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病例报告:以姿势性不稳为主的进行性核上性麻痹中的眼球跳动性乒乓样凝视。

Case report: Saccadic ping-pong gaze in progressive supranuclear palsy with predominant postural instability.

作者信息

Nunomura Hikari, Kasahara Taketoshi, Hatano Taku, Shimada Hitoshi, Takado Yuhei, Endo Hironobu, Inoshita Ayako, Inomata Atsuko, Murofushi Toshihisa, Misawa Shihoko, Machida Yutaka, Imai Hisamasa

机构信息

Department of Neurology, Tokyo Rinkai Hospital, Tokyo, Japan.

Division of Rehabilitation, Tokyo Rinkai Hospital, Tokyo, Japan.

出版信息

Front Neurol. 2023 Mar 1;14:1100931. doi: 10.3389/fneur.2023.1100931. eCollection 2023.

Abstract

We report a 63-year-old female patient with progressive supranuclear palsy (PSP). She presented predominant postural instability and "saccadic ping-pong gaze" (SPPG). She had unprovoked falls recurrently within a year from the onset of gait disturbance. She tended to fall backward with eye closure but had no freezing of gait on examination. She showed no signs of nuchal dystonia, limb tremor, rigidity, spasticity, or ataxia. The dopaminergic response was negative. On the initial examination, her vertical eye movements were normal, but frequent macro square wave jerks and SPPG were observed. SPPG consisted of short-cycle, horizontal conjugate irregular pendular oscillations of the eye position from the midpoint with superimposed small saccades. SPPG was observed usually in the dark, not in the daylight, and with eye closure by using electrooculogram and infrared charge-coupled device imaging. One and a half years after the first examination, she was diagnosed as probable PSP with vertical supranuclear gaze palsy. SPPG was first described in patients who are unconscious by Johkura in 1998 as a "saccadic" variant of "ping-pong gaze (PPG)." PPG, short-cycle periodic alternating gaze, has been described in comatose patients since 1967. On the other hand, abnormal eye movement, which looks the same as SPPG in coma, has been described in conscious patients with PSP or spinocerebellar degeneration (SCD) in Japanese literature since 1975. However, it has been called "transient alternating saccades (TAS)." Nowadays, we believe it is more appropriate to call this abnormal eye movement "SPPG" instead of TAS. Here, we propose that PSP, a neuro-degenerative disease, should be added as one of the etiologies of SPPG. We discuss the differences between PPG/SPPG in coma and SPPG in PSP and the possible pathophysiological mechanism of SPPG in relation to cerebellar oculomotor dysfunctions.

摘要

我们报告了一名63岁的进行性核上性麻痹(PSP)女性患者。她主要表现为姿势性不稳和“眼球跳动性乒乓样凝视”(SPPG)。自步态障碍发作起一年内,她反复无故跌倒。她倾向于在闭眼时向后跌倒,但检查时无步态冻结现象。她没有颈部肌张力障碍、肢体震颤、僵硬、痉挛或共济失调的体征。多巴胺能反应为阴性。初次检查时,她的垂直眼球运动正常,但观察到频繁的粗大方波急跳和SPPG。SPPG由眼球位置从中点开始的短周期、水平共轭不规则摆动组成,并叠加有小的眼球跳动。通过眼电图和红外电荷耦合器件成像观察到,SPPG通常在黑暗中出现,而非在日光下,且在闭眼时出现。首次检查一年半后,她被诊断为可能的PSP伴垂直核上性凝视麻痹。SPPG最早由Johkura于1998年在无意识患者中描述为“乒乓样凝视(PPG)”的一种“眼球跳动性”变体。自1967年以来,PPG(短周期周期性交替凝视)已在昏迷患者中被描述。另一方面,自1975年以来,日本文献中已描述了在有意识的PSP或脊髓小脑变性(SCD)患者中出现的与昏迷中SPPG外观相同的异常眼球运动。然而,它一直被称为“短暂交替性眼球跳动(TAS)”。如今,我们认为将这种异常眼球运动称为“SPPG”而非TAS更为合适。在此,我们提出神经退行性疾病PSP应被添加为SPPG的病因之一。我们讨论了昏迷中的PPG/SPPG与PSP中的SPPG之间的差异以及与小脑动眼功能障碍相关的SPPG可能的病理生理机制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/10014735/85625bb5c448/fneur-14-1100931-g0001.jpg

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