Mr Ali Yagan FRCOphth

Consultant Ophthalmic Surgeon

Neuro Ophthalmology and Ocular Motility
Manchester Royal Eye Hospital

Nystagmus and related

oscillations
 Overview of the session

• Definition of nystagmus
• Classification
• Symptoms
• Description
• Childhood nystagmus
• Adult nystagmus
• Investigations
• Management of nystagmus
 Definition

• An oscillation with rhythm

• Eye or eyes

• Slow eye movement breaks fixation then a refixation movement

 Classification - 1

• Physiological nystagmus

• Infantile nystagmus syndrome

• Acquired nystagmus
 Classification - 2

• Infantile or acquired
• Position of nystagmus
• Primary position nystagmus
• Gaze dependent nystagmus

• Waveform characteristics
• Jerk
• Pendular
 What can we say about nystagmus

• Always start by asking the patient

• Look at a distance target
• Eyes in the primary position
 Nystagmus description

• What would the nystagmus look like if I plotted it on a

graph
• Jerk nystagmus – slow phase / fast phase
• Pendular nystagmus – 2 slow phases
• In which plane are the eyes moving?
• Direction and plane
• Horizontal nystagmus
• Vertical nystagmus
• Torsional nystagmus
 Nystagmus description

• In which direction are the eyes moving?

• Same direction in both eyes (conjugate)
• Opposite or different directions (disjugate)
• Jerk nystagmus described by the direction of the fast phase

• Are both eyes doing the same thing?

• Only in one eye (INO, SO myokymia)
• Bilateral
• different intensity between eyes (Dissociated)
• different waveform between eyes
Disjugate nystagmus
 Nystagmus description

• How easy is it to see the nystagmus

• Amplitude of the movement
• Measured in degrees
• Graded as small, medium and large
• Frequency of the movement
• Beats within time frame (second)
• Graded as low, medium, high
• INTENSITY = Amplitude x Frequency
• Graded as low, medium, high
 Nystagmus description

• Observe the nystagmus before getting them to move their eyes?

• Variability with time
• Change of direction (PAN)

• What can I expect when I get them to look in different directions?

• Alexander’s law
• Intensity of the nystagmus increase with gaze in the direction of the fast
phase
• Pendular nystagmus can change to jerk nystagmus
• Jerk nystagmus can change its characteristics
• Near fixation
• The intensity of the nystagmus may reduce
 Ocular Movements

• Diagrammatic recording
• 9 positions
• Waveform/ direction/ amplitude

R L

12
 Nystagmus symptoms
• Oscillopsia
• Non-specific symptoms
• Blurred vision
• Jumbled vision
• Imbalance
• No symptoms
• Present for as long as he can remember
• Typical of infantile nystagmus
• Incidental finding by an optician or family
 Physiological nystagmus
• End point nystagmus
• Fine, jerk, moderate frequency
• Extreme gaze
• Opto Kinetic Nystagmus (OKN):
• Following the strips of the OKN drum
• Beating towards to the side where the strips come from
• Caloric nystagmus (COWS):
• Cold water – opposite direction nystagmus
• Warm water – same direction nystagmus
• Post rotation nystagmus:
• Few beats after stopping head or body rotation
End point nystagmus
 Childhood nystagmus

• Infantile forms
• Infantile idiopathic (congenital motor)
• Sensory deprivation
• Manifest / latent (fusional maldevelopmental) nystagmus
• Spasmus Nutans

• Acquired forms
Infantile nystagmus syndrome
• Infantile idiopathic (congenital motor) nystagmus
• Horizontal jerk / pendular
• Null zone
• Reduced vision
• Inherited in ~ 50%
• Sensory deprivation nystagmus
• Pendular
• Roving eye movements
• Manifest / latent (fusional maldevelopmental) nystagmus
• Horizontal jerk
• Fast phase towards fixing eye
• Worse on occlusion (latent component)
• Spasmus Nutans
• Unilateral or bilateral, horizontal (could be multi planar) nystagmus
• Head nodding
• Torticollis
Roving eyes
 History

• Infantile idiopathic (congenital motor) nystagmus

• Time of onset
• < 3 months
• What parents observe?
• Any change in the child’s behaviour?
• Distress?
• Lighting condition?
• Any AHP?
 History

 What can the child see?

 Any concern over vision?
 Schooling?
 Pregnancy history
 Alcohol
 recreational drugs
 complications
 Family history
 Albinism
 Retinal dystrophy
 Nystagmus
 Infantile nystagmus

• Features
• Onset usually within first 3 months, later onset possible with
acquired visual disease
• Jerk/pendular waveform
• Horizontal in all gaze positions
• Symmetrical
• Bilateral
Congenital nystagmus with AHP
Congenital nystagmus with Alexander law
 Investigation
• Visual acuity
• Orthoptic assessment
• Refraction
• Slit lamp examination
• Fundus examination
• Electrodiagnostic tests
• Neuro imaging
Disorder of the anterior visual system
• Cornea enlarged (congenital glaucoma)
• Clarity of the media
• Corneal opacities
• Cataracts
• Vitreous opacities
• Iris transillumination defects
• Albinism
• Aniridia
• Pupil reaction
• Paradoxical response
• retinal dystrophy
• Sluggish pupil reactions
• Leber’s congenital amaurosis
• Optic nerve hypoplasia
Disorder of the anterior visual system

• Retina
• retinopathy of prematurity
• Colobomas
• The presence of the fovea
• hypoplastic in albinism
• The optic nerves
• optic nerve hypoplasia
 Differential diagnosis in the absence of an
obvious media or retinal abnormality

• Most cases of infantile nystagmus are the result of a bilateral

sensory abnormality affecting the anterior visual pathway
• ocular albinism
• oculocutaneous albinism
• optic nerve hypoplasia
• Leber’s congenital amaurosis
• rod monochromatism
• congenital stationary night blindness
• idiopathic infantile nystagmus
Electrodiagnostic assessment
• ERG
• Absent response
• Leber’s congenital amaurosis
• Absent B wave
• X-linked retinoschisis
• CSNB
• Absent Cone response
• Rod monochromatism
• VEP
• Crossed asymmetry
• Albinism
• Diminished response
• Optic nerve hypoplasia
Latent and manifest latent nystagmus
(Fusion maldevelopment nystagmus)

• Latent nystagmus
• horizontal jerk nystagmus
• absent when both eyes are open
• present when the light stimulus to one eye is
reduced
• Bilateral
• Symmetrical
• Conjugate
 LN / MLN

• The fast phase is always directed towards the fixating eye

• The slow phase of the nystagmus shows a decelerating velocity waveform
• Reducing light stimulus to one eye results in latent nystagmus becoming manifest
• Intensity
• increases on covering either eye
• increases on abduction and decreases on adduction
• A face turn to the side of the fixing eye is commonly seen
• Binocular single vision
• absent or poor in most patients
• Commonly associated
• infantile esotropia
• DVD
Categories Binocular viewing Monocular viewing

Type 1 MLN Stable alignment Conjugate jerk nystagmus

Type 2 MLN Square Wave Jerk Conjugate jerk nystagmus

Type 3 MLN Torsional nystagmus Conjugate jerk nystagmus

Type 4 MLN Conjugate jerk nystagmus Conjugate jerk nystagmus

ML nystagmus
Acquired forms of childhood
nystagmus

• spasmus nutans
• nystagmus with intracranial disease
• nystagmus with ipsilateral visual loss
 Spasmus nutans

• asymmetrical nystagmus
• involuntary head movements
• abnormal head posture

(the triad of spasmus nutans)

 Spasmus nutans

 Onset
○ 3 and 18 months of age.
 Nystagmus
 Pendular waveform
 Horizontal, vertical or torsional
 Asymmetrical- appears unilateral
 Low amplitude - fast frequency
 Varies considerably in different positions of gaze
 If head nodding is present, the intensity increases if the head is
immobilized
 Spasmus nutans

 Head movement
 precedes the onset of the nystagmus
 involuntary nodding or shaking movement
 opposite phase to the eye movement
 Do not appear to compensate for the nystagmus as they are of
different frequency
 Disappear during sleep

Spasmus nutans may last from a few weeks to several years

Spasmus Nutans
Acquired nystagmus
 Peripheral vestibular nystagmus
• Features
• Symptoms of vestibular dysfunction
• Jerk nystagmus
• Horizontal / rotary
• Shimmering
• Small amplitude, fast frequency
• Unidirectional
• The nystagmus beats in the same directions irrespective of the direction of gaze
• Alexander’s law
• Fast phase away from side of lesion
• Often transient
• Enhanced by removing fixation / closing eyes
• Causes:
• Infections
• Toxic reactions
• Trauma
• Inflammation
 Jerk nystagmus

Primary positional

Conjugate

Brun’s
Vestibular
nystagmus

Peripheral Central

Down-beat Up-beat PAN

Vestibular nystagmus
 Central vestibular nystagmus
• Unidirectional waveforms
• Vertical
• horizontal or rotary
• Mixed waveforms
• Indistinguishable from those seen with peripheral vestibular lesions
• Amplitude
• variable and at times large
• intensity obeys Alexander’s law
• Removing fixation
• Does not affect the intensity of the nystagmus
• Gaze-evoked nystagmus may be present
• Oscillopsia is common
• Tinnitus and deafness are rare
 Jerk nystagmus

Primary positional

Conjugate

Brun’s
Vestibular
nystagmus

Peripheral Central

Down-beat Up-beat PAN

 Lesions

• Acute
• Infarction
• Inflammation
• Trauma
• Chronic
• Structural abnormalities
• Slow growing tumours
• Degenerative conditions
Down beat with rotary element
Down beat nystagmus
 Jerk
nystagmus

Primary Gaze
positional dependent

Conjugate Disjugate Gaze-evoked Gaze-paretic Dissociated

Other: Brun’s Convergence

Vestibular See-saw
nystagmus retraction

Peripheral Central

Down-beat Up-beat PAN

See saw nystagmus
See saw nystagmus
Convergence retraction
 Gaze dependent nystagmus

• This is a jerk nystagmus that only becomes manifest

when gaze is directed away from the primary position
• Direction
• Unilateral
• Bilateral
• Horizontal
• Vertical
 Jerk nystagmus

Primary positional Gaze dependent

Conjugate Disjugate Gaze-evoked Gaze-paretic Dissociated

Brun’s Convergence
Vestibular See-saw
nystagmus retraction

Peripheral Central

Down-beat Up-beat PAN

Gaze evoked nystagmus
 Adult nystagmus

Jerk nystagmus Pendular nystagmus

Horizontal Vertical

Multiple
Symmetrical Asymmetrical
sclerosis

Ocular Monocular
myoclonus Visual loss
Oculopalatal myoclonus
 Nystagmus Plus

• Multiple sclerosis • Vestibular symptoms

• Transient neurological • Vertigo
symptoms
• Transient visual loss • Dizziness
•
• Cerebellar syndrome •
Nausea
Deafness
•
•
Ataxia
Co-ordination difficulties
• Lower brainstem lesion
•
• Medications •
Dizziness
Dysarthria
• Epilepsy • Dysphagia
• Lithium • Diplopia
• Alcohol • Facial palsy
Nystagmus with facial palsy
 Differential Diagnosis
• Saccadic intrusions
• Involuntary saccades interfering with visual fixation
• Square wave jerks
• Small saccades move the eyes away from fixation and back with an intersaccadic interval
• Can be seen in cerebellar disease, Parkinson’s and PSP
• Saccadic oscillations
• Repeated saccadic intrusions
• Ocular flutter/ opsoclonus
• To and fro saccades. Horizontal: flutter, horizontal and vertical: opsoclonus.
• Roving eye movements
• Conjugate, large amplitude pendular like movement
• Superior oblique myokymia
• Monocular rapid intermittent torsional vertical movements
• Bobbing/ dipping
• Fast downward jerks of both eyes, followed by a slow drift to the midline (Comatose,
Pons)
Saccadic intrusions
Voluntary
Investigations

61
 Aims

• Diagnosis

• Differential Diagnosis

• Aetiology

• Effect on visual system

• Site of lesion

• Management plan

62
 Investigations

Orthoptic Additional
• Case History • Refraction
• Visual Acuity • Fundus and media
• Observation • EOG
• Abnormal head position • ERG
• Cover test • VEP
• Ocular motility • Neuro imaging
• Convergence
• OKN drum
• Binocular vision
• Squint measurements

63
 Nystagmus
Management
 Overview

• Treatment aims
• Improve visual function
• Correct an abnormal head posture
• Reduce nystagmus intensity
 Overview
• Treatment options
• Treat the cause / association
• Patient adjustment / compromise
• Optical
• Pharmacological – oral medications
• Pharmacological – Botulinum toxin
• Eye muscle surgery
• Anderson - Kestenbaum
• Artificial divergence
• Equatorial horizontal rectus muscle recession
• Tenotomy / reattachment
 Nystagmus Management
• Congenital nystagmus
• Improve visual acuity
• Refractive correction
• Amblyopia management
• Reduction in nystagmus intensity
• Drugs- Gabapentin, memantine
• Base-out prisms
• Rectus muscle tenectomies
• Abnormal head posture
• Deviating prisms
• Base-out prisms
• Surgery
• Artificial divergence
• Kestenbaum procedure
Nystagmus Management
• Adult nystagmus
• Improve visual acuity
• Head posture
• Prisms
• Surgery
• Oscillopsia
• Medical treatment
• Botulinum toxin
• Surgery
• Relieve diplopia
• Prisms
• Botulinum toxin
• surgery
 Treat the cause
• Childhood nystagmus
• Refractive errors
• Amblyopia
• INS
• Visual pathways disorders
• MLN
• Visual pathway disorders
• Strabismus

• Adult nystagmus
• Inflammatory brain disease
• Infective brain disease
• Structural brain abnormalities
• Brain tumours
• Medication side effects
 Congenital cataract and INS

• Investigation – TORCH screen

• Treat the cause
• Congenital cataracts: Early surgery
• Manage the aphakia
• Glasses
• CL
• IOL
 Treat the cause

• MLN / LN
• Strong association with early onset strabismus
• Damps with adduction of fixing eye and associated
with compensatory head posture
• Down beat nystagmus
• Acquired jerk nystagmus
• Structural abnormalities:
• Arnold Chiari malformation
• Syringomyelia / bulbia
• Drug reaction: Lithium
 Treat the cause

• Multiple sclerosis
• Typically, horizontal or vertical pendular
• Dissociated horizontal jerk with INO
• Other types common
• Treatment: steroids
• Spontaneous resolution of the episode
• Brain tumour – see saw
 Compromise

• At school
• Sit at the front of the class
• AHP with face turn to the right sit at right side of room
• Allow reading material to brought as close as necessary
 Optical treatment
• Refractive correction
• Glasses
• Contact lenses
• Viewing through optical centre as CL moves with eye
• Not affected by AHP
• Proprioceptive feedback from the feel of the lens

• Tints
• QoL in albinism reduced from reduced distance VA & Glare
• Prisms
• Artificial divergence
• Deviating
• Low vision aids
 Prisms
• Artificial divergence • Deviating prisms
• INS which damps on convergence • Assess the response to moving
and demonstrates BSV (Motor the null region before surgery
fusion)
• 10% patients with INS
• Base of prism orientated
towards AHP
• Prescribe BO prisms
• Most AHP’s requiring surgery
• Benefits >=20’ so need 40 PD prisms
• Improved visual function at distance • Frensel – visual blur
• Reduction in an AHP • Incorporated prisms - heavy
• Reduction in nystagmus intensity
 Medications
• INS
• Gabapentin
• Memantine
• Down beat nystagmus
• 3,4-diaminopyridine (3,4-DAP)
• Upbeat nystagmus
• Most cases of upbeat nystagmus show spontaneous resolution
• Drugs
• 3,4-diaminopyridine (3,4-DAP
• Memantine
• Baclofen?
• Gabapentin?
• Acquired pendular nystagmus in MS
• Memantine
• Gabapentin
• Oculopalatal myoclonus
• Gabapentin
• Memantine
• Periodic alternating nystagmus
• Baclofen
Botulinum toxin retrobulbar space

• In favour
Am J Ophthalmol. 1988 Nov 5;106(5):584-6.
Treatment of acquired nystagmus with botulinum A toxin.
Helveston EM1, Pogrebniak AE.

• Against
Am J Ophthalmol. 1995 Apr;119(4):489-96.
Unsatisfactory treatment of acquired nystagmus with retrobulbar injection of botulinum toxin.
Tomsak RL1, Remler BF, Averbuch-Heller L, Chandran M, Leigh RJ.
 Botulinum toxin
Retrobulbar space

Pre - BT Post- BT
 Surgery

• Aims
• Improve AHP
• Improve visual function
• Improve appearance (decrease intensity)
• Improve strabismus
 Surgery - Improve AHP
• Check list
• AHP secondary to nystagmus?
• Head turn
• Sixth n palsy
• Duane’s syndrome
• Hemianopia
• Head up / down
• Grave’s orbitopathy
• Brown’s syndrome
• Supranuclear palsy
• Alphabet pattern
• Ptosis
• Head tilt
• Superior oblique palsy
• DVD
• Skew deviation
• Non-ocular causes
 Surgery - Improve AHP
• Check list
• Refractive correction
• Amblyopia management
• Stable and consistent AHP
• Different AHP for near & distance
• Alternating AHP – PAN
• Components of AHP
• Head turn
• Head up / down
• Head tilt
• AHP causing concern
• Affecting visual function
• Activities
• Appearance (AHP >= 20 degrees)
 Surgery - Improve AHP
• Anderson and Kestenbaum independently suggested that an abnormal
head posture related to nystagmus could be alleviated by surgery

• Anderson's proposal
• Recession of the pair of rectus muscles whose action was in the opposite direction
of the face-turn

• Goto suggested resection of the antagonist muscles

• Kestenbaum favoured surgery on all four muscles, although he also suggested the
two eyes should have sequential surgery
 Surgery
AHP L head turn 20 degrees
Left head turn

R L
Move eyes in direction of head turn

LR – MR MR- LR +
7mm + 5mm 8mm
6mm
 Surgery
AHP Head down
Head down

R L

SR - IO - IO - SR -

IR+ SO+ SO+ IR+

Option: Bilateral SR-
Bilateral SR- & IR+
Bilateral SR- & IO –
 Surgery
AHP Head up
Head up

R L
SR+ IO+ IO+ SR+

IR- SO- SO- IR-

Options: Bilateral IR-
Bilateral IR- & SR+
Bilateral IR- & SO-
 Surgery
AHP Head tilt

Excyclorotate Incyclorotate

R L
 Surgery
AHP Head tilt
Excyclorotate Incyclorotate

R L

IO+ IO-
Option 1

SO- SO+
 Surgery
AHP Head tilt
Excyclorotate Incyclorotate

R L

Option 2
Horizontal
transposition
of vertical
recti
 Surgery
AHP Head tilt
Excyclorotate Incyclorotate

R L

Option 3
Vertical
transposition
of horizontal
recti
 Surgery
Equatorial rectus muscle recession

• All four horizontal recti are recessed to around the

equator of the globe
• Medial recti 10 mm
• Lateral recti 12 mm
• Minimal reduction in horizontal rotations of the eye
• Visual acuity is only slightly improved
• Most patients report a decrease in the time taken to
identify an object of regard
 Surgery
Muscle tenotomy / reattachment

• Disconnection of the tendon organ responsible for

proprioception abolishes the nystagmus

• Anterior tenotomy followed by reattachment of all rectus

reduced nystagmus
 Surgery
Artificial divergence
• Aim
• induce a latent divergent ocular position (exophoria)
• patient will then overcome by exerting fusional convergence, thereby
damping the nystagmus
• Patients
• must have adequate fusional reserves to overcome the induced
deviation
• Amount of surgery based on the strength of prism
• Procedures
• Bilateral medial rectus recession
• Unilateral MR recession & LR resection
 Latent / Manifest latent nystagmus

• Most patients do not report any significant visual symptoms

• Small group
• AHP with head turn towards fixing eye
• AHP moves fixing eye into adduction which damps the nystagmus
• Surgery directed at fixing eye which improves AHP & esotropia
• Option to add a posterior fixation suture to medial rectus recession

• Patients without AHP can show improved visual function after

rectus muscle tenotomy / reattachment
 Nystagmus surgery combined with
strabismus surgery
Left head turn Right eye fixing

-7 +6 -5 +8
Nystagmus
surgery
R L
Left exotropia

+5 -8
Exotropia surgery

-7 +6 Left eye tonotomy

Combined
/ reattachment
surgery
 Manifest latent nystagmus
Esotropia with face turn
Left head turn Left eye fixing

-7 +6 -5 +
Nystagmus
8
surgery
R L
Right esotropia
Head in primary
Esotropia surgery + -6 position
(Non- fixing eye) 7

Right eye -5 +8
Combined tonotomy /
surgery reattachment
 Take home message
1. 7 things to say about nystagmus
2. Making sense of what you see
3. Infantile nystagmus
Take a history
Examination
Investigation
4. Other forms of childhood nystagmus
5. Adult nystagmus
6. Investigations
7. Treatment
Thank you!

Questions?