Battaglia A, Nardelli E, Pampiglione G, Harden A
Ital J Neurol Sci. 1980 Jun;1(3):131-8. doi: 10.1007/BF02335842.
Repeated neurophysiological investigations over a six year period are reported in two children with histologically proven Alexander's leucodystrophy. The EEG alterations were already detectable at an early stage of the disease with subsequent progressive deterioration in both cases. There was no definite abnormality in the ERG-VEP studies which were only carried out in the younger patient. The EEG features in the course of the illness are different from those seen in most of the identified genetically determined neurometabolic diseases as well as in the group of so-called leucodystrophies of similar age groups. However, the EEG-ERG-VEP features are not sufficiently characteristic to be diagnostic.
报告了对两名经组织学证实患有亚历山大白质营养不良症儿童进行的为期六年的反复神经生理学研究。在疾病早期即可检测到脑电图改变，随后在两例中均出现进行性恶化。视网膜电图-视觉诱发电位研究仅在较年轻的患者中进行，未发现明确异常。疾病过程中的脑电图特征与大多数已确定的遗传性神经代谢疾病以及同年龄组所谓的白质营养不良症中所见的特征不同。然而，脑电图-视网膜电图-视觉诱发电位特征的诊断特异性不足。
