Lidov H G, Duchen L W, Thomas P K, Thrush D C
Department of Neuropathology, Institute of Neurology, London, UK.
J Neurol Neurosurg Psychiatry. 1989 May;52(5):643-7. doi: 10.1136/jnnp.52.5.643.
A clinicopathological report is presented of a British male, aged 59 years, who died after an illness of 10 years, manifested by progressive respiratory failure, ptosis, and dysphagia. At no time was there evidence of ophthalmoplegia, Parkinsonism or dementia. At necropsy the main finding was of neurofibrillary tangles in the neurons of the pontine and medullary reticular formation, with particularly severe involvement of the nucleus ambiguus, dorsal motor nucleus of the vagus and nucleus tractus solitarius. Morphologically, by light and electron microscopy and immunostaining, the tangles were similar to those of other neurofibrillary degenerative diseases. Although similar in some respects to progressive supranuclear palsy and amyotrophic lateral sclerosis of the Guam type, the combination of clinical and neuropathological features suggest that this is a distinct disease entity.
本文报告了一名59岁英国男性的临床病理情况。该患者患病10年后死亡，表现为进行性呼吸衰竭、上睑下垂和吞咽困难。病程中从未出现眼肌麻痹、帕金森综合征或痴呆的证据。尸检时的主要发现是脑桥和延髓网状结构神经元中的神经原纤维缠结，疑核、迷走神经背运动核和孤束核受累尤为严重。通过光镜、电镜和免疫染色观察，这些缠结在形态学上与其他神经原纤维变性疾病的缠结相似。尽管在某些方面与进行性核上性麻痹和关岛型肌萎缩侧索硬化相似，但临床和神经病理学特征的组合表明这是一种独特的疾病实体。
