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Key Features

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  • May be associated with esotropia or may occur with ocular lesions that cause deprivation amblyopia (eg, cataract and eyelid ptosis) or conditions in which the visual pathways are hypoplastic, sometimes referred to as "sensory nystagmus"

  • Seen with optic nerve hypoplasia, macular hypoplasia, aniridia, and albinism

  • Can also occur with normal ocular structures and seemingly normal CNS development, sometimes referred to as "motor nystagmus"

  • Latent nystagmus occurs when one eye is occluded

    • Occurs in patients with congenital esotropia

    • An associated amblyopia may be present

  • Most nystagmus occurring in childhood is of ocular origin, but CNS disease and, less frequently, inner ear disease are other causes

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Clinical Findings

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  • Rhythmic oscillation or jiggling of the eyes

  • May be unilateral or bilateral, more pronounced in one eye, or gaze-dependent

  • Evaluation for iris transillumination defects caused by albinism should be performed since albinism is a common cause of nystagmus

  • Spasmus nutans

    • Rapid, shimmering, disconjugate nystagmus occurs with head bobbing and torticollis

    • Glioma of the hypothalamus can mimic spasmus nutans

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Diagnosis

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  • Neuroimaging may be necessary to determine whether cause is due to a CNS disease

  • An electroretinogram may be required to rule out retinal pathology

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Treatment

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  • Directed at managing the underlying ocular or CNS disease

  • An ophthalmologist can optimize vision by correcting significant refractive errors and strabismus

  • Some patients may benefit from extraocular muscle surgery and contact lenses

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