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  • Jerk Nystagmus: saccadic, slow movement away from fixation and fast, corrective saccade back to fixation
  • Nystagmus direction is defined by the direction of the corrective saccade
  • Pendular nystagmus: not saccadic, slow movements in both directions at the same speed


  • Eyes stable in primary position?
  • Abnormal eye movements in cardinal positions?
  • Triggered by certain direction of gaze?
  • Unilateral or bilateral?
  • Movements in both eyes the same or dissociated?
  • Phases
    • only slow?
    • slow and fast?
    • only fast?
  • Is there a null point?


MRI necessary

  • if unclear whether congenital or acquired
  • Onset of nystagmus after 6-8 months of age
  • Dissociated nystagmus
  • Purely vertical or torsional nystagmus (without genetic disorders)
  • See-saw nystagmus
  • Spasmus nutans
  • Gaze direction nystagmus / rebound nystagmus
  • Vestibular nystagmus
  • Bruns nystagmus
  • Downbeat nystagmus
  • Upbeat nystagmus
  • Convergence retraction nystagmus
  • Periodic alternating nystagmus (PAN)
  • Saccadic intrusions/oscillations

No MRI necessary

  • Infantile Idiopathic Nystagmus (IIN)
    • Sensory nystagmus = IIN with pathology
      • Evidence of delayed visual development?
      • Eye pathologies: Corneal opacities, cataract, aniridia, coloboma, microphthalmia, albinism
      • MRI might be necessary in some cases, e.g. in optic hypoplasia
    • Fusion Maldevelopment Nystagmus syndrome (latent nystagmus)
      • Only after occlusion of one eye
      • Direction towards the uncovered eye (direction changes when other eye is occluded)
      • Associated with infantile esotropia

Localising Forms of Nystagmus

  • Down = Cervicomedullary
  • Upbeat = Vermis
  • Convergence-Retraction = Dorsal midbrain
  • See-Saw = Third ventricular area / parasellar region
  • Bruns = Cerebellopontine angle
    • Rebound, PAN = Cerebellum

Physiologic Nystagmus

End-Gaze Nystagmus

  • Triggered by extreme lateral gaze (>45°)
  • Low amplitude horizontal jerk nystagmus of medium frequency
  • Rapid phase beats in the direction of gaze
  • Fatigues

Optokinetic Nystagmus

  • Jerk nystagmus, occurs when looking out of a train window

Physiological vestibular nystagmus

  • Can be induced by caloric stimulation
  • Jerk nystagmus
  • Caused by altered input from vestibular nuclei to horizontal gaze centres

Voluntary Nystagmus

Pathologic Nystagmus

Infantile Idiopathic Nystagmus (IIN)

  • Sensory nystagmus = IIN with pathology
    • Evidence of delayed visual development?
    • Eye pathologies: Corneal opacities, cataract, aniridia, coloboma, microphthalmia, albinism
    • MRI might be necessary in some cases, e.g. in optic hypoplasia
  • Genetically determined, occurs in the 2nd-3rd month of life and persists throughout life, might weaken somewhat with increasing age
  • Pendular nystagmus in primary position
    • Transition to jerk nystagmus in lateral gaze
    • it remains horizontal when looking up and down
  • Null point present, might have abnormal head posture
  • No oscillopsia, usually slightly reduced visual acuity, improvement possible through fusional convergence -> consider base-out prism correction, Surgical correction (Kestenbaum procedure)
  • Work-up:
    • Organic cause for visual acuity reduction?
    • Optic neuropathy: Optic hypoplasia? consider imaging, endocrinological work-up
    • Foveal hypoplasia: often in albinism, aniridia
    • Retinal dystrophy

Spasmus nutans

  • Idiopathic, but similar picture possible in gliomas of the anterior visual pathway/chiasma.
    • Imaging necessary
    • might be caused by retinal dystrophies, neurodegenerative diseases
  • Occurrence typically during the first year of life, spontaneous regression usually at the age of 3 years (if idiopathic)
  • Usually unilateral or asymmetric bilateral horizontal nystagmus with a small amplitude and a high frequency
  • Visual acuity usually good
  • Common triad: nystagmus, head nodding and torticollis
  • Amblyopia and strabismus possible

Fusion Maldevelopment Nystagmus Syndrome / Nystagmus latens

  • Associated with infantile esotropia and dissociated vertical deviation (DVD)
  • Only occurs with one eye covered (nystagmus in the uncovered eye)
    • Eyes stable with fusion
  • Horizontal nystagmus beats in the direction of the fixating uncovered eye (direction changes when other eye is occluded)
  • “Manifest” latent nystagmus when both eyes are open but only 1 eye is used for vision (amblyopia or e.g. when covered during slit lamp examination)
  • Visual acuity testing: use opaque occlusion or occlusion with a +5.00 diopter lens
  • No imaging necessary

Peripheral Vestibular Nystagmus

  • Acquired nystagmus
  • Horizontal, torsional jerk nystagmus in primary position and in the different directions of gaze
  • Fast phase toward the healthy vestibular system, slow-phase toward the side of the problematic vetibular system
  • Often accompanied by dizziness, nausea and vomiting
  • Fixation may dampen nystagmus
  • Aetiology: Diseases of the vestibular system (labyrintitis, Meniere’s disease, vascular, traumatic, toxic)
    • associated with tinnitus, vertigo and hearing loss
  • Imaging necessary

Periodic Alternating Nystagmus (PAN)

  • Conjugate, strictly horizontal jerk nystagmus in primary position, changes direction periodically (~90 sec.), crescendo-decrescendo pattern
  • Between episodes there is a quiet period of about 4-20 seconds during which the eyes show slow movements, often pendulum movements
  • Observe each strictly horizontal nystagmus in primary position for at least 2 minutes!
  • Causes: congenital, cerebellar diseases, diseases at the cranio-cervical junction (e.g. Chiari malformation), ataxia teleangiectatica (Louis-Bar syndrome) and drugs such as phenytoin
  • Imaging necessary

Convergence Retraction Nystagmus (Parinaud Syndrome)

  • Due to contraction of the medial recti muscle
  • Causes: Lesions of the pretectal area = dorsal midbrain, such as pinealoma and vascular diseases (Parinaud syndrome, arterio-venous malformations in the brainstem, Multiple Sclerosis).
  • Imaging necessary

Downbeat Nystagmus

  • Most frequent form of central vestibular nystagmus
    • Vestibulocerebellum affected
  • Causes: Lesions of the cervicomedullary junction such as Arnold-Chiari type 1 malformation and syringobulbia, drugs (lithium, phenytoin, carbamazepine, barbiturates), Wernicke’s encephalopathy, demyelination, hydrocephalus
  • Imaging necessary

Upbeat Nystagmus

  • Lesions in brain stem (medulla), vermis lesions
  • Demyelination, cerebellar degeneration, smoking, drugs, Wernicke’s encephalopathy
  • Imaging necessary

See-Saw Nystagmus

  • Pendular nystagmus in which one eye elevates and intorts while the other eye depresses and extorts and then vice versa
  • Causes: parasellar/suprasellar tumours (e.g. craniopharyngeoma), syringobulbia, brainstem insults
  • Imaging necessary

Dissociated Nystagmus

  • Difference of nystagmus between the two eyes (different direction, amplitude, frequency), always pathological!
  • Aetiology: pathology of the posterior fossa, multiple sclerosis
  • Imaging necessary

Gaze-evoked Nystagmus / Rebound Nystagmus

  • Nystagmus occurs when looking laterally (<35°), non-fatigable (unlike end-gaze nystagmus, which occurs from >35-45° and is fatigable)
  • Changes direction depending on the direction of gaze
  • Absent in primary position
  • Rebound nystagmus: after looking laterally for a long time, the nystagmus weakens; when returning to the primary position, there is a jerk nystagmus in the opposite direction
  • Causes:
    • Toxic from drugs or medication
      • Alcohol, anticonvulsants, sedatives, antidepressants
    • Cerebellum or brainstem with stroke, demyelination or tumour
  • Imaging necessary

Bruns Nystagmus

  • Combination of peripheral and central vestibular nystagmus
  • Gaze toward the lesion: Coarse, large amplitude, low frequency
  • Gaze away from the lesion: Fine, low amplitude, high frequency
  • Causes: Cerebellar pontine angle tumors (e.g. acoustic neuromas or meningiomas)
  • Imaging necessary

Saccadic Intrusions/Oscillations

Square Wave Jerks (SWJ)

  • Small, conjugated, saccades away from the target object and back again at normal interssacadic intervals of ~200ms.
  • Cause: e.g. cerebellar diseases, PSP
    • CAVE: occurs also in healthy individuals in darkness, with closed eyes and with increasing age

Macro Square Wave Jerks

  • Large, conjugate, bilateral saccades away from the target object and back again at intervals of 70 – 150ms
  • Causes: e.g. Multiple Sclerosis, Charcot-Marie-Tooth syndrome

Ocular Flutter

  • Very high frequency (10-15Hz), small amplitude, horizontal saccades without intersaccadic interval
  • Causes: see Opsoclonus


  • Rapid chaotic saccades in all directions, no intersaccadic interval
  • Persists during sleep
  • Causes: Neuroblastoma, post-infectious in adults, viral encephalitis, myoclonic encephalopathy in infants, transient (idiopathic) in healthy neonates, paraneoplastic syndrome, drug-induced (lithium, amitriptyline, phenytoin)
  • Work-up: Imaging necessary, screen for tumours and paraneoplastic syndromes
    • Children: Neuroblastoma
    • Adults: small cell lung cancer, breast cancer, ovarian cancer

Ocular bobbing

  • In the unconscious patient, rapid conjugate downward movements of the eyes with a slow movement back to the primary position
  • Causes: pontine lesions, metabolic or toxic

Oculogyric Crisis

  • Dystonic conjugative gaze deviation with involuntary upward or lateral deviation of both eyes in conscious patients, lasting from 10min. to 24h, associated with dystonia
  • Causes: (Neuroleptic) drugs, neurometabolic & neurodegenerative disorders, brain lesions
  • Treatment: anticholinergic or dopaminergic medications


  • shaky vision, patients perceive that their environment is moving
  • Rare in infantile nystagmus
  • Acquired nystagmus
  • Myokymia of the superior oblique muscle


  • EyeWiki Nystagmus
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)
  • Jacobson DMCorbett JJ Nystagmus. Semin Ophthalmol. 1987;2183- 208
  • Käsmann-Kellner, Barbara. (2017). Nystagmus: Klinische Charakteristika, therapeutische Optionen. Spektrum der Augenheilkunde. 10.1007/s00717-017-0333-1.