Nystagmus

Danah Albreiki
Jan-2016

Eye Movement Disorders

What are they?
1. Ocular misalignment :
– Infra-nuclear : 3rd, 4th, 6th nerve palsies/ NMJ/ Muscle
– Nuclear: 3rd, and 4th nuclear palsy
– Inter-nuclear : INO
– Supra-nuclear : Skew

2. Conjugate gaze abnormalities :

– Nuclear : 6th nerve nucleus
– supra-nuclear
3. Nystagmus and nystagmoid eye movements

The six eye movement systems

• Hold images steady on the retina:
Fixation : holds image of a stationary object on the fovea
Vestibular ( VOR ): Holds images steady on the retina during brief head movements
Optokinetic : Holds images on the retinal during sustained head movement

• Direct the fovea on the object of interest:

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Saccades : bring images of an object of interest rapidly on to the fovea
Smooth pursuit: holds images of a small moving target on the fovea
Vergence: moves the eyes in opposite direction ( convergence or divergence) so that
the images of a single object are held simultaneously on both foveas

Nystagmus
Introduction

• Definition:
– involuntary eye oscillations that are initiated by SLOW eye movements which drive the
eye off target
• Arises from lesions in:
– VOR
– Gaze holding system
 – Fixation system
• Characteristics:
– Jerk vs pendular
– Trajectory: H,V, T, combined
– Conjugacy
– Amplitude
– Frequency
– Slow wave form : ↑,↓, or same

Nystagmus
Introduction
• Oscillopsia
• Nystagmus types

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Nystagmus
 Oscillopsia
• Present with head motion only: • Present with or without head motion :
– Vestibulopathy due to abnormal – Nystagmus:

VOR gain: • Jerk:

• BL peripheral vestibulopathy: – Vestibular:
– Ototoxicity – Eccentric gaze

(aminoglycoside) • Acquired pendular

– Cranial nerve 8 resection – Saccadic dyskinesia
– Tumor • Intrusions: SWJ
– Meningitis • Oscillations: macro saccadic, flutter
– Congenital ear anomalies
– Hereditary vestibular
areflexia
– Dolichoectasia
– Idiopathic
• Central vestibular dysfunction

Nystagmus

Congenital Acquired

Infantile
nystagmus Childhood Any age
(congenital/
infantile
idiopathic motor
Monocular
nystagmus) Malfusion/ latent Spasmus
nystagmus of Jerk Pendular
nystagmus nutans
childhood
Others:
See saw
PAN Vestibular Eccentric -MS
Imbalance of Leaky neural -V loss
VOR integrators -OPT
(defective gaze -OMM
holding -SCD
-GEN
mechanisms)
Peripheral Central
-Rebound
Bruns nystagmus =
central vestibular + GEN
 central vestibular + GEN -DBN
-UBN
Look for CPA tumor -TN
-PAN
-See Saw

CONGENITAL
Infantile nystagmus ( congenital/ infantile idiopathic motor nystagmus)

1. Age: First few months of life & is life long

2. VA : good or poor
3. Oscillopsia: NONE
4. Sleep: Absent
5. Trajectory: Uniplanar, horizontal irrespective of gaze position
6. Conjugate
7. Slow phase velocity: Exponential increase
8. Null point (may have resultant head turn)
9. Fixation: Amplified by attempted fixation (distant)
10. Convergence: Dampened by convergence and darkness
11. OKN: Reversal
12. Associations: esotropia
13. Isolated (motor): but may be associated with albinism, LCA, achromatopsia, or
optic atrophy (sensory)

ACQUIRED
 Acquired – CHILDHOOD

Fusional maldevelopment Spasmus nutans Monocular nystagmus

Here: talked about spasmus nutans (with head bobbing and can be
nystagmus syndrome monocular or binocular; horizontal/ vertical of
andchildhood
torsional; worse in the
( Latent nystagmus) abducting eye)

• Appears within first few months of life 1. Then also:

Asymmetric or monocular low-• Usually monocular,
• Horizontal jerk nystagmus appearing Monocular nystagmus of childhood: is manifest; chiasmal glioma; often only
amplitude oscillation/ May be
vertical vertical, low amplitude
only under monocular viewing horizontal, vertical or torsional oscillation
conditions
• Fast phase beats away from occluded 2. Head nodding • Eye with nystagmus may
eye 3. Torticollis or abnormal head have afferent visual
• Strong association with esotropia posture dysfunction
• Usually poor stereopsis • Requires neuroimaging
• May explain subnormal visual acuity (chiasmal glioma)
tested monocularly
• Manifest latent nystagmus:
• Present even when both eyes are • Begins in infancy, usually resolved
open by age 3 to 5
• Requires neuroimaging

Acquired – ANY AGE

 JERK

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vestibular nystagmus
Peripheral Central
1.Caused by disease in .. UL vestibular organ or nerve BS & connections with
vestibulocerebellum (F, PF, N)
2. Waveform Mixed 1. Caused
PurelybyV,UL disease
H, or T ( or mixed)
1. Caused by UL disease of
jerk Jerk
vestibular organ or nerve
3. Fixation 2. suppressed
waveform Not suppressed

4. Adaptation Subsides in days persists

5. Associated vestibular Sx Vertigo, hearing loss, tinnitus none

6. Associated neurologic Sx none BS and cerebellar signs

7. Examples BPPV Vascular

Menier’s Demyelinating
Viral labrynthitis Pharmacologic/ toxic
 Central vestibular nystagmus
Downbeat Upbeat Torsional Seesaw & Hemi- PAN
Upbeat Torsional
seesaw PAN
CCC • Alexander’s law Cerebellar
Alexander’s law Pontomedullry • pendular- seesaw •Ethology:
R 90 sec/ stops 5-20 sec
• Intensity increases on damage junction • jerk- hemi (null point) then L
Cerebellum Sea saw or hemi Congenital/ albinism
down and lateral gaze MS beating 90 sec
Tumor sea saw • Alexander’s law
Thats why base out
prisms are helpful Degeneration • +- periodic head trun
Can be pendular Localization:
towards quick phase
and jerk PAN for nodulus
Causes 1- Arnold-Chiari Not localized
1- cerebllar degen 1-Infarction 1- para-sellar tumor (pit 1- congenital (albinism)
Of cerebellum
2-Cerbellar degeneration well
2-BS & cereballar 2-MS adenoma)
Parasellar lesion MS2-A-C malf
3-VB infarction infarcts 3-V angioma 2-infarct 3-cerebllar degen
Diencephalon Drugs
4-MS Disruption
3-MS of 4-A-C malf 3-trauma 4-MS
Parachiasmal Ethanol
5-Drugs(phenytoin, anterior
4- Tumorscanal 5-tumor 4-congenital 5- infections
area Paraneoplastic
Carbamezapine, lithium) 5-other (infec, inflam, 6-encephalitis 5-v loss 6-tumors
6- Idiopathic toxic,
No trauma)
treatment 7-trauma 7-V loss
8-idiopathic
Velocity storage
mechanism
Location -Cervicomedullary -Not well localized • P-M junc • pendular- Nodulus:
• cerebllum diancephalon/midbrain • MS
Treat: baclofen
• midbrain lesion + Bitemporal • Drugs
hemianopsia • Ethanol
• jerk- UL midbrain or • Paraneoplastic
lateral medullary

Pathogenesis Disruption of posterior disruption of anterior Disruption of anterior and • abnormal central • Increased gain of the
canal projections to BS canal projections to the posterior canal projections otolith projection to the central velocity storage
leading to upward bias BS leading to downward on the same side INC mechanism of the VOR
bias • optic chiasm lesions • lack of V input to the
PC lead to damage of VOR
subcortical pathway to
the inf oliv nuc and
flocculs

Treatment • Treat underlying - ?baclofen ? ? Baclofen 10 mg TID

• clonzapeam,
valproate, baclofen
• BO prisms

Eccentric gaze
GEN

1- Physiological 2-Most
GEN common form of nystagmus
Defective gaze holding/ leaking neural
3- Centripetal and rebound
Centripetal and rebound
end- point integrators (MVN/ NPH) and INC
nystagmus Seen in patients with GEN
Causes: Centripetal: 30 seconds of eccentric gaze, nystagmus
Drugs (EtOH, anticonvulsants, phenytoin, • will beating towards primary position
Three types: • Most common form of nystagmus Seen in patients with GEN
carbamazepine), sedative (lithium)
1. un-sustained • Defective
Lesions gaze holding
on cerebellum / Leaky
(flocculus) and its • Centripetal
Rebound: when the - >30 seconds
eye returns to of
primary position,
2. sustained – neural integrators ( MVN, NPH/
projections to Bs: eccentric gaze, nystagmus will
beats opposite to direction of prior gaze
* spinocerebellar degeneration
normal if in INC) ataxia type 2
Episodic reverse beating towards primary
MVN/NPH
both H • MSCauses: position
directions and 1. Cerebellar/
DrugsBS:ischemia
alcohol, anticonvulsants• Rebound, when the eyes return to
Posterior fossa tumours
 directions and 1. Drugs : alcohol, anticonvulsants• Rebound, when the eyes return to
low in ( phenytoin, carbamazepine), primary position , beats opposite the
amplitude sedative ( phenobarb), anti- direction of prior eccentric gaze-
3. Fatigue depressant ( lithium) few sec. pathology ? MVN, NPH
induced – after 2. lesions on cerebellum • Attempt by BS or cerebellum to
eccentric (flocculus) and its projections to correct the drift of GEN
fixation for 30 BS:
sec • Spinocerebellar degeneration
• Episodic ataxia type 2
• MS
• Cerebellar / BS ischemia
• Posterior fossa tumors

4- Dissociated nystagmus in INO 5- Brun’s nystagmus

Brun's nystagmus:

• Abducting eye exhibits nystagmus beating 1. Lowlow freq, large

away from primary position
• Increased innervation to the weak adducting
eye , Herring's law dictates conjugate
increase in innervation of abducting eye.
This results in overshooting saccades and
post-saccadic drift in abducting eye
frequency, amplitude
large amplitude H
H nystagmus on looking
towards lesion due to defective gaze holding
nystagmus on looking towards the
lesion, due to defective gaze holding
+
2. Jerkhigh frequency, small amplitude on
looking away from the side of the
Due to CPA angle tumor
lesion due to vestibular imbalance.

• Since saccades initiate the oscillation, CPA tumor

abducting nystagmus is not a true
nystagmus, but rather a series of saccadic
pulses

Acquired – Any Age

 PENDULAR

Acquired pendular nystagmus

Causes Features
1. V loss ( including UL dis of the ON) • One of the more common types of nystagmus
One of the most common
2. Disorders of central myelin: • Oftenhas
Often oscillopsia
oscipllopsia
• MS • H, V,
Any T and one component may predominate ( congenital
trajectory
• Palizaeus-Merzbacher Conjugate
usually Horwith
dysconjugate
small T)
• Cockayne Can be monocular
• Nystagmus
Abnormal trajectory
between : if H and
brainstem V are
nucleui andin cerebellum
phase (oblique), if
• Perixisomal assembly disorders
H and
Most V are 180
commonly out ofwith
occurs phase ( elliptical),
VL (e.g. Cataract)if H and
and V are
goes 90 when
away
• Toluene abuse
out ofokphase ( circular)
vision
3. Spasmus nutans Hermann-bielschowsky: nysgmus greater in the eye with poor vision
• Conjugate, disconjugate, monocular, disjunctive
4. Oculopalatal tremor (OPT) • Pathogenesis: abn internal feedback circuits between brain
5. Oculomasticatory myorrythmia

5. Oculomasticatory myorrythmia
(whipple’s disease)
6. Acute brain stem infarct
7. Spinocerebellar degeneration
• Pathogenesis: abn internal feedback circuits between brain
stem nuclei and cerebellum ( gaze holding network) that are
important in long-term recalibration using visual input
• Treatment: ? Gabapentin, clonazepam, memantine
• APN with V loss:
• Most commonly with ON disease, may resolve when
vision Is restored
• If UL ON disease, mainly affects abnormal eye
(monocular) prominent V pendular less H jerk
waveform
• If BL ON disease, the amplitude of nystagmus is
greatest in the eye with poorer vision ( Heimann-
Bielsch9owsky phenomenon)

OPT Oculopalatal tremor

• Vertical pendular eye movements associated with rhythmic

upward movement of palate

• Caudal brainstem pathology: red nucleus, inferior olive, and

dentate nucleus ( Guillian-Mollaret Triangle)
Large inferior olive; palate is in synchrony with the
palate

Superior Red
Dentatecerebellar nucleus
nucleusInferior
peduncle Inferior
cerebellar
“Brachium olivary
peduncle
 Nystagmus Case

Oculomasticatory myokymia:

Convergence pendular nystagmus

Synchronous masticatory movements

History
• Onset of nystagmus
• Oscillopsia:
– Trajectory
– Monocular vs binocular
– Distance or near
– Vision
– Gaze evoked
• Associated symptoms:
 – Neurologic (brain stem/cerebellum): diplopia,
dysarthria, facial numbness, dysphagia, ataxia
– Vestibular : N, V, Vertigo, hearing loss, tinnitus

Exam
1. Afferent
2. Pupils
3. IOP
4. Anterior segment
5. Posterior segment
6. Orbit
7. Cranial nerves
8. Efferent:
– EOM
– Cover and uncover
– Saccades
– Smooth pursuit
– VOR
– Nystagmoid eye movements
– Nystagmus

Exam of nystagmus
• Is it present in primary gaze? Or only eccentric gaze?
• Primary gaze In distance and near & cardinal positions of gaze
– Trajectory
– Conjugacy
– Amplitude
– Dissociation
– Fixation
– Convergence
– OKN :
• congenital
• convergence retraction nystagmus
– Head shaking nystagmus