Index

A Nystagmus Diagnostic Aid

© Lars Frisén 2005

The following is an aid for the diagnosis of spontaneous nystagmus that depends on bedside observations only. While these observations are simple enough, they need to be recorded carefully. For each of the five cardinal directions of gaze, note whether there is nystagmus or not, and note its character (jerking, pendular, or rotatory) and the direction of any fast phase.


Right
eye
  Left
eye
 
 
 
   
©LF

The observations can be summarized in a small table, or two tables, if nystagmus is dissociated, as in the above schematic example:


 Right eye   Left eye 
 0     0  
0 0 No adduction   0 0 Jerk ->
  0     0  


The example depicts the combination of an adduction defect in the right eye with an abduction nystagmus in the other eye. The nystagmus is of the jerk type and the direction of the rapid phase is towards the left. There is no nystagmus in other directions of gaze. This is the typical picture of a complete, unilateral, internuclear ophthalmoplegia, INO. Additional schematic representations of nystagmus can be viewed in separate windows [ 1, 2, 3, 4].

Note: the examples contain sets of moving images (animated GIFs). Technical limitations may prevent the set members to synchronize properly. Although poor sync is distractive, it is still possible to arrive at a correct evaluation. Just concentrate on each direction of gaze separately.

A large collection of live video recordings of nystagmus is maintained at NOVEL, the Neuro-Ophthalmology Virtual Education Library. A smaller collection is maintained elsewhere on this site.

There are several forms of non-spontaneous nystagmus, i e, nystagmus that has to be induced by one maneuver or another. The diagnostic aid does not cover non-spontaneous forms of nystagmus. Perhaps some common variants should be mentioned briefly:

NameMode of inductionComment
CaloricIrrigation of earNormal phenomenon
Convergence/retraction   Upward saccadeSee example #4 above
LatentOcclusion of one eye  Congenital
OptokineticVisualLink to OKN page
Peripheral vestibularFrenzel gogglesLink to review
PositionalSpecific position Link to review
Abnormal VORHead rotationLink to EOM section  
VoluntaryAt willBrief spells (exhausting!)  


Diagnostic aid

This diagnostic aid for spontaneous nystagmus works by a successive elimination of alternatives. Read the various alternatives carefully and click on the one that is appropriate for the situation. Each selected alternative will be appended to the window top line to provide a terse summary. An erroneous selection will naturally lead to an erroneous diagnosis. An erroneous choice can be undone by clicking the browser's BACK button. If you happen to go astray, use the TOP link to return to the starting point.

Note that the diagnosis presupposes that only one form of nystagmus is present and that all entries are correct. To begin, select one of the following:



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Nystagmus in primary position



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Nystagmus in primary position, horizontal



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Nystagmus in primary position, horizontal, conjugated



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Nystagmus in primary position, horizontal, conjugated, invariable



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Nystagmus in primary position, horizontal, conjugated, invariable, pendular

This form of nystagmus is most often congenital but can also occur in advanced demyelinating disease. In the former case nystagmus tends to remain horizontal on upgaze, and it is often combined with compensatory head turning. In advanced demyelinating disease nystagmus is often irregular and dissociated, and combined with oscillopsia.

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Nystagmus in primary position, horizontal, conjugated, invariable, jerking



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Nystagmus in primary position, horizontal, conjugated, invariable, jerking, constant vector in horizontal gaze:

This form of nystagmus is commonly termed vestibular nystagmus. It may or may not contain a rotatory component.

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Nystagmus in primary position, horizontal, conjugated, invariable, jerking, changing vector in horizontal gaze:

This is so-called Bruns nystagmus, which is typical of a cerebello-pontine angle lesion, e g, an acoustic neurinoma. The nystagmus is coarser in amplitude on gaze to the side of the lesion.

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Nystagmus in primary position, horizontal, conjugated, periodically alternating



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Nystagmus in primary position, horizontal, conjugated, periodically alternating, pendular:

This form of nystagmus is most commonly congenital but may also occur in advanced demyelinating disease.

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Nystagmus in primary position, horizontal, conjugated, periodically alternating, jerking:

So-called periodic alternating nystagmus is usually congenital but may be due to acquired posterior fossa lesions, typically in the midline.

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Nystagmus in primary position, horizontal, conjugated, evanescent:

This transient form of nystagmus is most commonly seen in the primary position after sustained horizontal gaze, in cerebellar lesions. It is termed rebound nystagmus.

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Nystagmus in primary position, horizontal, dissociated:

Dissociated horizontal nystagmus in adults is most commonly due to demyelinating disease or Wernicke's disease. In children, diencephalic lesions and spasmus nutans need to be considered.

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Nystagmus in primary position, vertical



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Nystagmus in primary position, vertical, conjugated



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Nystagmus in primary position, vertical, conjugated, beating upwards:

Upbeat nystagmus is usually due to midline posterior fossa lesions or Wernicke's disease.

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Nystagmus in primary position, vertical, conjugated, beating downwards:

Downbeat nystagmus is usually due to a midline lesion close by the foramen magnum, but also occurs in an idiopathic form.

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Nystagmus in primary position, vertical, dissociated



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Nystagmus in primary position, vertical, dissociated, bilateral



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Nystagmus in primary position, vertical, dissociated, bilateral, regularly alternating:

See-saw nystagmus is often combined with bitemporal hemianopia, pointing towards a diencephalic lesion, but it may also occur with brainstem lesions.

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Nystagmus in primary position, vertical, dissociated, bilateral, invariable:

Downbeat nystagmus is usually due to a midline lesion close by the foramen magnum, but also occurs in an idiopathic form.

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Nystagmus in primary position, vertical, dissociated, monocular:

Dissociated vertical nystagmus is usually due to demyelinating disease in adults, or to bilateral blindness. Diencephalic tumors and spasmus nutans need to be considered in children.

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Nystagmus in primary position, rotatory

  • Nystagmus is conjugated
  • Nystagmus is dissociated
  • Nystagmus is alternating


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    Nystagmus in primary position, rotatory, conjugated

  • Nystagmus is purely rotatory
  • There is a large horizontal or vertical component


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    Nystagmus in primary position, rotatory, conjugated, pure rotatory:

    Rotatory nystagmus is a sign of medullary disease, e.g. syringobulbia.

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    Nystagmus in primary position, rotatory, conjugated, mixed:

    Mixed rotatory nystagmus signals a vestibular lesion, commonly peripheral.

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    Nystagmus in primary position, rotatory, dissociated:

    Dissociated rotatory nystagmus is usually due to demyelinating disease.

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    Nystagmus in primary position, rotatory, alternating:

    See-saw nystagmus is often combined with bitemporal hemianopia, pointing towards a diencephalic lesion but it may also occur with brainstem lesions.

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    Nystagmus occurs only outside the primary position



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    Nystagmus in horizontal gaze



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    Nystagmus in horizontal gaze, conjugated



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    Nystagmus in horizontal gaze, conjugated, symmetrical



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    Nystagmus in horizontal gaze, conjugated, symmetrical, only outside 40 degrees:

    Symmetrical endpoint nystagmus may be physiological or due to drugs.

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    Nystagmus in horizontal gaze, conjugated, symmetrical, at all gaze angles:

    Symmetrical gaze-evoked nystagmus may be physiological, or caused by drugs, or a midline posterior fossa lesion.

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    Nystagmus in horizontal gaze, conjugated, asymmetrical



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    Nystagmus in horizontal gaze, conjugated, asymmetrical, in one gaze direction only:

    Gaze-paretic nystagmus is due to an ipsilateral pontine lesion.

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    Nystagmus in horizontal gaze, conjugated, asymmetrical, in both directions:

    This is so-called Bruns nystagmus, which is typical of a cerebellopontine angle lesion, e g, an acoustic neurinoma. The nystagmus is coarser in amplitude on gaze to the side of the lesion.

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    Nystagmus in horizontal gaze, dissociated



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    Nystagmus in horizontal gaze, dissociated, restricted movement:

    Muscle-paretic nystagmus is usually due to myopathy or a sixth nerve lesion.

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    Nystagmus in horizontal gaze, dissociated, restricted adduction:

    Unilateral and bilateral internuclear ophthalmoplegia signifies a lesion in the pontomesencephalic area, and is usually due to demyelinating disease or a microvascular lesion. Myopathy may mimic INO. Bilateral INO is typically combined with up-beating nystagmus on upgaze.

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    Nystagmus occurs primarily in vertical gaze



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    Nystagmus occurs primarily in vertical gaze, always vertical



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    Nystagmus occurs primarily in vertical gaze, always vertical, upbeating:

    Upbeating nystagmus is typical of vermis lesions.

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    Nystagmus occurs primarily in vertical gaze, always vertical, downbeating:

    Downbeat nystagmus is usually due to a midline lesion close by the foramen magnum, but also occurs in an idiopathic form.

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    Nystagmus occurs primarily in vertical gaze, changing vector:

    This type of nystagmus is most likely due to drugs but may be caused by a midline posterior fossa lesion.

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