Robert Matthieu P, Michel Sarah, Adjadj Elias, Boddaert Nathalie, Desguerre Isabelle, Vidal Pierre-Paul
AP-HP, Hôpital Universitaire Necker-Enfants Malades, Service d'ophtalmologie, Rue de Sèvres, 75015 Paris, France; COGNition and ACtion Group (COGNAC-G), UMR 8257: CNRS-IRBA-Université Paris Descartes, Sorbonne Paris Cité, France.
AP-HP, Hôpital Universitaire Necker-Enfants Malades, Service d'ophtalmologie, Rue de Sèvres, 75015 Paris, France.
Eur J Paediatr Neurol. 2015 Mar;19(2):262-5. doi: 10.1016/j.ejpn.2014.12.013. Epub 2014 Dec 31.
Upbeat nystagmus in childhood is a rare phenomenon, which is classically associated with neurological or anterior visual pathway dysfunction.
We report a series of five infants with an intermittent upbeat nystagmus, without any other ocular or neurological abnormality, having spontaneously resolved before the age of 14 months.
Nystagmus beating frequency was between 1.5 and 2.5 Hz for all patients, amplitude was remarkably large due to a baseline position in downgaze. The nystagmus mostly occurred in supine position and could be triggered by head rotations in the supine position. Age of onset was from the first weeks of life to two months; age of resolution was 5-14 months. All infants underwent complete clinical examination with ophthalmological, oto-neurological and neurological assessments and MRI-scan.
Clinical characteristics of the nystagmus suggest an involvement of the otolithic system, with a secondary recalibration of the vestibulo-ocular pathways; however, no obvious vestibular abnormality was found. The pathophysiology of this nystagmus therefore remains unclear.
儿童期向上性眼球震颤是一种罕见现象，经典地与神经或前部视觉通路功能障碍相关。
我们报告了一系列五例间歇性向上性眼球震颤的婴儿，无任何其他眼部或神经异常，在14个月龄前自发缓解。
所有患者的眼球震颤跳动频率在1.5至2.5赫兹之间，由于向下注视时的基线位置，振幅非常大。眼球震颤大多发生在仰卧位，可由仰卧位时的头部旋转触发。发病年龄从出生后第一周开始至两个月；缓解年龄为5至14个月。所有婴儿均接受了全面的临床检查，包括眼科、耳神经科和神经科评估以及磁共振成像扫描。
眼球震颤的临床特征提示耳石系统受累，前庭眼通路继发性重新校准；然而，未发现明显的前庭异常。因此，这种眼球震颤的病理生理学仍不清楚。
