ABSTRACT
Pediatric nystagmus may be the harbinger of systemic or neurologic disease with significant morbidity and potential mortality. This paper reviews pediatriconset nystagmus, which may be associated with systemic diseases such as spasmus nutans, opsoclonus/ocular flutter, downbeat nystagmus, convergence retraction nystagmus, and see-saw nystagmus. These unique forms of nystagmus may be indicative of life- or vision-threatening diseases; thus, their recognition will facilitate earlier diagnosis and treatment, resulting in better outcomes.