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Nystagmus


Gauri S. Shrestha, M.Optom, FIACLE
What is Nystagmus?
 Involuntary and repetitive oscillatory movement
  of one or both eyes
 May be physiological or pathological or idiopathic
 May be early onset or later onset
 Often seriously reduces vision




                   Gauri S Shrestha, M.optom, FIACLE
Depth of Field
 Reduced by nystagmus
 These patients may be prone to tripping or
  clumsiness.
 Co-ordination is usually adequate for most tasks,
  but nystagmus patients are unlikely to excel at
  sports needing good hand to eye co-ordination

 Nystagmus is actually a sign not the
  diagnosis
                   Gauri S Shrestha, M.optom, FIACLE
Description
 Position (Primary or gaze-related)
 Frequency (Rapid or slow)
 Null zone:
     Nystagmus is minimal in this field of gaze (this may be
     left or right or on convergence
 Direction
    May be horizontal, vertical or rotational (Described by
     the direction of fast phase)
 Waveform (Jerk or pendular)
 Amplitude (How far the eye moves)

                      Gauri S Shrestha, M.optom, FIACLE
Representation
                  Jerky Nystagmus

                  Rt. Beating
                  Lt. beating
                  Pendular
                  Intense jerk Nystagmus
     o               No Nystagmus




            Gauri S Shrestha, M.optom, FIACLE
Diagrammatic depiction of Nystagmus




                   Gauri S Shrestha, M.optom, FIACLE

      Horizontal Nystagmus
Null Zone & Neutral Zone
 Null Zone
    The field of gaze in which nystagmus intensity is
     minimal
    The eye position in which a reversal of direction of jerk
     nystagmus occurs and in which no nystagmus
    The null and neutral zones usually overlap; however,
     several cases have been recorded where they do not.




                        Gauri S Shrestha, M.optom, FIACLE
Incidence
 Nystagmus affects about one in a thousand people.
 One survey identified one in every 670 children by
  the age of two as having nystagmus.




                   Gauri S Shrestha, M.optom, FIACLE
What Causes Nystagmus?
 May be inherited or result from a sensory problem
      some cases occur for no known reason
 It can also develop in later life, sometimes as a
  result of an accident or a range of illnesses,
  especially those affecting the motor system.




                     Gauri S Shrestha, M.optom, FIACLE
What Should We Know?
 Refractive error Correction
    Limitations

 Vision often varies during the day
      affected by emotional/physical factors such as stress,
       tiredness, nervousness or unfamiliar surroundings.
 Balance
    Poor depth perception, which can make it difficult to go up
     and down stairs.




                         Gauri S Shrestha, M.optom, FIACLE
Classification




             Gauri S Shrestha, M.optom, FIACLE
Non-Physiological Nystagmus
Based on Appearance of waveform
 1. Jerk Nystagmus
     Well-defined slow and
      fast phases
 2. Pendular Nystagmus
     No defined fast phase
      (pendulum movement)




                   Gauri S Shrestha, M.optom, FIACLE
Physiological nystagmus...
 Optokinetic nystagmus (OKN)
    Jerky nystagmus induced by
     moving patterned targets across
     the visual field.
    Slow phase along the direction
     of the moving patterns, followed
     by a fast phase in opposite
     direction.
    Also called railroad nystagmus.
    Clinical significance in detecting
     visual acuity.


                         Gauri S Shrestha, M.optom, FIACLE
Physiological nystagmus
 End point nystagmus
      Fine jerk nystagmus extreme gaze position
 Fixation nystagmus - fine oscilllatory movements
  during the maintenance of steady fixation
 Caloric nystagmus
      Jerk nystagmus caused by altered input from the
       vestibular nuclei to the horizontal gaze centers.
      Nystagmus induced by caloric test.


                      Gauri S Shrestha, M.optom, FIACLE
 Caloric test
    If hot water is irrigated into right ear – patient will
     develop right jerk nystagmus.
    Cold water into right ear – left jerk nystagmus
    COWS (cold – opposite, warm – same)
 If both ears are stimulated for
    Cold water – upbeat jerk nystagmus
    Warm water – downbeat jerk nystagmus




                      Gauri S Shrestha, M.optom, FIACLE
Classification




             Gauri S Shrestha, M.optom, FIACLE
Congenital nystagmus
 Infrequently observed at birth.
 Onset is usually during the first 3-4 months of life
  but may emerge as late as the teens.
 Prevalence is 1 in 6550. (Hemmes in 1927).
 Compensatory head nodding develops at the age
  of 20 years.




                    Gauri S Shrestha, M.optom, FIACLE
Congenital Nystagmus
 Latent nystagmus
     Jerky horizontal nystagmus seen when light stimulus
      to one or other eye diminished.
     Involuntary rhythmical oscillation of both eyes with
      fast phase to the fixing eye
     Latent nystagmus applies to patients with binocular
      single vision




                     Gauri S Shrestha, M.optom, FIACLE
Latent nystagmus…




           Gauri S Shrestha, M.optom, FIACLE
Manifest congenital nystagmus
 Nystagmus evoked when both eyes are open.
 Amplitude doesnot change on covering one eye.
     Sensory defect nystagmus.
     Motor defect nystagmus.




                    Gauri S Shrestha, M.optom, FIACLE
Sensory defect nystagmus
 Basic cause is the inadequate image formation on
  the fovea
      As a result of inadequate anterior visual pathway
       disease.
 Inadequate image formation interferes with the
  oculomotor control of fixation mechanism.
 Always bilateral & horizontal.
 Often is of pendular type
      Assumes jerky character in extreme position of
       gaze.

                      Gauri S Shrestha, M.optom, FIACLE
Motor defect nystagmus
 Primary defect is in the efferent mechanism.
 No ocular abnormalities are present.
 Amplitude & frequency may decrease or
  nystagmus may disappear completely in one
  position of gaze (null point / neutral zone).
      Visual acuity may improve at the null point /
       neutral zone.
 Patient may assume anomalous head posture to
    To assume null point.
    To improve visual acuity.

                      Gauri S Shrestha, M.optom, FIACLE
Congenital Nystagmus
 Pendular Nystagmus
    Can be found in patients with known foveal
     disorder
       Macular Scarring, Macular Hypoplasia, Optic

        Nerve Hypoplasia

 Congenital idiopathic nystagmus
    Bilateral, horizontal and jerky with the fast phase
     to the right on right gaze and vice versa.
    There is null point between the two positions and
     convergence with improved VA


                      Gauri S Shrestha, M.optom, FIACLE
 Latent manifest nystagmus
     Occurs in children with decreased vision in one eye
      where the poorly seeing eye behaves as an
      occluded eye.
     May be due to blindness in one eye or by deep
      suppression due to strabismus.




                     Gauri S Shrestha, M.optom, FIACLE
Nystagmus blockage syndrome
(NBS)
 Congenital nystagmus dampens
  with convergence or adduction.
 Demonstrates an esotropia to
  dampen nystagmus.
 Features
      Infantile esotropia
      Pseudoabducens palsy
      Manifest nystagmus occurs when
       eye moves from adduction to
       abduction.
 All patients with infantile esotropia
  should be screened for nystagmus.
                      Gauri S Shrestha, M.optom, FIACLE
Periodic alternating nystagmus
 Unusual form of congenital motor jerk nystagmus
 Patient starts with a jerk nystagmus in one direction &
  lasts for 60 – 90 sec & then slowly begins to dampen.
 A period of no nystagmus lasts for 10-20 seconds and then
  the nystagmus begins to jerk in opposite direction (60-90
  sec).
 Cycle again repeated.
 Etiology not known but is associated with oculocutaneous
  albinism.




                      Gauri S Shrestha, M.optom, FIACLE
Characteristics of Congenital
Nystagmus
 Binocular
 Similar amplitude in both eyes
 Usually uniplanar (horizontal) in all gazes
 Distinctive waveforms
 Diminished (damped) by convergence
 Increased by fixation attempt
 Superimposition of latent component
 Intensity increase at lateral gazes – fast phase towards the
  direction of gaze
 Associated head oscillation
 No oscillopsia
 Abolished in sleep




                       Gauri S Shrestha, M.optom, FIACLE
Acquired : Spasmus nutans
 combination of nystagmus, involuntary head nodding and
  abnormal head posture. Intermittent rapid small oscillation
  with onset at 3-18 months, resolution at 36 months.
 A rare constellation of ocular oscillation
      Head nodding &
      Torticollis
      Begins in infancy (usually between 4 and 18 months of age)
      Disappears in childhood (usually before 3 years of age).




                        Gauri S Shrestha, M.optom, FIACLE
Acquired
 Acquired pendular nystagmus - result of cerebellar
  or brainstem lesions. equal amplitude of
  nystagmus in all gazes
 Acquired jerk nystagmus - (slow and fast phase),
  may be horizontal, vertical or rotary, due to
  supranuclear defect

 Vestibular nystagmus - horizontal jerky
  nystagmus with rotary or vertical element, due to
  the destruction of inner ear, vestibular nerves and
  vestibular nuclei.
                   Gauri S Shrestha, M.optom, FIACLE
Acquired
 Gaze evoked nystagmus -an inability to maintain the eyes
  in a lateral or vertical gaze position. The eyes drift back to
  the primary position, then make a correction saccade to
  look in the position of defective gaze. Cause is
  supranuclear defect

 Dissociated nystagmus -oscillatory movements of eyes,
  dissimilar in direction, amplitude and speed. Cause is
  internuclear ophthalmoplegia

 Convergence retraction nystagmus -on attempted upgaze,
  eyes are converging and retracting with nystagmoid jerk
  movements
                       Gauri S Shrestha, M.optom, FIACLE
See-saw nystagmus
 Unusual & dramatic type.
 Has both vertical & Torsional
    components.
   Eyes make alternating
    movement of elevation &
    intorsion followed by
    depression & extorsion.
   Often associated with lesions
    in the rostral midbrain or the
    suprasellar area.
   Visual field defects are
    bitemporal hemianopia.
   Neuroradiologic evaluation
    mandatory.           Gauri S Shrestha, M.optom, FIACLE
 Downbeat nystagmus: jerky vertical nystagmus
  seen with increased amplitude on downgaze. The
  eyes drift up and beat down again. Cause is CNS
  lesion
 Upbeat nystagmus :jerky vertical nystagmus seen
  with increased amplitude on upgaze. The eyes
  drift down and beat up again. Cause is lesion of
  cerebellum or medulla.
 Periodic alternating nystagmus :jerk nystagmus
  is seen which alters in direction with every few
  minutes. Cause may be drugs or multiple sclerosis
                  Gauri S Shrestha, M.optom, FIACLE
Downbeat nystagmus




          Gauri S Shrestha, M.optom, FIACLE
Upbeat nystagmus




           Gauri S Shrestha, M.optom, FIACLE
Nystagmus Management
 Neurological Work-up and appropriate
  medical treatment
      Refer if necessary
 Provide Best Corrected VA
   Recommend with contact lenses
 Utilize Null Point
   Certain eye/head position that minimizes
     nystagmus
 Vision Therapy


                    Gauri S Shrestha, M.optom, FIACLE
How Can You Utilize Null Point?
 Locate with version (ocular motility) testing
 Use of Prisms:
    Base-Out: if convergence reduces nystagmus
    Yoked: in extreme head turn reduces nystagmus
       Base toward Null point to reduce head turn

       Use Equal power (RE, LE)


 EOM Surgery:
      Move Null point to primary position




                           Gauri S Shrestha, M.optom, FIACLE
Gauri S Shrestha, M.optom, FIACLE
Gauri S Shrestha, M.optom, FIACLE
Gauri S Shrestha, M.optom, FIACLE
Near Work With Nystagmus
 The angle of vision is important.
    Null point. Adopt a head posture
 Small print.
    Visual Aids, Large print materials.
 Good Lighting
    Be Careful with Light sensitive paients
 Reading speed
    Reduced due to the extra time needed to scan
    Should not be taken as a sign of poor reading.




                        Gauri S Shrestha, M.optom, FIACLE

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Nystagmus

  • 2. What is Nystagmus?  Involuntary and repetitive oscillatory movement of one or both eyes  May be physiological or pathological or idiopathic  May be early onset or later onset  Often seriously reduces vision Gauri S Shrestha, M.optom, FIACLE
  • 3. Depth of Field  Reduced by nystagmus  These patients may be prone to tripping or clumsiness.  Co-ordination is usually adequate for most tasks, but nystagmus patients are unlikely to excel at sports needing good hand to eye co-ordination  Nystagmus is actually a sign not the diagnosis Gauri S Shrestha, M.optom, FIACLE
  • 4. Description  Position (Primary or gaze-related)  Frequency (Rapid or slow)  Null zone:  Nystagmus is minimal in this field of gaze (this may be left or right or on convergence  Direction  May be horizontal, vertical or rotational (Described by the direction of fast phase)  Waveform (Jerk or pendular)  Amplitude (How far the eye moves) Gauri S Shrestha, M.optom, FIACLE
  • 5. Representation Jerky Nystagmus Rt. Beating Lt. beating Pendular Intense jerk Nystagmus o No Nystagmus Gauri S Shrestha, M.optom, FIACLE
  • 6. Diagrammatic depiction of Nystagmus Gauri S Shrestha, M.optom, FIACLE Horizontal Nystagmus
  • 7. Null Zone & Neutral Zone  Null Zone  The field of gaze in which nystagmus intensity is minimal  The eye position in which a reversal of direction of jerk nystagmus occurs and in which no nystagmus  The null and neutral zones usually overlap; however, several cases have been recorded where they do not. Gauri S Shrestha, M.optom, FIACLE
  • 8. Incidence  Nystagmus affects about one in a thousand people.  One survey identified one in every 670 children by the age of two as having nystagmus. Gauri S Shrestha, M.optom, FIACLE
  • 9. What Causes Nystagmus?  May be inherited or result from a sensory problem  some cases occur for no known reason  It can also develop in later life, sometimes as a result of an accident or a range of illnesses, especially those affecting the motor system. Gauri S Shrestha, M.optom, FIACLE
  • 10. What Should We Know?  Refractive error Correction  Limitations  Vision often varies during the day  affected by emotional/physical factors such as stress, tiredness, nervousness or unfamiliar surroundings.  Balance  Poor depth perception, which can make it difficult to go up and down stairs. Gauri S Shrestha, M.optom, FIACLE
  • 11. Classification Gauri S Shrestha, M.optom, FIACLE
  • 12. Non-Physiological Nystagmus Based on Appearance of waveform 1. Jerk Nystagmus  Well-defined slow and fast phases 2. Pendular Nystagmus  No defined fast phase (pendulum movement) Gauri S Shrestha, M.optom, FIACLE
  • 13. Physiological nystagmus...  Optokinetic nystagmus (OKN)  Jerky nystagmus induced by moving patterned targets across the visual field.  Slow phase along the direction of the moving patterns, followed by a fast phase in opposite direction.  Also called railroad nystagmus.  Clinical significance in detecting visual acuity. Gauri S Shrestha, M.optom, FIACLE
  • 14. Physiological nystagmus  End point nystagmus  Fine jerk nystagmus extreme gaze position  Fixation nystagmus - fine oscilllatory movements during the maintenance of steady fixation  Caloric nystagmus  Jerk nystagmus caused by altered input from the vestibular nuclei to the horizontal gaze centers.  Nystagmus induced by caloric test. Gauri S Shrestha, M.optom, FIACLE
  • 15.  Caloric test  If hot water is irrigated into right ear – patient will develop right jerk nystagmus.  Cold water into right ear – left jerk nystagmus  COWS (cold – opposite, warm – same)  If both ears are stimulated for  Cold water – upbeat jerk nystagmus  Warm water – downbeat jerk nystagmus Gauri S Shrestha, M.optom, FIACLE
  • 16. Classification Gauri S Shrestha, M.optom, FIACLE
  • 17. Congenital nystagmus  Infrequently observed at birth.  Onset is usually during the first 3-4 months of life but may emerge as late as the teens.  Prevalence is 1 in 6550. (Hemmes in 1927).  Compensatory head nodding develops at the age of 20 years. Gauri S Shrestha, M.optom, FIACLE
  • 18. Congenital Nystagmus  Latent nystagmus  Jerky horizontal nystagmus seen when light stimulus to one or other eye diminished.  Involuntary rhythmical oscillation of both eyes with fast phase to the fixing eye  Latent nystagmus applies to patients with binocular single vision Gauri S Shrestha, M.optom, FIACLE
  • 19. Latent nystagmus… Gauri S Shrestha, M.optom, FIACLE
  • 20. Manifest congenital nystagmus  Nystagmus evoked when both eyes are open.  Amplitude doesnot change on covering one eye.  Sensory defect nystagmus.  Motor defect nystagmus. Gauri S Shrestha, M.optom, FIACLE
  • 21. Sensory defect nystagmus  Basic cause is the inadequate image formation on the fovea  As a result of inadequate anterior visual pathway disease.  Inadequate image formation interferes with the oculomotor control of fixation mechanism.  Always bilateral & horizontal.  Often is of pendular type  Assumes jerky character in extreme position of gaze. Gauri S Shrestha, M.optom, FIACLE
  • 22. Motor defect nystagmus  Primary defect is in the efferent mechanism.  No ocular abnormalities are present.  Amplitude & frequency may decrease or nystagmus may disappear completely in one position of gaze (null point / neutral zone).  Visual acuity may improve at the null point / neutral zone.  Patient may assume anomalous head posture to  To assume null point.  To improve visual acuity. Gauri S Shrestha, M.optom, FIACLE
  • 23. Congenital Nystagmus  Pendular Nystagmus  Can be found in patients with known foveal disorder  Macular Scarring, Macular Hypoplasia, Optic Nerve Hypoplasia  Congenital idiopathic nystagmus  Bilateral, horizontal and jerky with the fast phase to the right on right gaze and vice versa.  There is null point between the two positions and convergence with improved VA Gauri S Shrestha, M.optom, FIACLE
  • 24.  Latent manifest nystagmus  Occurs in children with decreased vision in one eye where the poorly seeing eye behaves as an occluded eye.  May be due to blindness in one eye or by deep suppression due to strabismus. Gauri S Shrestha, M.optom, FIACLE
  • 25. Nystagmus blockage syndrome (NBS)  Congenital nystagmus dampens with convergence or adduction.  Demonstrates an esotropia to dampen nystagmus.  Features  Infantile esotropia  Pseudoabducens palsy  Manifest nystagmus occurs when eye moves from adduction to abduction.  All patients with infantile esotropia should be screened for nystagmus. Gauri S Shrestha, M.optom, FIACLE
  • 26. Periodic alternating nystagmus  Unusual form of congenital motor jerk nystagmus  Patient starts with a jerk nystagmus in one direction & lasts for 60 – 90 sec & then slowly begins to dampen.  A period of no nystagmus lasts for 10-20 seconds and then the nystagmus begins to jerk in opposite direction (60-90 sec).  Cycle again repeated.  Etiology not known but is associated with oculocutaneous albinism. Gauri S Shrestha, M.optom, FIACLE
  • 27. Characteristics of Congenital Nystagmus  Binocular  Similar amplitude in both eyes  Usually uniplanar (horizontal) in all gazes  Distinctive waveforms  Diminished (damped) by convergence  Increased by fixation attempt  Superimposition of latent component  Intensity increase at lateral gazes – fast phase towards the direction of gaze  Associated head oscillation  No oscillopsia  Abolished in sleep Gauri S Shrestha, M.optom, FIACLE
  • 28. Acquired : Spasmus nutans  combination of nystagmus, involuntary head nodding and abnormal head posture. Intermittent rapid small oscillation with onset at 3-18 months, resolution at 36 months.  A rare constellation of ocular oscillation  Head nodding &  Torticollis  Begins in infancy (usually between 4 and 18 months of age)  Disappears in childhood (usually before 3 years of age). Gauri S Shrestha, M.optom, FIACLE
  • 29. Acquired  Acquired pendular nystagmus - result of cerebellar or brainstem lesions. equal amplitude of nystagmus in all gazes  Acquired jerk nystagmus - (slow and fast phase), may be horizontal, vertical or rotary, due to supranuclear defect  Vestibular nystagmus - horizontal jerky nystagmus with rotary or vertical element, due to the destruction of inner ear, vestibular nerves and vestibular nuclei. Gauri S Shrestha, M.optom, FIACLE
  • 30. Acquired  Gaze evoked nystagmus -an inability to maintain the eyes in a lateral or vertical gaze position. The eyes drift back to the primary position, then make a correction saccade to look in the position of defective gaze. Cause is supranuclear defect  Dissociated nystagmus -oscillatory movements of eyes, dissimilar in direction, amplitude and speed. Cause is internuclear ophthalmoplegia  Convergence retraction nystagmus -on attempted upgaze, eyes are converging and retracting with nystagmoid jerk movements Gauri S Shrestha, M.optom, FIACLE
  • 31. See-saw nystagmus  Unusual & dramatic type.  Has both vertical & Torsional components.  Eyes make alternating movement of elevation & intorsion followed by depression & extorsion.  Often associated with lesions in the rostral midbrain or the suprasellar area.  Visual field defects are bitemporal hemianopia.  Neuroradiologic evaluation mandatory. Gauri S Shrestha, M.optom, FIACLE
  • 32.  Downbeat nystagmus: jerky vertical nystagmus seen with increased amplitude on downgaze. The eyes drift up and beat down again. Cause is CNS lesion  Upbeat nystagmus :jerky vertical nystagmus seen with increased amplitude on upgaze. The eyes drift down and beat up again. Cause is lesion of cerebellum or medulla.  Periodic alternating nystagmus :jerk nystagmus is seen which alters in direction with every few minutes. Cause may be drugs or multiple sclerosis Gauri S Shrestha, M.optom, FIACLE
  • 33. Downbeat nystagmus Gauri S Shrestha, M.optom, FIACLE
  • 34. Upbeat nystagmus Gauri S Shrestha, M.optom, FIACLE
  • 35. Nystagmus Management  Neurological Work-up and appropriate medical treatment  Refer if necessary  Provide Best Corrected VA  Recommend with contact lenses  Utilize Null Point  Certain eye/head position that minimizes nystagmus  Vision Therapy Gauri S Shrestha, M.optom, FIACLE
  • 36. How Can You Utilize Null Point?  Locate with version (ocular motility) testing  Use of Prisms:  Base-Out: if convergence reduces nystagmus  Yoked: in extreme head turn reduces nystagmus  Base toward Null point to reduce head turn  Use Equal power (RE, LE)  EOM Surgery:  Move Null point to primary position Gauri S Shrestha, M.optom, FIACLE
  • 37. Gauri S Shrestha, M.optom, FIACLE
  • 38. Gauri S Shrestha, M.optom, FIACLE
  • 39. Gauri S Shrestha, M.optom, FIACLE
  • 40. Near Work With Nystagmus  The angle of vision is important.  Null point. Adopt a head posture  Small print.  Visual Aids, Large print materials.  Good Lighting  Be Careful with Light sensitive paients  Reading speed  Reduced due to the extra time needed to scan  Should not be taken as a sign of poor reading. Gauri S Shrestha, M.optom, FIACLE