Abstract

The pathology, clinical features, and disorders associated with optic nerve hypoplasia in children are reviewed from the Tennent Institute of Ophthalmology, Weston Infirmary, Glasgow, Scotland.

Highlights

  • TWenty-eight patients (14 females and 14 males aged between four and 34 years) with myelomeningocele and Chiari malformations were examined neuro-ophthalmologically at the Karolinska Institute, Huddinge University Hospital, Huddinge, Sweden

  • Downbeat nystagnus has been considered almost pathognomonic of the Chiari malformation but was absent in the cases in this study

  • A girl aged ten referred to me recently because of migraine headaches had downbeat nystagmus and a refractive error; the MRI was negative for Chiari malformation and brain stem auditory evoked responses were normal

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Summary

Introduction

TWenty-eight patients (14 females and 14 males aged between four and 34 years) with myelomeningocele and Chiari malformations were examined neuro-ophthalmologically at the Karolinska Institute, Huddinge University Hospital, Huddinge, Sweden. The Chiari malformation, determined with MRI, was type I in three patients and type II in 25. No signs of optic atrophy or other changes in the visual pathways were found.

Results
Conclusion
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