Supranuclear eye movements and nystagmus in children: A review of the literature and guide to clinical examination, interpretation of findings and age-appropriate norms

Abstract

Abnormal eye movements in children, including nystagmus, present a significant challenge to ophthalmologists and other healthcare professionals. Similarly, examination of supranuclear eye movements and nystagmus in children and interpretation of any resulting clinical signs can seem very complex. A structured assessment is often lacking although in many cases, simple clinical observations, combined with a basic understanding of the underlying neurology, can hold the key to clinical diagnosis. As the range of underlying diagnoses for children with abnormal eye movements is broad, recognising clinical patterns and understanding their neurological basis is also imperative for ongoing management. Here, we present a review and best practice guide for a structured, methodical clinical examination of supranuclear eye movements and nystagmus in children, a guide to clinical interpretation and age-appropriate norms. We also detail the more common specific clinical findings and how they should be interpreted and used to guide further management. In summary, this review will encourage clinicians to combine a structured assessment and a logical interpretation of the resulting clinical signs, in order to recognise patterns of presentation and avoid unnecessary investigations and protracted delays in diagnosis and clinical care.

Fig. 1

The vestibulo-ocular reflex (VOR). LR lateral rectus; MR medial rectus; MLF medial longitudinal fasciculus; VI sixth nerve nucleus; VIII 8th nerve (vestibular) nuclei; III third nerve nucleus. This diagram represents a right face turn head movement with eye movement to the left

Fig. 2

How to perform the doll’s head manoeuvre on an infant in a clinical setting

Fig. 3

Performing the spinning baby test in a clinical setting

Fig. 4

Using an OKR Drum in a clinical setting

Fig. 5

Saccades. LR lateral rectus; MR medial rectus; MLF medial longitudinal fosiculus; EBN excitory burst neurons; IBN inhibitory burst neurons; SC superior colliculus; PPRF pontine paramedian reticular formations. This diagram shows the pathway through the midbrain/pons of a saccade to the patient right

Fig. 6

Recording nystagmus amplitude, frequency, direction and waveform in nine positions of gaze