Phillips J P, Lockman L A, Shapiro E G, Blazar B R, Loes D J, Moser H W, Krivit W
Division of Pediatric Neurology, University of Minnesota Medical School, Minneapolis.
Pediatr Neurol. 1994 Jun;10(4):289-94. doi: 10.1016/0887-8994(94)90124-4.
The childhood-onset cerebral form of adrenoleukodystrophy has a devastating neurologic prognosis. Unfortunately, there is no early method of distinguishing it from the more benign forms of adrenoleukodystrophy, such as adrenomyeloneuropathy. To evaluate the manner in which this disease entity may be reflected in the cerebrospinal fluid, we studied a consecutive series of 19 patients, all with biochemically proved adrenoleukodystrophy. total protein, immunoglobulin production, cytokine levels, and cerebrospinal fluid pressure were measured. In this single sample of cerebrospinal fluid, a significant correlation existed between clinical stage of the illness and cerebrospinal fluid myelin basic protein. No correlation existed with total protein, cytokines, or measures of immunoglobulin production.
儿童期起病的脑型肾上腺脑白质营养不良具有毁灭性的神经学预后。不幸的是,目前尚无早期方法将其与肾上腺脑白质营养不良的较良性形式(如肾上腺脊髓神经病)区分开来。为了评估这种疾病实体在脑脊液中的反映方式,我们研究了连续的19例患者,所有患者均经生化证实患有肾上腺脑白质营养不良。测量了总蛋白、免疫球蛋白产生、细胞因子水平和脑脊液压力。在这个单一的脑脊液样本中,疾病的临床分期与脑脊液髓鞘碱性蛋白之间存在显著相关性。与总蛋白、细胞因子或免疫球蛋白产生指标均无相关性。