Tullberg M, Månsson J E, Fredman P, Lekman A, Blennow K, Ekman R, Rosengren L E, Tisell M, Wikkelsø C
Institute of Clinical Neuroscience, Sahlgren's University Hospital, University of Göteborg, Sweden.
J Neurol Neurosurg Psychiatry. 2000 Jul;69(1):74-81. doi: 10.1136/jnnp.69.1.74.
To examine the CSF concentrations of molecules reflecting demyelination, neuronal and axonal degeneration, gliosis, monoaminergic neuronal function, and aminergic and peptidergic neurotransmission in a large series of patients with normal pressure hydrocephalus (NPH) or subcortical arteriosclerotic encephalopathy (SAE), to elucidate pathogenic, diagnostic, and prognostic features.
CSF concentrations of glycosphingolipid (sulfatide), proteins (neurofilament triplet protein (NFL), glial fibrillary acidic protein (GFAP)), neuropeptides (vasoactive intestinal peptide (VIP), 4-aminobutyric acid (GABA)), and monoamines (homovanillic acid (HVA), 5-hydroxy-indoleacetic acid (5-HIAA), 4-hydroxy-3-methoxyphenylglycol (HMPG)) were analysed in 43 patients with NPH and 19 patients with SAE. The diagnoses of NPH and SAE were based on strict criteria and patients with NPH were subsequently operated on. Twelve clinical variables, psychometric tests measuring perceptual speed, accuracy, learning, and memory and a psychiatric evaluation were performed in all patients and before and after a shunt operation in patients with NPH.
The CSF sulfatide concentration was markedly increased in patients with SAE (mean 766, range 300-3800 nmol/l) compared with patients with NPH (mean 206, range 50-400 nmol/l) (p<0.001). 5-HIAA, GABA, and VIP in CSF were higher in patients with SAE than in patients with NPH. The patients with NPH with cerebrovascular aetiology had higher sulfatide concentrations and a poorer outcome after shunt surgery than patients with NPH with other aetiologies.
The pathogenesis of the white matter changes in NPH and SAE is different and ischaemic white matter changes can be a part of the NPH state. The markedly increased CSF sulfatide concentrations in patients with SAE indicate ongoing demyelination as an important pathophysiological feature of SAE. The CSF sulfatide concentration distinguished between patients with SAE and those with NPH with a sensitivity of 74% and a specificity of 94%, making it an important diagnostic marker.
检测大量正常压力脑积水（NPH）或皮质下动脉硬化性脑病（SAE）患者脑脊液中反映脱髓鞘、神经元及轴突退变、胶质增生、单胺能神经元功能以及胺能和肽能神经传递的分子浓度，以阐明其致病、诊断及预后特征。
分析43例NPH患者和19例SAE患者脑脊液中糖鞘脂（硫脂）、蛋白质（神经丝三联蛋白（NFL）、胶质纤维酸性蛋白（GFAP））、神经肽（血管活性肠肽（VIP）、γ-氨基丁酸（GABA））以及单胺（高香草酸（HVA）、5-羟吲哚乙酸（5-HIAA）、4-羟基-3-甲氧基苯乙二醇（HMPG））的浓度。NPH和SAE的诊断基于严格标准，NPH患者随后接受手术。对所有患者进行12项临床变量、测量感知速度、准确性、学习和记忆的心理测试以及精神评估，对NPH患者在分流手术前后也进行了这些评估。
与NPH患者（平均206，范围50 - 400 nmol/l）相比，SAE患者脑脊液硫脂浓度显著升高（平均766，范围300 - 3800 nmol/l）（p<0.001）。SAE患者脑脊液中的5-HIAA、GABA和VIP高于NPH患者。有脑血管病因的NPH患者比其他病因的NPH患者硫脂浓度更高，分流手术后预后更差。
NPH和SAE中白质改变的发病机制不同，缺血性白质改变可能是NPH状态的一部分。SAE患者脑脊液硫脂浓度显著升高表明正在进行的脱髓鞘是SAE的重要病理生理特征。脑脊液硫脂浓度区分SAE患者和NPH患者的敏感性为74%，特异性为94%，使其成为重要的诊断标志物。
