1. M. SOLTAN SANJARI, M.D. RASSOUL AKRAM HOSPITAL I. U. M. S.
NYSTAGMUS
M. SOLTAN SANJARI, M.D.
RASSOUL AKRAM HOSPITAL
I. U. M. S.

2. NYSTAGMUS
Nystagmus is a rhythmic oscilation of one or both eyes about one or more axes.

3. NYSTAGMUS Ethiology 1. Secondary to visual deficit
2. Secondary to intracranial
lesions and drug toxicit
3. Congenital benign idiopathic

4. NYSTAGMUS Mechanisms:
1. The nystagmus intensity is too high, or vision is too poor for complete suppression
2. Concomitant disorder of the smooth pursuit system
3. The fixation and smooth pursuit systems are themselves at fault

5. NYSTAGMUS Classification: 1. Clinical appearance
2. The waveform as revealed by ENG
3. Etiological grounds

6. NYSTAGMUS Terminology: Congenital Jerk and Pendular Nystagmus
1.Sensory Defect Nystagmus (SDN)
2. Congenital Idiopathic Nystagmus (CIN)
Jerk and Pendular Nystagmus
Axes of Oscillations
Direction
Null Zone
Amplitude, Frequency, Intensity

8. NYSTAGMUS Terminology: Manifest, Latent, Latent Component
Gaze-evoked, Gaze-paretic, Gaze-dependent
Asymmetry and Dissociated

9. NYSTAGMUS Examination: Family History Time of onset Ocular Examination
ERG, PVEP
ENG

10. NYSTAGMUS ENG 1. Jerk N. with an accelerating slow phase
2. Jerk N. with an decelerating slow phase
3. Constant velocity or linear slow phase
4. Pendular N.

12. NYSTAGMUS Physiologic Nystagmus 1. OKN 2. Induced Vestibular N.
3. End Point N.
4. Voluntary N.

13. Voluntary

14. Vestibular

15. OKN

16. NYSTAGMUS Latent Nystagmus (LN): Most common Before 6 mo.
Horizontal, Jerk, Conjugate
Wave form
Primary position, Add., Abd. Head turn
Genetic factor

17. MLN plus Alternating fixation strabismus
fast phases always in the direction of the fixating eye
misdiagnosed as having CN, because the nystagmus is present with both eye opens

18. NYSTAGMUS SDN, and CIN: Sensory Defect ? Incidence 9/1
Horizontal, Circumrotatory in early infancy
Null Zone ( 1/3 is eccentric )
Intensity
Inheritance
Optokinetic Response
Wave form
Visual Performance
Oscillopsia

19. Congenital nystagmus
Characteristics: Binocular Similar amplitude in both eyes Usually uniplanar (horizontal) in all gazes Diminished by convergence Increased by fixation attempt Superimposition of latent component Abolished in sleep Head oscillations

21. Head posture

22. Wandering eye

23. Sensory Defect Nystagmus
Consequent to bilateral visual loss
cannot be distinguished from CIN in a patient with coexisting primary visual abnormalities.
Monocular visual loss may produce monocular nystagmus, usually vertical, at any age from birth through adult life (it may mimic spasmus nutans, particularly if there is associated head nodding)

24. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
Gaze Paretic Nystagmus
The most common form of N. after infancy
Mismatch between gaze-holding circuit and EOM dynamics
Head thrusts
Cerebellar Disease, Drugs, Myasthenia, Vestibular Disease, …………….

27. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
Rebound Nystagmus
Usually with GPN
Unilateral or bilateral
Not dependent to vision
No change with illumination
Flocculus tumors
Chronic vestibulocerebellar disease

28. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
Acquired Pendular Nystagmus
High frequency, Low frequency, Horizontal, Vertical, Circular, Elliptical, Unilateral, Bilateral
Demyelinating, Oculopalatal myocolonus, Drugs, Glue stiffing, Late low vision, Neurodegeneratives, Chiasmal Glioma, Craniopharyngioma
Head thrust

29. Acquired Pendular Nystagmus

30. Acquired Pendular Nystagmus

31. Acquired Pendular Nystagmus

32. Acquired Pendular Nystagmus

33. See-saw Nystagmus

34. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
See-saw Nystagmus
Elevates and Intorts
Depresses and Extorts
Bitemporal hemianopia (Maddox 1914)
Parasellar and Chiasmal Lesions
Damage to the pathway of zonaincerta to the interstitial nucleus of Cajal (Thalamic lesion)
Congenital and Idiopathic

35. See-saw Nystagmus

36. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
Vestibular Nystagmus
Peripheral and Central
Dizziness, Vertigo, Oscillopsia
Central adaptation
Recovery Nystagmus
Vestibular Neuritis
Positional Nystagmus
Benign paroxysmal Positional Vertigo

37. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
Downbeat Nystagmus
 Lateral gaze
Craniocervical Abnormality, Cerebellar Degeneration,  ICP, Drugs, Nutritional Deficiencies
Upbeat Nystagmus
Pontomedulary Junction, Midbrain, Vermis Lesions
Organophosphate Poisoning

39. Down beaten and horizontal Nystagmus MS

40. Upbeat Nystagmus

41. Upbeat Nystagmus

42. Downbeat Nystagmus

45. Downbeat Nystagmus

46. Downbeat Nystagmus

47. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
Torsional Nystagmus
Sometimes only detect by ophthalmoscopy
Midpontine , Central vestibular connections Lesions
Part of SSN, Peripheral vestibular Nystagmus, SDN/CIN, LN.

49. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
Abduction Nystagmus
INO, Myasthenia, After strabismus surgery
Periodic Alternating Nystagmus
Acquired. Part of SDN/CIN
90 Second each cycle
Ping-Pong Gaze
Lower brain stem, Cerebellar, Anomalies
Drugs, Chronic alcoholism.
Baclofen Sometimes useful

50. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
Epileptic Nystagmus
Rare but may be the only sign of seizure
Usually Horizontal
Lid Nystagmus
Usually associated with vertical nystagmus
Icthal phenomenon, Posterior fossa lesions,

52. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
Convergence-Retraction Nystagmus
Posterior commissure Lesions: Hemorrhage, Infarct, Demyelinating, Hydrocephalus.
Downward OKN  CRN

54. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
Head Oscilations (Head shaking - Head nodding)
1. SDN/CIN
2. Bobble-Head Doll Syndrome
3. Spasmus Nutans

55. NEUROLOGICAL AND NEUROMUSCULAR NYSTAGMUS
Spasmus Nutans (Nodding Spasm)
Triad: 1. Asymetric APN
2.Head Nodding
3. Head Tilt or Turn
4mo. - 2 years improved before 5 years
Poor Family
ERG
Neuroimaging

56. Management

57. Management Aims Vision improvement Oscillopsia Improvement
Head posture elimination
Treatment of Strabismus

58. Non-Surgical Management
Treatment of amblyopia
Refractive errors correction
Minus therapy
Partial field occlusion
Contact lenses
Prisms
Head posture
Vision

59. Non-Surgical Management
Systemic medications
Baclofen ( CN, See-saw, PAN )
Clonazepam ( Downbeaen )
Carbamazepine ( SO myokymia )
Botulinum toxin
( Diplopia, ptosis, filamentary keratopathy,... )

60. Surgical Treatment To eliminate a compensatory head posture
To decrease nystagmus amplitude, or for both reasons
strabismus surgery may convert manifest-latent nystagmus to latent nystagmus, causing improvement of binocular visual acuity

61. Head Turn
“the eyes should always be shifted in the direction of the anomalous head posture”
Head turn or tilt of more than 15 to 20 is of cosmetic or functional significance
Ascertain beyond doubt by repeated examinations that the direction of the null zone and thus of the head turn is consistent

62. Head Turn Kestenbaum-Anderson Procedure Posterior Fixation Suture
Modified Anderson Procedure
Modification for coexisting heterotropia

63. Chin-Up or Down Large recession of vertical muscles
Think about lids position
Vertical R & R

64. Head Tilt Horizontal or vertical muscles displacement
For head tilt to right:
Transposing right SR nasally and right IR temporally and opposite in left eye or
Supraplacement of right LR and infraplacement
of right MR

65. To decrease nystagmus amplitude
Large recession of 4 horizontal muscles
Disinsertion and reinsertion of 4 horizontal muscles (Hertel)

66. THANK YOU