Wulff C H, Trojaborg W
Neurology. 1985 Dec;35(12):1776-8. doi: 10.1212/wnl.35.12.1776.
The visual and somatosensory evoked potentials were delayed in two cases of the adult form of metachromatic leukodystrophy. Brainstem auditory evoked potentials were normal. The conduction velocity along peripheral nerves was 50% slowed in one case and near normal in the other. The findings are compatible with demyelination in the central and peripheral nervous systems. The diagnosis of metachromatic leukodystrophy should be considered in cases of early dementia, with or without psychosis or other neurologic deficits, in which evoked potentials are delayed and peripheral nerve conduction is slowed.
在两例成人型异染性脑白质营养不良患者中，视觉和体感诱发电位延迟。脑干听觉诱发电位正常。一例患者沿周围神经的传导速度减慢50%，另一例接近正常。这些发现与中枢和周围神经系统的脱髓鞘相符。对于早期痴呆患者，无论有无精神病或其他神经功能缺损，若诱发电位延迟且周围神经传导减慢，均应考虑异染性脑白质营养不良的诊断。
