犬发作性睡病中的神经元变性
Neuronal degeneration in canine narcolepsy.
作者信息
Siegel J M, Nienhuis R, Gulyani S, Ouyang S, Wu M F, Mignot E, Switzer R C, McMurry G, Cornford M
机构信息
Veterans Administration Medical Center Sepulveda and Department of Psychiatry and Brain Research Institute, University of California Los Angeles School of Medicine, Neurobiology Research 151A3, Sepulveda, California 91343, USA.
出版信息
J Neurosci. 1999 Jan 1;19(1):248-57. doi: 10.1523/JNEUROSCI.19-01-00248.1999.
Narcolepsy is a lifelong illness characterized by persistent sleepiness, hypnagogic hallucinations, and episodes of motor paralysis called cataplexy. We have tested the hypothesis that a transient neurodegenerative process is linked to symptom onset. Using the amino-cupric silver stain on brain sections from canine narcoleptics, we found elevated levels of axonal degeneration in the amygdala, basal forebrain (including the nucleus of the diagonal band, substantia innominata, and preoptic region), entopeduncular nucleus, and medial septal region. Reactive neuronal somata, an indicator of neuronal pathology, were found in the ventral amygdala. Axonal degeneration was maximal at 2-4 months of age. The number of reactive cells was maximal at 1 month of age. These degenerative changes precede or coincide with symptom onset. The forebrain degeneration that we have observed can explain the major symptoms of narcolepsy.
发作性睡病是一种终身性疾病，其特征为持续嗜睡、入睡前幻觉以及称为猝倒的运动麻痹发作。我们检验了一种假说，即短暂的神经退行性过程与症状发作有关。通过对犬发作性睡病患者脑切片进行氨基铜银染色，我们发现杏仁核、基底前脑（包括斜角带核、无名质和视前区）、内缰核和内侧隔区的轴突变性水平升高。在腹侧杏仁核中发现了反应性神经元胞体，这是神经元病理学的一个指标。轴突变性在2至4个月大时最为严重。反应性细胞数量在1个月大时最多。这些退行性变化先于症状发作或与之同时出现。我们观察到的前脑变性可以解释发作性睡病的主要症状。
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