrant P, Rosengren L
Department of Paediatrics, Orebro University Hospital, Orebro, Sweden.
Neuropediatrics. 2001 Dec;32(6):307-12. doi: 10.1055/s-2001-20406.
Glial fibrillary acidic protein (GFAP) is the major structural protein of the intermediate filaments found in glial cells. Increased levels in the cerebrospinal fluid (CSF) have been found to indicate gliosis. Neurofilament (NFL) is a structural element of neurons, mainly found in large myelinated axons. Its presence in the CSF has been suggested to reflect destruction of axons. The aim of this study was to see if GFAP and NFL in the CSF of children with neurological disabilities and an abnormal signal on magnetic resonance imaging (MRI) of the cerebral white matter could be used to clarify the underlying neuropathology. The potential of GFAP and NFL to differentiate a progressive disease from a stationary disorder was investigated, as was the correlation with disability and clinical findings. CSF from 26 children, eleven with progressive and 15 with non-progressive disorders, was analysed. GFAP was increased in all, interpreted to reflect gliosis. NFL was elevated in seven and considered to indicate ongoing neuroaxonal damage as all but one patient were found to have a progressive disease. GFAP did not differentiate between progressive and non-progressive disorders, although low levels were found in stationary and high levels in progressive disorders. The severity of the disability correlated with the NFL levels, but not with the concentration of GFAP.
胶质纤维酸性蛋白(GFAP)是神经胶质细胞中中间丝的主要结构蛋白。脑脊液(CSF)中该蛋白水平升高表明存在神经胶质增生。神经丝(NFL)是神经元的一种结构成分,主要存在于有髓大轴突中。脑脊液中出现NFL被认为反映了轴突的损伤。本研究的目的是探讨患有神经功能障碍且脑白质磁共振成像(MRI)信号异常的儿童脑脊液中的GFAP和NFL是否可用于阐明潜在的神经病理学情况。研究了GFAP和NFL区分进展性疾病和静止性疾病的潜力,以及它们与残疾程度和临床发现的相关性。分析了26名儿童的脑脊液,其中11名患有进展性疾病,15名患有非进展性疾病。所有患者的GFAP均升高,这被解释为反映了神经胶质增生。7名患者的NFL升高,由于除1名患者外所有患者均患有进展性疾病,因此认为这表明存在持续的神经轴突损伤。GFAP无法区分进展性疾病和非进展性疾病,尽管在静止性疾病中发现其水平较低,而在进展性疾病中水平较高。残疾严重程度与NFL水平相关,但与GFAP浓度无关。