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The Problem
“My child’s eyes jiggle back and forth.”
Common Causes
Congenital motor nystagmus (vision otherwise normal)
Sensory nystagmus (nystagmus secondary to decreased vision)
Other Causes
Latent nystagmus (associated with infantile strabismus)
Voluntary nystagmus
Central nervous system tumors, malformations
Pharmacological
KEY FINDINGS
History
Congenital motor nystagmus (infantile nystagmus syndrome)
Onset in first few months of life
Vision otherwise seems normal
May be hereditary
Sensory nystagmus
Onset in first few months of life
Variable vision—profoundly impaired to near-normal
Possible family history (depending on diagnosis)
Other problems affecting development
Examination
Congenital motor nystagmus
Horizontal nystagmus
Vision seems normal
Pupils react normally
Possible abnormal head posture (to decrease nystagmus)
Sensory nystagmus
Usually horizontal, possible vertical or rotary
Vision variable (very poor to near-normal)
Poor pupil reactions
Possible abnormal head posture (to decrease nystagmus)
Other findings depending on underlying diagnosis
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Children with nystagmus should be referred for further evaluation. This is usually done most efficiently by initially referring the child to an ophthalmologist. Nystagmus that presents in infancy and early childhood is usually due to either congenital motor nystagmus or is secondary to an underlying ocular disorder. Acquired nystagmus in older children is more likely to be associated with an underlying neurological disorder. Older children with nystagmus may need to be evaluated by both an ophthalmologist and a neurologist.

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What Shouldn’t Be Missed

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Sensory nystagmus in infants may be due to septo-optic dysplasia. This is often associated with pituitary gland dysfunction. Affected infants may not be able to mount a normal stress response and are therefore at risk for decompensating with minor illnesses. If this diagnosis is suspected, the infant’s family should be warned of this possibility while waiting for an endocrinological evaluation.

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  • 1. Congenital motor nystagmus (infantile nystagmus syndrome). In congenital motor nystagmus, the eyes themselves are fine. The nystagmus results from abnormalities of the ocular motor system. Despite the nystagmus, most children see surprisingly well (Table 12–1).
  • 2. Sensory nystagmus. Any disorder that affects the vision in both eyes during infancy may present with nystagmus in the first few months of life. The prognosis for vision depends on the underlying disorder. Common etiologies include albinism, optic nerve hypoplasia (septo-optic dysplasia), and Leber’s congenital amaurosis (Table 12–2).
  • 3. Acquired nystagmus in older children. Acquired nystagmus is relatively rare in childhood. Unlike infantile nystagmus, older children with acquired nystagmus may complain of oscillopsia, the sensation of the world moving back and forth. Acquired nystagmus may result from central nervous system lesions or as a side effect of medication (Table 12–3).
  • 4. Voluntary nystagmus. Some patients are able to voluntarily elicit nystagmus. This is a high-frequency horizontal oscillation. It cannot be sustained longer than a few seconds.
  • 5. Latent nystagmus (fusion maldevelopment nystagmus). Patients with infantile esotropia develop nystagmus when one of their eyes is covered. The nystagmus is usually not visible when both ...

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