Apkarian P, Koetsveld-Baart J C, Barth P G
Department of Visual Systems Analysis, The Netherlands Ophthalmic Research Institute, Amsterdam.
Arch Neurol. 1993 Sep;50(9):981-5. doi: 10.1001/archneur.1993.00540090078014.
Early diagnosis of Pelizaeus-Merzbacher disease; assessment of disease progression.
Pediatric neuro-ophthalmology evaluation of visual function with pattern and luminance visual evoked potentials; behavioral state assessment; electrophysiological diagnostic test; baseline estimates at the age of 11 weeks; 1-year follow-up.
University hospital electrodiagnostic vision research laboratory.
X-linked Pelizaeus-Merzbacher disease; leukodystrophy; patient tested at the age of 11 weeks and at the age of 58 weeks for follow-up; five age-matched normal controls.
Physical therapy, medication, evaluation.
Early diagnosis; diagnostic confirmation; objective monitor of disease progression.
Abnormal spatial and temporal vision and abnormal visual pathway maturation; visual evoked potentials of proband with Pelizaeus-Merzbacher disease were grossly abnormal, reflecting myelination disorder.
Visual evoked potential pediatric electrodiagnosis yields reliable measures of visual function and visual system maturation in leukodystrophy.
早期诊断佩利措伊斯-梅茨巴赫病；评估疾病进展情况。
采用图形和亮度视觉诱发电位对视觉功能进行儿科神经眼科评估；行为状态评估；电生理诊断测试；11周龄时进行基线评估；随访1年。
大学医院电诊断视觉研究实验室。
X连锁佩利措伊斯-梅茨巴赫病；脑白质营养不良；患者在11周龄和58周龄时接受测试以进行随访；5名年龄匹配的正常对照者。
物理治疗、药物治疗、评估。
早期诊断；确诊；疾病进展的客观监测。
空间和时间视觉异常以及视觉通路成熟异常；佩利措伊斯-梅茨巴赫病先证者的视觉诱发电位严重异常，反映了髓鞘形成障碍？
视觉诱发电位儿科电诊断可提供脑白质营养不良中视觉功能和视觉系统成熟的可靠测量方法。
