de Coo I F, Gabreëls F J, Renier W O, de Pont J J, van Haelst U J, Veerkamp J H, Trijbels J M, Jaspar H H, Renkawek K
Institute of Neurology, University Hospital Nijmegen, The Netherlands.
Clin Neuropathol. 1991 Mar-Apr;10(2):73-8.
In this study we present a patient with Canavan disease or Van Bogaert and Bertrand type of spongiform leukodystrophy, proven by brain biopsy. We performed morphological studies and biochemical assays on fresh homogenates of the grey and white matter. Quantitative neuromorphological analysis of the cortex showed normal values except for poor dendritic arborization of the inner layers. No signs of neuronal damage were observed. The Na-K-ATPase activity was increased. Pyruvate and ketone bodies oxidation rates and the activity of cytochrome-c oxidase were normal. We conclude that there is neither a primary neuronal damage nor a primary mitochondrial dysfunction in the oxidative processes despite the abnormal morphology of mitochondria in this disease.
在本研究中，我们报告了一名经脑活检证实患有卡纳万病或范博加特与贝特朗型海绵状脑白质营养不良的患者。我们对新鲜的灰质和白质匀浆进行了形态学研究和生化分析。对皮质的定量神经形态学分析显示，除内层树突分支不良外，其他值均正常。未观察到神经元损伤的迹象。钠钾ATP酶活性增加。丙酮酸和酮体氧化率以及细胞色素c氧化酶活性正常。我们得出结论，尽管该疾病中线粒体形态异常，但在氧化过程中既不存在原发性神经元损伤，也不存在原发性线粒体功能障碍。
