Ochs R, Markand O N, DeMyer W E
Neurology. 1979 Aug;29(8):1089-93. doi: 10.1212/wnl.29.8.1089.
Brainstem auditory evoked responses (BAERs) were recorded in seven patients with Pelizaeus-Merzbacher leukodystrophy (PMD), two with adrenoleukodystrophy (ALD), and one with metachromatic leukodystrophy (MLD). BAERs were altered in all patients, and the alterations were severe in 9 of the 10 patients. A patient with ALD who as yet had no neurologic symptoms showed only minimal abnormality of the BAERs, consisting of prolongation of the latency of wave V. In the remaining nine patients, only wave I generated in the extramedullary portion of the eighth nerve was recorded with or without a wave II. Subsequent components (III through VII) were absent. No abnormality of BAERs was observed in the 10 known carriers of PMD. The combination of BAERs and EEG is helpful in differentiating leukodystrophy from progressive gray matter diseases.
对7例佩利措伊斯-梅茨巴赫病（PMD）、2例肾上腺脑白质营养不良（ALD）和1例异染性脑白质营养不良（MLD）患者进行了脑干听觉诱发电位（BAER）记录。所有患者的BAER均有改变，10例患者中有9例改变严重。1例尚无神经症状的ALD患者BAER仅有轻微异常，表现为V波潜伏期延长。其余9例患者，无论有无II波，仅记录到由第八神经髓外部分产生的I波。后续成分（III至VII波）缺失。10例已知的PMD携带者未观察到BAER异常。BAER和脑电图相结合有助于鉴别脑白质营养不良与进行性灰质疾病。
