Frasca J, Blumbergs P C, Henschke P, Burns R J
Department of Medicine (Neurology), Flinders Medical Centre, Adelaide.
Clin Exp Neurol. 1991;28:79-89.
The clinical features of 26 patients diagnosed as progressive supranuclear palsy are reviewed. The atypical findings were the relatively low frequency of visual complaints (23%) and of significant dementia (20%). As well, the characteristic eye signs, supranuclear ophthalmoplegia of vertical gaze, occurred some years after the onset of the initial symptoms in a small but substantial number (31%), which significantly delayed the diagnosis, in one case by as long as 18 years. The pathological studies on 2 of the clinical cases and an additional 6 cases showed the characteristic pattern of involvement of particular subcortical and brainstem nuclei. In particular, significant degeneration of the pedunculopontine tegmental nucleus was confirmed. Degeneration of the pedunculopontine tegmental nucleus may well play an important role in the motor disability in progressive supranuclear palsy.
对26例被诊断为进行性核上性麻痹的患者的临床特征进行了回顾。非典型表现为视觉主诉相对较少（23%）以及显著痴呆较少（20%）。同样，特征性眼征——垂直凝视核上性眼肌麻痹，在一小部分但数量可观的患者（31%）中，在初始症状出现数年之后才出现，这显著延迟了诊断，其中一例长达18年。对2例临床病例及另外6例病例的病理研究显示了特定皮质下和脑干核团受累的特征性模式。特别是，证实了脚桥被盖核有显著退变。脚桥被盖核的退变很可能在进行性核上性麻痹的运动功能障碍中起重要作用。
