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NYSTAGMUS
PRESENTER- DR. SREYA CHAKRABORTY
GUIDE – DR. SANJUKTA MAHAPATRA
INTRODUCTION
 Nystagmus- is defined as repetitive to and fro movement of the eyes that is
initiated by a slow phase(drift) followed by rapid corrective eye movement.
 Saccadic intrusions- spontaneous rapid eye movement without slow phase.
 Thus the difference between the two lies in the initial eye movement.
PATHOPHYSIOLOGY
 Foveal centration of an object of regard is necessary to obtain the highest level of visual
acuity.
 3 control mechanisms maintain steady gaze
1) FIXATION
2) THE VESTIBULO-
OCULAR REFLEX
3) THE NEURAL
INTEGRATOR
TERMINOLOGIES
 Amplitude – is the excursion of the nystagmus.
1. Fine- less than 5º
2. Medium - 5º-15º
3. Coarse- greater than 15º.
 Frequency – number of to and fro movements in one second.
 slow- (1-2 Hz)
 Medium- (3-4 Hz)
 Fast – (5Hz or more)
 Intensity – amplitude × frequency
 Null zone – position where intensity of nystagmus is minimized .Patient assumes a
head posture, such that the eyes are in null zone.
JERK AND PENDULAR NYSTAGMUS
JERK NYSTAGMUS PENDULAR NYSTAGMUS
ALTERNATION OF SLOW DEFOVEATING
DRIFT AND RAPID CORRECTIVE SACCADE
IN OPPOSITE DIRECTION
SINUSOIDAL OSCILLATION WITH SLOW
PHASE IN BOTH DIRECTIONS AND NO
CORRECTIVE SACCADE
DIRECTION OF JERK NYSTAGMUS=
DIRECTION OF THE FAST PHASE
PENDULAR NYSTAGMUS MAYBE
HORIZONTAL OR VERTICAL
. RIGHT OR LEFT BEATING NYSTAGMUS
. UPBEAT OR DOWNBEAT NYSTAGMUS
NOT CHARACTERIZED BY RIGHT, LEFT,UP
,DOWN BEATING AS THERE IS NO FAST
PHASE
CONJUGATE/DISSOCIATE
 Conjugate – Nystagmus which is symmetric in direction, amplitude and rate
between two eyes.
 Dissociated – when it differs in any one of the parameters between two eyes.
 Disconjugate- Direction of oscillations differ between two eyes.
ALEXANDER’S LAW
 It states that the amplitude of jerk nystagmus is largest in the gaze
of direction of fast component.
 Grade 1- nystagmus only in the direction of fast component
 Grade 2 – nystagmus in primary gaze position.
 Grade 3 – nystagmus evident in all positions of the eyes.
CLASSIFICATION
PHYSIOLOGICAL
EARLY ONSET(CHILDHOOD)
PATHOLOGICAL
PHYSIOLOGICAL NYSTAGMUS
 TYPES OF PHYSIOLOGICAL NYSTAGMUS
 END POINT NYSTAGMUS
 VESTIBULAR(CALORIC OR ROTATIONAL)
 OPTOKINETIC
END POINT NYSTAGMUS
 Fine jerk nystagmus of moderate frequency
 Seen in extremes of gazes
 Fast phase in the direction of gaze
 Common in older patients.
 Pathological if :
1. Asymmetric
2. Persistent
OPTOKINETIC NYSTAGMUS
 Jerk nystagmus
 Induced by – moving repetitive targets across visual field
 Slow phase(pursuit) – eye follows the target
 Fast phase(saccades)- eye fixates on next target
 Uses :
1. Detecting malingering
2. Functional blindness
3. Testing visual potential in children
4. Assessment of isolated homonymous hemianopia.
VESTIBULAR NYSTAGMUS
 Jerk nystagmus due to altered inputs from vestibular nuclei to PPRF
 SLOW PHASE – vestibular nuclei
 FAST PHASE – brainstem and frontomesencephalic pathway
 Vestibular nystagmus maybe elicited by caloric stimulation
• Cold water – opposite side
• Warm water – same side
• Cold water in both ears – upwards
• Warm water in both ears - downwards
EARLY ONSET(CHILDHOOD) NYSTAGMUS
 3 types
1. Congenital nystagmus
2. Latent nystagmus
3. Spasmus nutans
CONGENITAL NYSTAGMUS
 80% of all nystagmus
 Infantile nystagmus syndrome
 Usually diagnosed during infancy
 Almost always conjugate and horizontal
 Pathophysiology:
 Sensory deficit (afferent nystagmus)-impairment of central vision in early life.
 Congenital motor(efferent) nystagmus-
I. family history common
II. X-linked(dominant or recessive)
III. Visual acuity better than sensory deficit
CHARACTERISTICS
 Elicited when tries to fixate on an object/attention/anxiety
 Eyelid closure/convergence might suppress
 Decreases when eyes moved to null position
 Distinctive feature – waveforms (increasing velocity and pendular)
 Hallmark- FOVEATION PERIODS
 Other associated finding – “inverted pursuit” or “reversed optokinetic
nystagmus”
 Head turns – adaptive strategy
 No oscillopsia
LATENT NYSTAGMUS
 Horizontal conjugate jerk nystagmus
 Appears when one eye is covered
 After mono-ocular occlusion- fast phase beats towards viewing eye and slow
phase towards the other.
 Usually associated with strabismus- esotropia
 Amblyopia is frequent
 Pathophysiology – defect in cortical motor processing
Results from lack of development of binocular vision.
SPASMUS NUTANS
TRIAD OF SPASMUS NUTANS
Pendular
nystagmus
Head
nodding
Spasmus
nutans
Anomalous
head
position
CHARACTERISTICS
 Onset usually in the first year of life
 Disappears by 3-4 yrs.
 Intermittent , binocular , small- amplitude, high frequency ,
 Pendular oscillations
 Can be monocular , asymmetric and variable in different positions of gaze
 Usually benign
 Head nodding- first abnormality noticed
 Neuroimaging recommended
ACQUIRED NYSTAGMUS
 Clinical features of nystagmus with lesions affecting visual pathway
 Diseases of retina- congenital or acquired retinal disorders causing blindness lead to continuous jerk
nystagmus
With components in all three planes.
 Diseases of optic nerves-
1. Pendular nystagmus
2. Monocular pendular nystagmus in monocular ON affection
3. Binocular ON disease- HEIMANN BIELSCHOWSKY PHENOMENON
 LESIONS AFFECTING OPTIC CHIASMA-
Parasellar lesions such as pituitary tumours a/w – SEE-SAW NYSTAGMUS
 POST CHIASMAL LESIONS-
Horizontal nystagmus documented with unilateral disease of cerebral hemisphere
Especially when lesion is large and posterior
AETIOLOGY OF PENDULAR NYSTAGMUS
 UNILATERAL DISEASE OF OPTIC NERVE
 DEMYELINATING DISEASE
 TOLUENE ABUSE
 OCULOPALATAL MYOCLONUS
 ACUTE BRAINSTEM STROKE
 WHIPPLE’S DISEASE
 CONGENITAL NYSTAGMUS
NYSTAGMUS CAUSED BY VESTIBULAR IMBALANCE
TYPES OF CENTRAL VESTIBULAR NYSTAGMUS
 3 TYPES
1. UPBEAT
2. DOWNBEAT
3. TORSIONAL
DOWNBEAT NYSTAGMUS
 Nystagmus intensity is greatest in downgaze , least in upgaze.
 Enhanced- patient looking down and to one side.
 Most commonly associated with disease affecting cerebellum, craniocervical
junction.
 Manifestation of drug toxicity- lithium
 Convergence- may convert it to upbeat.
 Consequences- oscillopsia and postural instability
AETIOLOGY OF DOWNBEAT NYSTAGMUS
 CEREBELLAR DEGENERATION
 CRANIOCERVICAL ANOMALIES
 MULTIPLE SCLEROSIS
 ENCEPHALITIS
 HEAD TRAUMA
 TOXIC
 ANTICONVULSANT MEDICATION
 LITHIUM
 ALCOHOL
 VITB12 DEF
DOWNBEAT NYSTAGMUS
UPBEAT NYSTAGMUS
 Nystagmus intensity greatest in upgaze.
 Doesn’t increase in left or right gaze
 Convergence may enhance , suppress or convert upbeat to downbeat
 Causes- lesions of lower pontine tegmentum , medulla
midbrain
CAUSES OF UPBEAT NYSTAGMUS
 CEREBELLAR DEGENERATIONS
 MS
 INFARCTION/TUMOURS OF MEDULLA,MIDBRAIN OR CEREBELLUM
 WERNICKE’S ENCEPHALOPATHY
 BEHCET’S SYNDROME
 TOBACCO
 LEBER’S CONGENITAL AMAUROSIS
 MIDDLE EAR DISEASE
UPBEAT
TORSIONAL NYSTAGMUS
 LESS COMMON
 CAUSES-
1. SYRINGOBULBIA
2. CHIARI MALFORMATION
3. BRAINSTEM TUMOUR
4. HEAD TRAUMA
5. MS
6. OCULAR TILT REACTION
7. CONGENITAL
PERIODIC ALTERNATING NYSTAGMUS
 Conjugate horizontal jerk nystagmus
 Periodically reverses direction
 During active phase- frequency first increase and then decrease
 Active phase followed by interlude lasting 4-20s(eyes are steady)
 Followed by similar sequence in opposite direction
 Cause – cerebellar disease, ataxia telangiectasia ,phenytoin.
CONVERGENCE-RETRACTION NYSTAGMUS
 Jerk nystagmus
 Cause- co-contraction of extraocular muscles(esp medial recti)
 Induced by rotating OKN drum downwards.
 Refixation brings two eyes towards each other.
 Associated retraction of globe into orbit
 Causes- lesions of pretectal area such as pinealoma and vascular accidents.
TREATMENT OF NYSTAGMUS
 AMBLYOPIA AND REFRACTIVE ERROR CORRECTION-
CONTACT LENSES
COMBINATION OF HIGH MINUS CONTACT LENS WITH HIGH PLUS SPECTACLE LENSES
 MEDICATIONS – BACLOFEN AND GABAPENTIN
 BOTULINUM TOXIN INJECTION INTO THE EOM
 SURGERY – RECESSION OF HORIZONTAL RECTI
TAKE HOME MESSAGE
 NYSTAGMUS CAN BE PHYSIOLOGICAL, CHILDHOOD ONSET AND PATHOLOGICAL
 ASSOCIATED WITH STRABISMUS AND AMBLYOPIA
 NEURO-CONSULTATION SHOULD BE DONE
 VISUAL REHABILITATION SHOULD BE CONSIDERED IN SEVERE RETINAL AND OPTIC NERVE DISEASE.
 PROPER GENETIC COUNSELLING
THANK YOU

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NYSTAGMUS In neurophthalmology by sreya c

  • 1. NYSTAGMUS PRESENTER- DR. SREYA CHAKRABORTY GUIDE – DR. SANJUKTA MAHAPATRA
  • 2. INTRODUCTION  Nystagmus- is defined as repetitive to and fro movement of the eyes that is initiated by a slow phase(drift) followed by rapid corrective eye movement.  Saccadic intrusions- spontaneous rapid eye movement without slow phase.  Thus the difference between the two lies in the initial eye movement.
  • 3. PATHOPHYSIOLOGY  Foveal centration of an object of regard is necessary to obtain the highest level of visual acuity.  3 control mechanisms maintain steady gaze 1) FIXATION 2) THE VESTIBULO- OCULAR REFLEX 3) THE NEURAL INTEGRATOR
  • 4. TERMINOLOGIES  Amplitude – is the excursion of the nystagmus. 1. Fine- less than 5º 2. Medium - 5º-15º 3. Coarse- greater than 15º.  Frequency – number of to and fro movements in one second.  slow- (1-2 Hz)  Medium- (3-4 Hz)  Fast – (5Hz or more)  Intensity – amplitude × frequency  Null zone – position where intensity of nystagmus is minimized .Patient assumes a head posture, such that the eyes are in null zone.
  • 5. JERK AND PENDULAR NYSTAGMUS JERK NYSTAGMUS PENDULAR NYSTAGMUS ALTERNATION OF SLOW DEFOVEATING DRIFT AND RAPID CORRECTIVE SACCADE IN OPPOSITE DIRECTION SINUSOIDAL OSCILLATION WITH SLOW PHASE IN BOTH DIRECTIONS AND NO CORRECTIVE SACCADE DIRECTION OF JERK NYSTAGMUS= DIRECTION OF THE FAST PHASE PENDULAR NYSTAGMUS MAYBE HORIZONTAL OR VERTICAL . RIGHT OR LEFT BEATING NYSTAGMUS . UPBEAT OR DOWNBEAT NYSTAGMUS NOT CHARACTERIZED BY RIGHT, LEFT,UP ,DOWN BEATING AS THERE IS NO FAST PHASE
  • 6. CONJUGATE/DISSOCIATE  Conjugate – Nystagmus which is symmetric in direction, amplitude and rate between two eyes.  Dissociated – when it differs in any one of the parameters between two eyes.  Disconjugate- Direction of oscillations differ between two eyes.
  • 7. ALEXANDER’S LAW  It states that the amplitude of jerk nystagmus is largest in the gaze of direction of fast component.  Grade 1- nystagmus only in the direction of fast component  Grade 2 – nystagmus in primary gaze position.  Grade 3 – nystagmus evident in all positions of the eyes.
  • 9. PHYSIOLOGICAL NYSTAGMUS  TYPES OF PHYSIOLOGICAL NYSTAGMUS  END POINT NYSTAGMUS  VESTIBULAR(CALORIC OR ROTATIONAL)  OPTOKINETIC
  • 10. END POINT NYSTAGMUS  Fine jerk nystagmus of moderate frequency  Seen in extremes of gazes  Fast phase in the direction of gaze  Common in older patients.  Pathological if : 1. Asymmetric 2. Persistent
  • 11. OPTOKINETIC NYSTAGMUS  Jerk nystagmus  Induced by – moving repetitive targets across visual field  Slow phase(pursuit) – eye follows the target  Fast phase(saccades)- eye fixates on next target  Uses : 1. Detecting malingering 2. Functional blindness 3. Testing visual potential in children 4. Assessment of isolated homonymous hemianopia.
  • 12. VESTIBULAR NYSTAGMUS  Jerk nystagmus due to altered inputs from vestibular nuclei to PPRF  SLOW PHASE – vestibular nuclei  FAST PHASE – brainstem and frontomesencephalic pathway  Vestibular nystagmus maybe elicited by caloric stimulation • Cold water – opposite side • Warm water – same side • Cold water in both ears – upwards • Warm water in both ears - downwards
  • 13. EARLY ONSET(CHILDHOOD) NYSTAGMUS  3 types 1. Congenital nystagmus 2. Latent nystagmus 3. Spasmus nutans
  • 14. CONGENITAL NYSTAGMUS  80% of all nystagmus  Infantile nystagmus syndrome  Usually diagnosed during infancy  Almost always conjugate and horizontal  Pathophysiology:  Sensory deficit (afferent nystagmus)-impairment of central vision in early life.  Congenital motor(efferent) nystagmus- I. family history common II. X-linked(dominant or recessive) III. Visual acuity better than sensory deficit
  • 15. CHARACTERISTICS  Elicited when tries to fixate on an object/attention/anxiety  Eyelid closure/convergence might suppress  Decreases when eyes moved to null position  Distinctive feature – waveforms (increasing velocity and pendular)  Hallmark- FOVEATION PERIODS  Other associated finding – “inverted pursuit” or “reversed optokinetic nystagmus”  Head turns – adaptive strategy  No oscillopsia
  • 16. LATENT NYSTAGMUS  Horizontal conjugate jerk nystagmus  Appears when one eye is covered  After mono-ocular occlusion- fast phase beats towards viewing eye and slow phase towards the other.  Usually associated with strabismus- esotropia  Amblyopia is frequent  Pathophysiology – defect in cortical motor processing Results from lack of development of binocular vision.
  • 17. SPASMUS NUTANS TRIAD OF SPASMUS NUTANS Pendular nystagmus Head nodding Spasmus nutans Anomalous head position
  • 18. CHARACTERISTICS  Onset usually in the first year of life  Disappears by 3-4 yrs.  Intermittent , binocular , small- amplitude, high frequency ,  Pendular oscillations  Can be monocular , asymmetric and variable in different positions of gaze  Usually benign  Head nodding- first abnormality noticed  Neuroimaging recommended
  • 19. ACQUIRED NYSTAGMUS  Clinical features of nystagmus with lesions affecting visual pathway  Diseases of retina- congenital or acquired retinal disorders causing blindness lead to continuous jerk nystagmus With components in all three planes.  Diseases of optic nerves- 1. Pendular nystagmus 2. Monocular pendular nystagmus in monocular ON affection 3. Binocular ON disease- HEIMANN BIELSCHOWSKY PHENOMENON
  • 20.  LESIONS AFFECTING OPTIC CHIASMA- Parasellar lesions such as pituitary tumours a/w – SEE-SAW NYSTAGMUS  POST CHIASMAL LESIONS- Horizontal nystagmus documented with unilateral disease of cerebral hemisphere Especially when lesion is large and posterior
  • 21. AETIOLOGY OF PENDULAR NYSTAGMUS  UNILATERAL DISEASE OF OPTIC NERVE  DEMYELINATING DISEASE  TOLUENE ABUSE  OCULOPALATAL MYOCLONUS  ACUTE BRAINSTEM STROKE  WHIPPLE’S DISEASE  CONGENITAL NYSTAGMUS
  • 22. NYSTAGMUS CAUSED BY VESTIBULAR IMBALANCE
  • 23. TYPES OF CENTRAL VESTIBULAR NYSTAGMUS  3 TYPES 1. UPBEAT 2. DOWNBEAT 3. TORSIONAL
  • 24. DOWNBEAT NYSTAGMUS  Nystagmus intensity is greatest in downgaze , least in upgaze.  Enhanced- patient looking down and to one side.  Most commonly associated with disease affecting cerebellum, craniocervical junction.  Manifestation of drug toxicity- lithium  Convergence- may convert it to upbeat.  Consequences- oscillopsia and postural instability
  • 25. AETIOLOGY OF DOWNBEAT NYSTAGMUS  CEREBELLAR DEGENERATION  CRANIOCERVICAL ANOMALIES  MULTIPLE SCLEROSIS  ENCEPHALITIS  HEAD TRAUMA  TOXIC  ANTICONVULSANT MEDICATION  LITHIUM  ALCOHOL  VITB12 DEF
  • 27. UPBEAT NYSTAGMUS  Nystagmus intensity greatest in upgaze.  Doesn’t increase in left or right gaze  Convergence may enhance , suppress or convert upbeat to downbeat  Causes- lesions of lower pontine tegmentum , medulla midbrain
  • 28. CAUSES OF UPBEAT NYSTAGMUS  CEREBELLAR DEGENERATIONS  MS  INFARCTION/TUMOURS OF MEDULLA,MIDBRAIN OR CEREBELLUM  WERNICKE’S ENCEPHALOPATHY  BEHCET’S SYNDROME  TOBACCO  LEBER’S CONGENITAL AMAUROSIS  MIDDLE EAR DISEASE
  • 30. TORSIONAL NYSTAGMUS  LESS COMMON  CAUSES- 1. SYRINGOBULBIA 2. CHIARI MALFORMATION 3. BRAINSTEM TUMOUR 4. HEAD TRAUMA 5. MS 6. OCULAR TILT REACTION 7. CONGENITAL
  • 31. PERIODIC ALTERNATING NYSTAGMUS  Conjugate horizontal jerk nystagmus  Periodically reverses direction  During active phase- frequency first increase and then decrease  Active phase followed by interlude lasting 4-20s(eyes are steady)  Followed by similar sequence in opposite direction  Cause – cerebellar disease, ataxia telangiectasia ,phenytoin.
  • 32. CONVERGENCE-RETRACTION NYSTAGMUS  Jerk nystagmus  Cause- co-contraction of extraocular muscles(esp medial recti)  Induced by rotating OKN drum downwards.  Refixation brings two eyes towards each other.  Associated retraction of globe into orbit  Causes- lesions of pretectal area such as pinealoma and vascular accidents.
  • 33.
  • 34. TREATMENT OF NYSTAGMUS  AMBLYOPIA AND REFRACTIVE ERROR CORRECTION- CONTACT LENSES COMBINATION OF HIGH MINUS CONTACT LENS WITH HIGH PLUS SPECTACLE LENSES  MEDICATIONS – BACLOFEN AND GABAPENTIN  BOTULINUM TOXIN INJECTION INTO THE EOM  SURGERY – RECESSION OF HORIZONTAL RECTI
  • 35. TAKE HOME MESSAGE  NYSTAGMUS CAN BE PHYSIOLOGICAL, CHILDHOOD ONSET AND PATHOLOGICAL  ASSOCIATED WITH STRABISMUS AND AMBLYOPIA  NEURO-CONSULTATION SHOULD BE DONE  VISUAL REHABILITATION SHOULD BE CONSIDERED IN SEVERE RETINAL AND OPTIC NERVE DISEASE.  PROPER GENETIC COUNSELLING