Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Yamaguchi, Japan.
Epilepsy Res. 2012 Nov;102(1-2):8-12. doi: 10.1016/j.eplepsyres.2012.07.002. Epub 2012 Jul 24.
West syndrome is an epileptic encephalopathy with a poor developmental outcome. Tau protein levels in the cerebrospinal fluid (CSF) are reported to be markers of axonal damage and neurodegeneration. This study aimed to investigate axonal damage and the effects of adrenocorticotropic hormone (ACTH) therapy on axons in West syndrome, as measured by tau protein levels in CSF.
Tau protein levels in CSF before and after ACTH therapy were determined by an enzyme-linked immunosorbent assay in 26 children with West syndrome. Of these 26 children, 18 were symptomatic, and 8 had a cryptogenic form of West syndrome. A group of 41 unaffected children was included in the study as a control group.
The levels of tau protein in CSF were significantly higher in children with West syndrome than in the control group, and these levels remained high after ACTH therapy. ACTH therapy was effective for 20 of the 26 children with West syndrome, and their CSF tau protein levels were significantly higher after ACTH therapy than before therapy.
Our results suggest that axonal damage occurs in West syndrome, as judged by tau protein levels in CSF.
婴儿痉挛症是一种伴有不良发育结局的癫痫性脑病。脑脊液（CSF）中的 Tau 蛋白水平被报道为轴突损伤和神经退行性变的标志物。本研究旨在通过 CSF 中的 Tau 蛋白水平来研究婴儿痉挛症中的轴突损伤以及促肾上腺皮质激素（ACTH）治疗对轴突的影响。
通过酶联免疫吸附试验，测定了 26 例婴儿痉挛症患儿 ACTH 治疗前后 CSF 中的 Tau 蛋白水平。这 26 例患儿中，18 例为症状性，8 例为隐源性婴儿痉挛症。将 41 例无影响的儿童纳入研究作为对照组。
婴儿痉挛症患儿 CSF 中的 Tau 蛋白水平明显高于对照组，且 ACTH 治疗后水平仍较高。ACTH 治疗对 26 例婴儿痉挛症中的 20 例有效，其 CSF Tau 蛋白水平在 ACTH 治疗后明显高于治疗前。
我们的结果表明，婴儿痉挛症中存在轴突损伤，这可以通过 CSF 中的 Tau 蛋白水平来判断。
