Internuclear Ophthalmoplegia

    • Impaired ipsilateral adduction caused by a lesion of the medial longitudinal fasciculus
    • Common causes: demyelination in youth, ischemic stroke in older adults
    • Uncommon causes: neoplasm, trauma, vascular malformation
    • Core clinical features
      • Patient reports diplopia, blurred vision, or “something wrong with my vision” on side gaze
      • Trap: patient may not report any symptoms because the eyes often eventually reach normal alignment even in side gaze
      • Absent, reduced, or slow adduction of the eye on the side of the lesion, often with jerk nystagmus in abduction of the other eye
      • Tip: adduction may appear normal on pursuit testing, so you must test saccades to bring out slow adduction
      • Eyes may be aligned in straight-ahead gaze or have exotropia
      • Eyes may be vertically misaligned (skew deviation)
      • Convergence may be spared
      • Pursuit is often saccadic
      • Jerk nystagmus is often present on side gaze or on upgaze in both eyes
    • Possible accompanying neurologic features
      • Ataxia
    • Imaging features
      • MRI may be normal or show a lesion often near the floor of the fourth ventricle
    • Myasthenia gravis
    • Third nerve palsy
    • Orbital inflammation, trauma, tumor
    • Duane syndrome type 2
    • Assess ocular versions in pursuit and saccades, looking for slow or deficient adduction
    • Look for incomitant exodeviation greatest in contralateral gaze
    • Tip: testing with the optokinetic strip or drum, which elicits repetitive saccades, helps to detect a subtle saccadic adduction deficit
    • Tip: look for nystagmus, saccadic pursuit, skew deviation, and ataxia to support a diagnosis of internuclear ophthalmoplegia
    • Trap: a misdiagnosis of partial third nerve palsy occurs often in this setting
    • Tip: the sparing of adduction in convergence helps exclude a partial third nerve palsy, but it is not a trustworthy sign or easy to interpret
    • Tip: if there is no ptosis, pupillary abnormality, deficit in vertical ductions, or incomitant vertical misalignment, the diagnosis of third nerve palsy is unlikely
    • Tip: internuclear ophthalmoplegia may display normal alignment or exodeviation is primary gaze position; the finding of normal alignment is a vote against the diagnosis of partial third nerve palsy
    • Trap: myasthenia gravis and orbital restrictive syndromes are other important imitators of internuclear ophthalmoplegia (“pseudointernuclear ophthalmoplegia”), so be sure to look for the attributes of those conditions
    • Tip: distinguishing internuclear ophthalmoplegia from partial third nerve palsy is important because internuclear ophthalmoplegia is always caused by an intra-axial (brainstem) lesion whereas partial third nerve palsy is usually caused by an extra-axial lesion; if you diagnose internuclear ophthalmoplegia, order brain MRI; if you diagnose partial third nerve palsy, order CTA to rule out aneurysm
    • Trap: in internuclear ophthalmoplegia, MRI often does not show a correlative lesion
    • Deficit may resolve spontaneously or persist
    • Normal MRI favors full recovery
    • Diplopia may be relieved with
      • Ground-in or press-on spectacle prisms
      • Eye patch
      • Partial or complete spectacle occluder
      • Opaque contact lens
      • Extraocular muscle surgery, but it is rarely necessary and may be unsuccessful

    Brainstem Ocular Motor Disorders

    Internuclear Ophthalmoplegia Skew Deviation Dorsal Midbrain Syndrome Thalamic or Tegmental Midbrain Syndrome Unilateral Pontine Syndrome Bilateral Pontine Syndrome Dorsolateral Medullary (Wallenberg) Syndrome Ototoxic Vestibulo-ocular Dysfunction Syndrome Acute Upgaze Deviation Acute Downgaze Deviation Acute Comitant Esotropia Omnidirectional Slow Saccades Omnidirectional Saccadic Pursuit