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Gaze Palsy
Presenter- Dr Shubhangini J
Moderator-Dr Monica Samant
Ocular Motor system-
Nuclear
SupranuclearInfranuclear
Inter nuclear
Supranuclear control of ocular
motility-
Versions-
Same
direction
Vergence-
Opposite
direction
Supranuclear
control
Eye Movements-
Eye
Movements
Version
Saccades
Smooth
Pursuit
Optokinetic
Vestibulo-
ocular
Vergence
Divergence
Convergence
Saccadic System-
Cells in PPRF
Burst Cell-sends
pulse step to
move the eye
Pause Cell-
inhibits firing of
burst cell allowing
burst cell to
initiate saccade
Tonic Cell –
maintain the eye
position
Smooth Pursuit System
Vestibulocerebellar system-
 Important input of gaze system
 Modulate eye movements
 Stabilize eye against the gravitatinal & accelerational
force
 Maintaining clear vision
Cerebellum-
 Eye movements
 Fixation accuracy
 Suppress the vestibulo-ocular reflex
 Controls smoothness of pursuit movements
 Accuracy of saccades
Lesion of Supranuclear oculomotor
pathways -
 Based on anatomical location-
 Lesions of internuclear system
 Immediate premotor structure in the brain
 PPRF
 Posterior commisure
 Rostral mesencephalon
 Cerebral hemisphere
 Descending pathway from cerebral hemisphere
 Superior colliculus
 Thalamus
Clinical Examination
 Asymptomatic for gaze palsy
 Blurring of vision
 Diplopia
Pre-requisite-
 Observe position of eye in primary gaze
 Ductions
 Versions & vergence
 Pursuit
 Saccades
Oculocephalic maneuvers-
 Dolls eye reflex
 Tilt the head 30 degree forward & fixate a distant
target
 Rotate the head in direction opposite to gaze palsy
 Direct projection from vestibular system to ocular
motor nuclei
 Prenuclear,nuclear infranuclear reflex does not
overcome
 Lesion in cerebral cortex overcome by VOR
Vestibular ocular reflex -
 Tilt the head by 60 degree & irrigate external auditary
meatus with cool/warm water
 In normal subject/supranuclear gaze palsy eye deviate
towards the irrigated side- nystagmus with fast phase
to opposite side
 Fast phase towards the stimulated eye when warm
water is used
Supranuclear eye movement
disorder-
Gaze palsy
Horizontal
Vertical
Vertical gaze palsy-
 Midbrain lesion
 B/L cerebral hemisphere
dysfunction
 Parkinsons disease
 Progressive supranuclear palsy
 lipidosis
Parinaud syndrome-
 Dorsal midbrain syndrome
 Lesion of posterior commisure & MRF
 Cause- compression by mass in pineal region
 Dilatation of third ventricle
 Midbrain infarction
 multiple sclerosis
 AV malfomation
 Poor to absent upgaze
 Convergence retraction nystagmus in upgaze
 Colliers sign
 Setting sun sign
Parinaud syndrome-
 EMG shows co-contraction of occulomotor innervated
muscles- retraction of globe
 Neuroimaging scan
 Surgical treatment causes resolution of ocular
findings
Progressive supranuclear palsy-
Lesion of mesencephalic structure-
 Steele-Richardson-Olszewski syndrome
 Onset –after 40 years
 Disorder of basal ganglia
 Marked rigidity –trunk & neck
 Little tremor
 Difficulty with vertical eye movements down > up
 Progresses to horizontal gaze disorder
 End stage – global ophthalmoplegia
Progressive supranuclear palsy-
 Vertical direction more severely affected initially
 Voluntary saccades affected first, convergence, and
smooth pursuit later
 Slowing of saccade velocity
 Supranuclear movements primarily affected (vestibulo-
ocular reflex spared)
 Square wave jerks
 Gait abnormalities
 Nuchal rigidity
Progressive supranuclear palsy-
 Eyelid abnormalities:
 upper eyelid retraction
 reduced blink rate
 apraxia of eyelid opening
 blepharospasm
 Postural instability with falls (often backwards)
 Cervical and axial dystonia
Progressive supranuclear palsy-
 Wilson’s disease
 Huntington diseas
 Kernicterous
Parkinsons disease-
 Lesion of descending pathway from cerebral
hemisphere
 Upgaze palsy affecting saccades followed by pursuit
 Cogwheel pursuit
Lipidosis-
 Lipid storage disease variant of niemann picks disease
 Vertical saccades
 Intact vertical oculocephalic maneuvers
 Progressive dementia in late childhood
 Choreoathetosis
 hepatosplenomegaly
Whipples disease-
 Involvement of CNS – supranuclear gaze palsy
 Initially vertical
 Progressive dementia
 Hypersomnia
 Ataxia
 Uveitis
Monoocular elevation paresis-
 No ocular deviation in primary gaze
 Inability to elevate one eye
 Prenuclear congenital unilateral midbrain lesion
 Oculocephalic maneuver is normal
 Lesion in pretectum
 Connection of riMLF to the occulomotor nuclei
 Forced duction & tensilon test are negative
Monoocular elevation paresis-
Skew deviation-
 Skew deviation is a vertical divergence
 “prenuclear” lesion of the vertical vestibulo-ocular
pathways in the brainstem or cerebellum.
 Comitant, associated with cyclotorsion of one or both
eyes.
 Noncomitant it can mimic a partial third or fourth
cranial nerve palsy
Skew deviation-
 Occur most commonly with vascular lesions of the
pons or lateral medulla (Wallenberg's syndrome)
 lesions of the midbrain or upper pons
 Alternating skew deviation, the hypertropia changes
with the direction of gaze. The adducting eye usually
is hypotropic,mimick superior oblique overaction.
Skew deviation-
Ocular tilt reaction-
 cyclotorsion of both eyes, and paradoxical head tilt,
all to the same side – that of the lower eye
 A tonic (sustained) ocular tilt reaction occurs with
lesions of the ipsilateral utricle, vestibular nerve or
nuclei, or a lesion in the region of the contralateral
interstitial nucleus of Cajal and medial thalamus
 A phasic (paroxysmal) ocular tilt reaction occurs with
lesions of the ipsilateral interstitial nucleus of Cajal
and may respond to baclofen.

Horizontal gaze palsy-
 More common
 Vary from
 Gaze evoked nystagmus
 Dysmetria of movements
 Total inability to move the eye
 Commonly occur in CVA patients
Internuclear ophthalmoplegia-
 Lesion in MLF
 Between the abducens nucleus and C/L medial rectus
subnucleus of the oculomotor nerve
 Impairs adducting saccades of the ipsilateral eye,
which become either slow or absent
 Dysmetria
 Disconjugate nystagmus.
Internuclear ophthalmoplegia-
 If INO is bilateral
 abduction saccades also may be slow
 Upward beating and torsional nystagmus
 Other clinical features
 skew deviation
 defective vertical smooth pursuit
 impairment of the vertical VOR
 impaired ability to suppress or cancel the vertical VOR.
Internuclear ophthalmoplegia-
 Occur with a variety of disorders of brainstem
 Vascular
 Demyelinating
 Metastatic
 Must be differentiated from the pseudo-INO of
myasthenia or a long-standing exotropia.
One & half syndrome-
 Damage to the caudal pons
 Ipsilateral MLF and either the ipsilateral PPRF or the
abducens nucleus
 It results in an ipsilateral gaze palsy with an ipsilateral
INO
 Intact horizontal movement is abduction of the
contralateral eye
One & half syndrome-
 If the facial nerve nucleus or fasciculus is involved,
oculopalatal myoclonus may develop
 Most common causes
 multiple sclerosis and
 brainstem stroke
 followed by metastatic
 primary brainstem tumors
 Ocular myasthenia may cause a pseudo-one-and-a-
half syndrome
Ocular motor apraxia-
 Loss of or severely diminished volitional saccades
 Retention of the fast phases of vestibular nystagmus
• Difficult horizontal saccades
• Head thrust towards desired
direction
Congenital
• Balint syndrome
• Both Horizontal & Vertical
• Simutagnosia/optic ataxia
Acquired
Convergence paralysis-
 Midbrain lesions ,dorsal midbrain syndrome.
 Cerebellar degeneration, Parkinson's disease, and
progressive supranuclear palsy, are associated with
poor convergence.
 Lack of pupillary constriction on attempted
convergence may differentiate psychogenic
convergence paralysis from organic disease.
Divergence paralysis-
 Uncrossed horizontal diplopia
 Intermittent or constant esotropia
 Abduction is full.
 Break in fusion later in life
 Treated easily with base-out prisms for the distance
correction
 Divergence paralysis is a controversial entity, difficult
to differentiate from divergence insufficiency and
bilateral sixth cranial nerve palsies.
Functional gaze palsies-
 Horizontal gaze palsy – miosis during attempted gaze
 Saccades-VOR should be stimulated (oculocephalic
maneuvers,calorics,chair rotation ), OKN test
 Pursuit
THANK YOU

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Gaze palsy

  • 1. Gaze Palsy Presenter- Dr Shubhangini J Moderator-Dr Monica Samant
  • 3. Supranuclear control of ocular motility- Versions- Same direction Vergence- Opposite direction Supranuclear control
  • 6. Cells in PPRF Burst Cell-sends pulse step to move the eye Pause Cell- inhibits firing of burst cell allowing burst cell to initiate saccade Tonic Cell – maintain the eye position
  • 8.
  • 9. Vestibulocerebellar system-  Important input of gaze system  Modulate eye movements  Stabilize eye against the gravitatinal & accelerational force  Maintaining clear vision
  • 10. Cerebellum-  Eye movements  Fixation accuracy  Suppress the vestibulo-ocular reflex  Controls smoothness of pursuit movements  Accuracy of saccades
  • 11. Lesion of Supranuclear oculomotor pathways -  Based on anatomical location-  Lesions of internuclear system  Immediate premotor structure in the brain  PPRF  Posterior commisure  Rostral mesencephalon  Cerebral hemisphere  Descending pathway from cerebral hemisphere  Superior colliculus  Thalamus
  • 12. Clinical Examination  Asymptomatic for gaze palsy  Blurring of vision  Diplopia
  • 13. Pre-requisite-  Observe position of eye in primary gaze  Ductions  Versions & vergence  Pursuit  Saccades
  • 14. Oculocephalic maneuvers-  Dolls eye reflex  Tilt the head 30 degree forward & fixate a distant target  Rotate the head in direction opposite to gaze palsy  Direct projection from vestibular system to ocular motor nuclei  Prenuclear,nuclear infranuclear reflex does not overcome  Lesion in cerebral cortex overcome by VOR
  • 15. Vestibular ocular reflex -  Tilt the head by 60 degree & irrigate external auditary meatus with cool/warm water  In normal subject/supranuclear gaze palsy eye deviate towards the irrigated side- nystagmus with fast phase to opposite side  Fast phase towards the stimulated eye when warm water is used
  • 16. Supranuclear eye movement disorder- Gaze palsy Horizontal Vertical
  • 17. Vertical gaze palsy-  Midbrain lesion  B/L cerebral hemisphere dysfunction  Parkinsons disease  Progressive supranuclear palsy  lipidosis
  • 18. Parinaud syndrome-  Dorsal midbrain syndrome  Lesion of posterior commisure & MRF  Cause- compression by mass in pineal region  Dilatation of third ventricle  Midbrain infarction  multiple sclerosis  AV malfomation  Poor to absent upgaze  Convergence retraction nystagmus in upgaze  Colliers sign  Setting sun sign
  • 19. Parinaud syndrome-  EMG shows co-contraction of occulomotor innervated muscles- retraction of globe  Neuroimaging scan  Surgical treatment causes resolution of ocular findings
  • 20.
  • 21.
  • 22. Progressive supranuclear palsy- Lesion of mesencephalic structure-  Steele-Richardson-Olszewski syndrome  Onset –after 40 years  Disorder of basal ganglia  Marked rigidity –trunk & neck  Little tremor  Difficulty with vertical eye movements down > up  Progresses to horizontal gaze disorder  End stage – global ophthalmoplegia
  • 23. Progressive supranuclear palsy-  Vertical direction more severely affected initially  Voluntary saccades affected first, convergence, and smooth pursuit later  Slowing of saccade velocity  Supranuclear movements primarily affected (vestibulo- ocular reflex spared)  Square wave jerks  Gait abnormalities  Nuchal rigidity
  • 24. Progressive supranuclear palsy-  Eyelid abnormalities:  upper eyelid retraction  reduced blink rate  apraxia of eyelid opening  blepharospasm  Postural instability with falls (often backwards)  Cervical and axial dystonia
  • 25.
  • 26. Progressive supranuclear palsy-  Wilson’s disease  Huntington diseas  Kernicterous
  • 27. Parkinsons disease-  Lesion of descending pathway from cerebral hemisphere  Upgaze palsy affecting saccades followed by pursuit  Cogwheel pursuit
  • 28. Lipidosis-  Lipid storage disease variant of niemann picks disease  Vertical saccades  Intact vertical oculocephalic maneuvers  Progressive dementia in late childhood  Choreoathetosis  hepatosplenomegaly
  • 29. Whipples disease-  Involvement of CNS – supranuclear gaze palsy  Initially vertical  Progressive dementia  Hypersomnia  Ataxia  Uveitis
  • 30. Monoocular elevation paresis-  No ocular deviation in primary gaze  Inability to elevate one eye  Prenuclear congenital unilateral midbrain lesion  Oculocephalic maneuver is normal  Lesion in pretectum  Connection of riMLF to the occulomotor nuclei  Forced duction & tensilon test are negative
  • 32. Skew deviation-  Skew deviation is a vertical divergence  “prenuclear” lesion of the vertical vestibulo-ocular pathways in the brainstem or cerebellum.  Comitant, associated with cyclotorsion of one or both eyes.  Noncomitant it can mimic a partial third or fourth cranial nerve palsy
  • 33. Skew deviation-  Occur most commonly with vascular lesions of the pons or lateral medulla (Wallenberg's syndrome)  lesions of the midbrain or upper pons  Alternating skew deviation, the hypertropia changes with the direction of gaze. The adducting eye usually is hypotropic,mimick superior oblique overaction.
  • 35. Ocular tilt reaction-  cyclotorsion of both eyes, and paradoxical head tilt, all to the same side – that of the lower eye  A tonic (sustained) ocular tilt reaction occurs with lesions of the ipsilateral utricle, vestibular nerve or nuclei, or a lesion in the region of the contralateral interstitial nucleus of Cajal and medial thalamus  A phasic (paroxysmal) ocular tilt reaction occurs with lesions of the ipsilateral interstitial nucleus of Cajal and may respond to baclofen. 
  • 36. Horizontal gaze palsy-  More common  Vary from  Gaze evoked nystagmus  Dysmetria of movements  Total inability to move the eye  Commonly occur in CVA patients
  • 37. Internuclear ophthalmoplegia-  Lesion in MLF  Between the abducens nucleus and C/L medial rectus subnucleus of the oculomotor nerve  Impairs adducting saccades of the ipsilateral eye, which become either slow or absent  Dysmetria  Disconjugate nystagmus.
  • 38.
  • 39. Internuclear ophthalmoplegia-  If INO is bilateral  abduction saccades also may be slow  Upward beating and torsional nystagmus  Other clinical features  skew deviation  defective vertical smooth pursuit  impairment of the vertical VOR  impaired ability to suppress or cancel the vertical VOR.
  • 40. Internuclear ophthalmoplegia-  Occur with a variety of disorders of brainstem  Vascular  Demyelinating  Metastatic  Must be differentiated from the pseudo-INO of myasthenia or a long-standing exotropia.
  • 41.
  • 42. One & half syndrome-  Damage to the caudal pons  Ipsilateral MLF and either the ipsilateral PPRF or the abducens nucleus  It results in an ipsilateral gaze palsy with an ipsilateral INO  Intact horizontal movement is abduction of the contralateral eye
  • 43. One & half syndrome-  If the facial nerve nucleus or fasciculus is involved, oculopalatal myoclonus may develop  Most common causes  multiple sclerosis and  brainstem stroke  followed by metastatic  primary brainstem tumors  Ocular myasthenia may cause a pseudo-one-and-a- half syndrome
  • 44. Ocular motor apraxia-  Loss of or severely diminished volitional saccades  Retention of the fast phases of vestibular nystagmus • Difficult horizontal saccades • Head thrust towards desired direction Congenital • Balint syndrome • Both Horizontal & Vertical • Simutagnosia/optic ataxia Acquired
  • 45. Convergence paralysis-  Midbrain lesions ,dorsal midbrain syndrome.  Cerebellar degeneration, Parkinson's disease, and progressive supranuclear palsy, are associated with poor convergence.  Lack of pupillary constriction on attempted convergence may differentiate psychogenic convergence paralysis from organic disease.
  • 46. Divergence paralysis-  Uncrossed horizontal diplopia  Intermittent or constant esotropia  Abduction is full.  Break in fusion later in life  Treated easily with base-out prisms for the distance correction  Divergence paralysis is a controversial entity, difficult to differentiate from divergence insufficiency and bilateral sixth cranial nerve palsies.
  • 47. Functional gaze palsies-  Horizontal gaze palsy – miosis during attempted gaze  Saccades-VOR should be stimulated (oculocephalic maneuvers,calorics,chair rotation ), OKN test  Pursuit