Krause K H, Brosi K, Rittmann M, Scheglmann K, Frank K, Ziegler R
EEG EMG Z Elektroenzephalogr Elektromyogr Verwandte Geb. 1985 Jun;16(2):101-3.
In a 31 year old patient with adrenocortical gland insufficiency and minor clinical signs of involvement of peripheral and central nervous system an adrenoleucodystrophy was diagnosed, based upon the characteristic elevation of hexacosanoic acid in plasma. The motor and sensory nerve conduction velocities in upper and lower extremities were markedly reduced; in accordance, the somatosensory evoked potentials showed distinct reduction in the peripheral part. In contrast, the interpeak latencies N 20 - NSEP 3 (median nerve) and N 22 - P 40 (tibial nerve) were in the upper normal range, so that a damage of the central sensory system could not be evaluated. The visual and brainstem acoustic evoked potentials were a little prolonged. The problems in differential diagnosis of this form of adrenoleucodystrophy, which especially involves the peripheral nervous system, are discussed.
在一名31岁患有肾上腺皮质功能不全且有轻微外周和中枢神经系统受累临床体征的患者中，基于血浆中二十六烷酸的特征性升高，诊断为肾上腺脑白质营养不良。上下肢运动和感觉神经传导速度明显降低；相应地，体感诱发电位在外周部分显示明显降低。相比之下，N 20 - NSEP 3（正中神经）和N 22 - P 40（胫神经）的峰间潜伏期在正常上限范围内，因此无法评估中枢感觉系统的损伤。视觉和脑干听觉诱发电位稍有延长。本文讨论了这种特别累及外周神经系统的肾上腺脑白质营养不良的鉴别诊断问题。
