Kato S, Oda M, Tanabe H
Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan.
Neuropathol Appl Neurobiol. 1993 Aug;19(4):300-4. doi: 10.1111/j.1365-2990.1993.tb00444.x.
The substantia nigra was examined immunohistochemically using the antibody to tyrosine hydroxylase in 15 patients with sporadic amyotrophic lateral sclerosis (ALS). The number of dopaminergic neurons was diminished in the substantia nigra of seven cases. The diminution was not related to the age, duration of the illness or use of respirators. Supranuclear ophthalmoplegia developed in four and dementia in three out of seven patients with reduction of nigral dopaminergic neurons. In addition, five out of the seven patients developed respiratory failure within 2 years after the onset of the illness. The nigral dopaminergic system may be involved in rapidly progressive ALS patients with supranuclear ophthalmoplegia and/or dementia.
对15例散发性肌萎缩侧索硬化症（ALS）患者，采用抗酪氨酸羟化酶抗体进行免疫组织化学检查黑质。7例患者黑质中的多巴胺能神经元数量减少。这种减少与年龄、病程或是否使用呼吸器无关。7例黑质多巴胺能神经元减少的患者中，4例出现核上性眼肌麻痹，3例出现痴呆。此外，7例患者中有5例在发病后2年内出现呼吸衰竭。黑质多巴胺能系统可能参与伴有核上性眼肌麻痹和/或痴呆的快速进展型ALS患者。
