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Management of nystagmus in children: a review of the literature and current practice in UK specialist services

A Correction to this article was published on 28 May 2020

This article has been updated

Abstract

Nystagmus is an eye movement disorder characterised by abnormal, involuntary rhythmic oscillations of one or both eyes, initiated by a slow phase. It is not uncommon in the UK and regularly seen in paediatric ophthalmology and adult general/strabismus clinics. In some cases, it occurs in isolation, and in others, it occurs as part of a multisystem disorder, severe visual impairment or neurological disorder. Similarly, in some cases, visual acuity can be normal and in others can be severely degraded. Furthermore, the impact on vision goes well beyond static acuity alone, is rarely measured and may vary on a minute-to-minute, day-to-day or month-to-month basis. For these reasons, management of children with nystagmus in the UK is varied, and patients report hugely different experiences and investigations. In this review, we hope to shine a light on the current management of children with nystagmus across five specialist centres in the UK in order to present, for the first time, a consensus on investigation and clinical management.

摘要

眼球震颤是一种眼球运动障碍以单眼或双眼异常的、不自主的节律性摆动为特征, 其发病缓慢。在英国的发病率不低, 通常就诊于小儿眼科和成人普通/斜视门诊。眼球震颤在某些情况下单发, 也可伴发多系统疾病、严重视力损害或神经系统疾病。同样, 在一些病例中, 患者视力正常, 另一些病例中, 患者视力严重下降。而且其对视力的影响远超过对静态视敏度的影响, 几乎检测不到, 而且可能在每分钟、每天或每月的基础上不断变化。基于以上原因, 在英国, 针对儿童眼球震颤的管理是多样的, 而且患者的症状与调查报告也有很大的差别。我们希望本文能从英国五个专科中心的眼球震颤儿童的现行的管理有所启发, 以便首次达成调查与临床管理共识。

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Fig. 1: How to draw a pedigree diagram whilst taking a family history.
Fig. 2: Diagnostic use of OCT in INS.
Fig. 3: Foveal tomograms obtained from children, which demonstrate some of the diagnostic features seen in INS on OCT imaging.
Fig. 4: Grading foveal hypoplasia.
Fig. 5: Examples of the use of eye tracking in clinical cases.
Fig. 6: The use of visual electrodiagnostics in diagnosing nystagmus.
Fig. 7: A diagnostic workflow that forms the basis of our clinical practice across a number of specialist paediatric nystagmus services in the UK.

Change history

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Nystagmus UK Eye research group (NUKE)

G. E. Arblaster10, A . Bjerre10, M. J. Dunn3, J. T. Erichsen3, I . Gottlob4, H. J. Griffiths5, C. Harris6, H. Lee1,2, L. McIlreavy3, J. Owen6, J. Sanders7, J. E. Self1,2, F. Shawkat1, M. Theodorou8,9, J. P. Whittle10, D. Osborne1,2, M. Ranger1,2, C. Norman2, K. MacKenzie8, N. Venturi8, TailorV8, Frank Proudlock4, Rebecca McLean4, Mervyn Thomas4, Viral Sheth4, Perry Carter1

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  1. Members of the Nystagmus UK Eye research group (NUKE) are listed at the end of the paper.

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    Self, J.E., Dunn, M.J., Erichsen, J.T. et al. Management of nystagmus in children: a review of the literature and current practice in UK specialist services. Eye 34, 1515–1534 (2020). https://doi.org/10.1038/s41433-019-0741-3

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