Vanhatalo S, Riikonen R
Department of Child Neurology, Children's Hospital, University of Kuopio, Kuopio, Finland.
Epilepsia. 2000 Jun;41(6):705-8. doi: 10.1111/j.1528-1157.2000.tb00231.x.
To compare the levels of brain nitric oxide production in patients with PEHO or PEHO-like syndrome and in controls with other neurologic disease.
Nitric oxide metabolites, nitrates, and nitrites (NNx), were measured in the cerebrospinal fluid (CSF) of children with PEHO syndrome or PEHO-like syndrome, and in controls with other neurologic diseases.
The NNx levels were markedly higher in both PEHO (mean, 48 microM; p < 0.001) and PEHO-like (22 microM; p < 0.003) patients as compared with the controls (6 microM), but did not correlate with age or with brain atrophy or CSF levels of insulin-like growth factor-1 (IGF-1).
Our findings suggest that in PEHO syndrome, production of nitric oxide is markedly increased, suggesting that nitric oxide is involved in the pathologic phenomena (i.e., seizures and neurodegeneration) of the disease.
比较PEHO或类PEHO综合征患者与患有其他神经系统疾病的对照组的脑内一氧化氮生成水平。
检测了PEHO综合征或类PEHO综合征患儿以及患有其他神经系统疾病的对照组患儿脑脊液（CSF）中的一氧化氮代谢产物、硝酸盐和亚硝酸盐（NNx）。
与对照组（6微摩尔）相比，PEHO患者（平均48微摩尔；p<0.001）和类PEHO患者（22微摩尔；p<0.003）的NNx水平均显著更高，但与年龄、脑萎缩或胰岛素样生长因子-1（IGF-1）的脑脊液水平无关。
我们的研究结果表明，在PEHO综合征中，一氧化氮的生成显著增加，提示一氧化氮参与了该疾病的病理现象（即癫痫发作和神经退行性变）。
