婴儿期良性间歇性上跳性眼球震颤：一种新的临床病症。

Benign intermittent upbeat nystagmus in infancy: a new clinical entity.

作者信息

Robert Matthieu P, Michel Sarah, Adjadj Elias, Boddaert Nathalie, Desguerre Isabelle, Vidal Pierre-Paul

机构信息

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.

出版信息

Eur J Paediatr Neurol. 2015 Mar;19(2):262-5. doi: 10.1016/j.ejpn.2014.12.013. Epub 2014 Dec 31.

DOI:10.1016/j.ejpn.2014.12.013

PMID:25601640

Abstract

BACKGROUND

Upbeat nystagmus in childhood is a rare phenomenon, which is classically associated with neurological or anterior visual pathway dysfunction.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.

摘要