Klarendic Maja, Hribar Manja, Urbancic Nina Bozanic, Zupancic Nina, Kramberger Milica G, Trost Maja, Battelino Saba, Kaski Diego, Kojovic Maja
Department of Neurology, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Department of Otorhinolaryngology, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Parkinsonism Relat Disord. 2021 Mar;84:15-22. doi: 10.1016/j.parkreldis.2021.01.003. Epub 2021 Jan 12.
Progressive supranuclear palsy (PSP) features parkinsonism characterized by early postural instability, falls and prominent eye movement abnormalities that consist of saccadic slowing, followed by gaze limitation. Nystagmus is not considered typical for PSP, being more commonly associated with multiple system atrophy.
To describe the prevalence and phenomenology of nystagmus in patients with PSP.
42 patients with probable PSP underwent detailed clinical eye movement examination. Patients with nystagmus performed video-nystagmography. T-test, Chi-Square test and Wilcoxon signed-rank test were used to test differences in demographic data, disease duration and PSP subtype between patients with and without nystagmus, and for analysis of video-nystagmographic data.
Among 42 patients with PSP, we identified 15 patients (35,7%) with gaze-evoked nystagmus, predominantly horizontal. Clinically, 10/15 patients had symmetrical or asymmetrical gaze - evoked nystagmus (Type 1), while 5/15 patients had dissociated gaze-evoked nystagmus related to internuclear ophthalmoplegia (Type 2). Nystagmus and eye movement abnormalities were further characterized by video-nystagmography. There was no significant difference in age, disease duration or PSP subtypes between patients with and without nystagmus.
Central nystagmus is present in more than a third of patients with progressive supranuclear palsy. It may present as symmetrical or asymmetrical gaze-evoked nystagmus or as dissociated gaze-evoked nystagmus related to internuclear ophthalmoplegia and probably arises from neurodegeneration of the neural integrator. Nystagmus in PSP has been a hitherto under-described feature and its presence should not deter clinicians from a diagnosis of PSP.
进行性核上性麻痹（PSP）的帕金森综合征特征包括早期姿势不稳、跌倒以及明显的眼球运动异常，表现为扫视减慢，随后出现凝视受限。眼球震颤不被认为是PSP的典型症状，更常见于多系统萎缩。
描述PSP患者中眼球震颤的患病率及表现。
42例可能患有PSP的患者接受了详细的临床眼球运动检查。有眼球震颤的患者进行了视频眼震图检查。采用t检验、卡方检验和Wilcoxon符号秩检验来检验有和没有眼球震颤的患者在人口统计学数据、病程和PSP亚型方面的差异，并分析视频眼震图数据。
在42例PSP患者中，我们确定了15例（35.7%）有凝视诱发性眼球震颤的患者，主要为水平方向。临床上，10/15例患者有对称或不对称的凝视诱发性眼球震颤（1型），而5/15例患者有与核间性眼肌麻痹相关的分离性凝视诱发性眼球震颤（2型）。通过视频眼震图进一步描述了眼球震颤和眼球运动异常。有和没有眼球震颤的患者在年龄、病程或PSP亚型方面没有显著差异。
超过三分之一的进行性核上性麻痹患者存在中枢性眼球震颤。它可能表现为对称或不对称的凝视诱发性眼球震颤，或与核间性眼肌麻痹相关的分离性凝视诱发性眼球震颤，可能源于神经整合器的神经变性。PSP中的眼球震颤是一个迄今描述不足的特征，其存在不应妨碍临床医生对PSP的诊断。
