Extraocular Movements -

Saccades and disorders

Dr.T.Abhilash
6-11-2018
• Anatomy

• Clinical examination

• Disorders and localisation

Eye movements
• To Maintain Gaze :
VORs,Fixation,OKN

• Change of gaze :
VOR cancellation,Saccades,Smooth Pursuit,Vergence
• Supranuclear pathways affect both eyes simultaneously

• Infranuclear pathways affect eyes indifferently.

 FEF,SEF,PEF,BG,SC,Cer
• ebellum,Thalamus

Brainstem and oculomotor nuclei

Oculomotor nerves and extraocular muscles

• The saccadic system moves the eyes rapidly (up to 800 degrees per
second) to fixate new targets .

• Saccades may be generated voluntarily or in response to verbal

commands in the absence of a visible target.
• Intentional saccades (internally triggered, with a goal):
Visually guided saccades , Memory-guided saccades (with
visual/vestibular input) , Predictive saccades, Target-searching
saccades, Antisaccades

• Reflexive saccades (externally triggered):

Visually guided saccades, Auditory saccades

• Spontaneous saccades (internally triggered, without a goal):

During another motor activity, At rest, When sleeping

• Quick phases of nystagmus:

Premotor burst neurons (PBN)
• Also called short-lead or medium-lead burst neurons (SLBN or MLBN)

• Consist of excitatory (EBN) and inhibitory (IBN) units

• EBN output is the pulse of innervation for ipsilateral saccades

• IBN output inhibits contralateral EBN and IBN and ipsilateral cMRF
• discharge starts 10–15 ms prior
to ipsilateral saccade start

• For horizontal saccades, EBN lie

in the PPRF and IBN lie in the
MedRF

• For vertical and torsional

saccades, EBN lie in rostral
interstitial nucleus of medial
longitudinal fasciculus (riMLF)
and IBN lie in the region of INC
and riMLF
Horizontal motor system
• EBN (glutaminergic) project monosynaptically to abducens nucleus
motoneurons and internuclear neurons(which ascend in the
contralateral MLF to the medial rectus) and ipsilateral IBN.

• Neural integrator for horizontal eye movements: NPH and MVN

Vertical motor system

• EBN (glutamatergic) and IBN (GABAergic) project monosynaptically

to oculomotor (III) and trochlear (IV) nuclei

• Neural integrator for vertical and torsional eye movements:

INC,rostral vestibular nuclei.
Omnipause neurons (OPN)
• tonically active when awake but pause for saccades in all directions.

• OPN monosynaptically inhibits all PBN

• Act as a switch to change from fixation to saccade mode.

• tonic discharge stops ~ 20 +/- 10 ms before saccade onset and

restarts ~ 10 +/- 5 ms before saccade end.
• OPN neurons all lie in nucleus raphe interpositus (RIP) in the midline
pons.

• OPN receive bilateral excitatory inputs from rostral pole of SC (fixation

and small saccade zone)

• Inhibitory inputs from LLBN,Inputs from frontal and supplementary

eye fields, LLBN and cFN.
Long-lead burst neurons (LLBN)
• LLBN discharge >40 ms prior to saccade onset

• Distributed throughout the brainstem reticular formation,especially in

the cMRF and NRTP

• May provide information on the target location in retinotopic

coordinates to the PBN and cerebellum

• May also provide a trigger signal through PBN to shut down OPN
Accuracy
• Cerebellum -

• Stimulation of vermal lobule V evokes saccades that range from

upward to horizontal

• stimulation of lobules VI and VII evokes saccades that range from

horizontal to downward.

• Purkinje cells in the dorsal vermis discharge ~ 15 ms before saccades

in a preferred direction
• The caudal part of the fastigial nucleus (cFN) is responsible for
sending saccade commands to the region of the PBN in the
contralateral brainstem.

• FNN fire tonically with a low rate, and burst near the time of a
saccade.

• FNN fire for saccades in all directions, but electrical stimulation in the
cFN elicits contralateral saccades
• Central mesencephalic reticular formation (cMRF)

• burst ~ 30 ms before the saccade.

• Lesions in the cMRF result in hypermetric c/l saccades, reduced

latency, and macrosaccadic squarewave jerks.

• These results suggest that the cMRF is important for saccade

function at both the initiation and termination stages.
How to examine saccades
• Saccades can be clinically tested in a self-paced or verbally guided manner

• by asking the patient to make repeated saccades between two visual targets
without verbal commands (such as looking quickly back and forth between two
pencils placed to the right and left of central fixation)

• by asking a patient to look at the examiner’s nose and then at a target (such as
the examiner’s finger) to the left or right of central fixation only upon verbal
command.

• reflexive component of saccades can also be assessed by observation of the fast

phases of optokinetic nystagmus (OKN).
What to look for
• Saccade initiation: Do the eyes promptly generate saccades after
commands?

• Delayed initiation of saccades, also called prolonged latency

• seen in oculomotor apraxia, and in some neurodegenerative disorders

such as Huntington’s disease (HD).
• Patients with delayed saccadic initiation often employ head thrusts
or eye blinks to generate saccades, and these features may be the
sole clinical sign indicating a mild defect in saccadic initiation.
• Range of motion and conjugacy of saccades:

• Do the eyes move to the full gaze extremes up and down and right
and left, or is there limitation in the range of motion?

• Do they move together at the same rate?

• If there is limited range of motion, the next step is to see if such
limitations are present with smooth pursuit and vestibular ocular
reflexes (Doll’s eye maneuvers).

• The hallmark of a supranuclear brainstem saccadic gaze palsy with

impaired range of motion, such as that seen with progressive
supranuclear palsy (PSP), is a prominent deficit with saccade testing
that is improved with smooth pursuit testing and completely
overcome with vestibular ocular reflexes.
• Speed of saccades: Do the eyes move slowly during the trajectory
from the initial position to the target position?

• Accuracy of saccades: Do the eyes move accurately to the new target?

Are saccades hypermetric or hypometric?
• Is there correction of the saccade to target, and is this correction
accurate?
• Saccadic intrusions or oscillations: are abnormalitiesof ocular fixation,
spontaneous unwanted saccades on regular fixation of a target.

• Examples include square wave jerks,macrosaccadic oscillations and

ocular flutter/opsoclonus

• A clinical pearl to detect saccadic intrusions - have the patient look in

lateral extreme gaze and then back to center, as they are often provoked
by gaze shifts.

• Finally, it should be noted if saccadic intrusions are present during

smooth pursuit.
 Tonic Gaze deviation
I/L
•
acute C/L
C/L Frontal Downward
irritativ Upward
Hydrocephalus
Pontine Oculogyric
e frontal crises
Thalamic Hmg
lesion Met Enc

Horizont lesion
al Vertical
Slow Saccades
• Saccades of low velocity result
from pontine disease,
presumably because of burst
cell dysfunction.
Prolonged Saccadic Latency
• Disorders of saccadic initiation, resulting in prolonged latencies for
voluntary saccades.

• occur in patients with inattention,acquired immunodeficiency

syndrome (AIDS)-dementia complex,and a variety of
encephalopathies and degenerative disorders of the nervous system,
such as AD,FTDs, HD, and PD
Square-Wave Jerks
• Square-wave jerks (SWJs) are
spontaneous, small
amplitude,paired saccades with
an intersaccadic latency of 150
to 200 msec that briefly
interrupt fixation
 Ocular Flutter
• consists of horizontal conjugate back
toback saccades that occur spontaneously
in intermittent bursts . It is aggravated by
attempts at fixation.

• Ocular flutter results from loss of pause

cell inhibition of the burst neurons in the
paramedian pontine reticular formation
(PPRF)to the cerebellar neurons that
influence the pause cells.
 Opsoclonus
• Opsoclonus is a spontaneous, chaotic,
multivector saccadic eye movement disorder
in which the abnormal movements are
virtually always conjugate,

• Dysfunction of the pause cells in the pons

due to cerebellar or brainstem
Ocular Dysmetria
• Ocular dysmetria occurs with
refixation saccades that
overshoot the target and often
oscillate with an intersaccadic
latency of approximately 200
milliseconds before coming to
rest .

• It results from dysfunction of

dorsal vermis and fastigial
nuclei in the cerebellum
 Ocular Bobbing
• Ocular bobbing is a rapid
downward movement of both
eyes followed by a slow drift
back to primary position.

• The oscillation recurs between 2

and 15 times per minute and is
found in patients (usually
comatose) with severe central
pontine destruction and
horizontal gaze palsies.
• Reverse bobbing - the initial fast
phase is upward, followed by a slow
downward drift,

• Inverse bobbing (dipping) - the

initial deviation is a slow downward
movement followed by a rapid
return to primary position.

• Reverse dipping, a slow upward

movement followed by a fast
downward movement
Supranuclear (predominantly horizontal
• Congenital ocular motor apraxia or congenital saccadic palsy
• Gaucher disease (types 2 and 3)
• Ictal (transient, adversive)
• Juvenile-onset GM2 gangliosidosis (mimics juvenile SMA)
• Postictal (transient, ipsiversive)
• Paraneoplastic (prostatic adenocarcinoma)
Supranuclear (predominantly vertical):
• Adult-onset GM2 gangliosidosis
• Autosomal dominant parkinsonian-dementia
complex
• Vitamin B12 deficiency
• Cerebral amyloid angiopathy with
leukoencephalopathy
• Dentatorubral-pallidoluysian atrophy
• Diffuse Lewy body disease (ophthalmoplegia may• PSP
be global)
• Dorsal midbrain syndrome
• Familial Creutzfeldt-Jakob disease
• Gerstmann-Sträussler-Scheinker disease
• HARP syndrome (hypoprebetalipoproteinemia,
acanthocytosis, retinitis pigmentosa, pallidal degeneration)
• Hydrocephalus (untreated, decompensated shunt)
• Joseph disease
• Kernicterus
• Niemann-Pick disease type C (initially loss of downgaze,
which may become global)
• Paraneoplastic disorders
• Stiff person syndrome
• Subcortical gliosis
• Variant Creutzfeld-Jakob disease
• Wilson Disease (also slow horizontal saccades
Supranuclear (global):
• Malignant neuroleptic syndrome
• Abetalipoproteinemia
• Neurosyphilis
• AIDS encephalopathy
• Opportunistic infections
• Alzheimer Disease (pursuit)
• Paraneoplastic disorders
• Cerebral adrenoleukodystrophy
• Parkinson Disease
• Corticobasal ganglionic degeneration
• Pelizaeus-Merzbacher disease (H>V)
• Fahr disease (idiopathic striatopallidodentate
calcification) • Pick disease (impaired saccades)
• Gaucher disease • Progressive multifocal leukoencephalopathy
• Hexosaminidase A deficiency • Stiff person syndrome-late (Oskarsson et al., 2008)
• Huntington Disease • Tay-Sachs disease (infantile GM2 gangliosidosis) (V>H)
• Joubert syndrome • Wernicke encephalopathy
• Leigh disease • Whipple disease (V>H)
• X-linked dystonia-parkinsonism (Lubag disease)
Ocular Motor Apraxia
• Ocular motor apraxia is the inability to
perform voluntary saccades while
spontaneous saccades and reflex eye
movements (vestibular and OKN slow
phases) are preserved.

• Acquired ocular motor apraxia occurs in

patients with bilateral parietal damage,
or with diffuse bilateral cerebral disease
Saccades in movement disorders
 Hypokinetic movement disorders

D/D VSGP :

CBD,FTD, Kufor-Rakeb syndrome (KRS),Niemann-Pick type C (NPC),

neuronal intranuclear inclusion disease, Gaucher's disease, and Whipple's
disease
Hypokinetic movement disorders
Hyperkinetic movement disorders
Hyperkinetic movement disorders
Hyperkinetic movement disorders
Hyperkinetic movement disorders
Summary
References
• Pichet Termsarasab et al.The diagnostic value of saccades in movement
disorder patients: a practical guide and review.Journal of Clinical Movement
Disorders (2015) 2:14 DOI 10.1186/s40734-015-0025-4

• Bradley's Neurology in clinical practice 7/E.

• Ramat et al.What clinical disorders tell us about the neural control of saccadic
eye movements.Brain (2007), 130, 10–35.doi:10.1093/brain/awl309.

• Strupp et al.Central ocular motor disorders, including gaze palsy and

nystagmus.J Neurol (2014) 261 (Suppl 2):S542–S558DOI 10.1007/s00415-014-
7385-9
•

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