1. Management of Nystagmus – the Ophthalmologist’s perspective
Dr. R.R.Battu
Consultant Pediatric Ophthalmologist
Narayana Nethralaya
Bangalore

2. Historically What is the presenting feature? Informant:::
Nystagmus - “Wobbly eyes”
Anomalous Head Posture
Poor vision
Photophobia
Night blindness
Oscillopsia
Vertigo
Diplopia
Head nodding
Many times a combination of the above !!

3. Historically
Family history
Poor vision
Nystagmus
Neurological disease

4. Historically When did this start?
At birth or shortly thereafter [ “Congenital” or infantile nystagmus ]
Congenital sensory or motor nystagmus
Congenital neurological nystagmus
Rare variants
PAN
Spasmus nutans

5. Historically Medication Occupation [ - and hobbies? ] Epilepsy
Anticonvulsants
Sedatives
“Psychiatric medications”
Occupation [ - and hobbies? ]
Epilepsy
Head Trauma
Neurological abnormalities……..
Craniofacial anomalies

6. Is there a visual defect? Is this likely to be an “ Ocular nystagmus”
If so, qualify and quantify
Is this likely to be an “ Ocular nystagmus”
Sensory defect nystagmus [ SDN ]
Latent nystagmus [ LN/ MLN ]

7. Observe One time observation Multiple session observation
Usually required in children
Tired adults

8. What to Observe The eye The alignment The nystagmus
Anomalous Head position

9. The Eye Evaluate refractive error Evaluate the anterior segment
Evaluate the posterior segment

10. Visual Acuity Behaviour Eye poking Pre verbal child or infant
Fix and follow
Other techniques

11. Special problems with Latent nystagmus - Infantile Esotropia
Fogging
Polarised glasses – Vectograph
Neutral density filter
Remote occlusion
The Spielman Occluder

12. The Eye
Microphthalmos
Obvious malformations
AFFERENT PUPILLARY DEFECT

13. The Eye Iris The lens Obvious or subtle transillumination defects
Ocular or oculocutaneous albinism is usually a straightforward diagnosis. The anterior segment clues you onto the typical posterior segment abnormalities
The lens
Cataract

14. The Eye Optic nerve abnormalities Retinal abnormalities Hypoplasia
Atrophy
Coloboma
Retinal abnormalities
Albinism
Macular hypoplasia
Cicatricial ROP
Dysplasia
Pigmentary retinopathy

15. Nystagmus Compensation Syndrome
The Alignment
Ortho, Eso or Exo?
In an infant:
Eso - Infantile esotropia with LN/MLN
Nystagmus Compensation Syndrome
Exo – Infantile exo,
many times with neuro-developmental issues

16. The Nystagmus Pendular or Jerk Direction Frequency and Amplitude
Variation with gaze
Variation with convergence
Variation with monocular occlusion
Binocular symmetric
Binocular asymmetric
Monocular

17. “How long” to “observe” ?
Single concentrated ‘effort’ of observation of at least 3 minutes !!!
Periodic Alternating Nystagmus

18. Serious neurological disease?
Asymmetric nystagmus
Monocular nystagmus
Visual pathway disorders !
Vertical nystagmus
Purely torsional nystagmus

19. Evaluation Asymmetric nystagmus INO Spasmus nutans
Rarely Congenital nystagmus
Parasellar tumours
Restrictive or paralytic ocular muscular disorders

20. Congenital Idiopathic Nystagmus
Observation
Most commonly horizontal
Pendular or jerk
Horizontal nystagmus in vertical gaze positions [ Uniplanar ]
Null position – Eccentric or on near gaze
Usually symmetric
Fulcrum of rotation in “apparently” asymmetric nystagmus.

21. Congenital Idiopathic Nystagmus
Typically 3 phases of development [ Dr. Robert Reinecke]
Phase 1- Broad triangular wave form [ 3-6 mths]
Phase 2- low amp pendular waveform [6-24 months]
Phase 3-Typical jerk nystagmus [24-36 months]
Historically:
No oscillopsia
Invariably improves with age

22. Spasmus nutans Head nodding Anomalous head position
Monocular/asymmetric nystagmus –
“ Shimmering”
RULE OUT CNS TUMOUR [ glioma ]

23. Latent nystagmus/ Manifest Latent Nystagmus
Probably the only cause of Infantile nystagmus which does not need Electrophysiologic study or Neuro imaging

24. Latent nystagmus
Beats away from the covered eye [ towards the fixing eye ]

25. Anomalous Head Position
Null point
Beware PAN
Wandering Null point
Usually in an eccentric gaze position
Head is positioned AWAY from the null point
i.e. Null point to left, face turn to right
Mostly lateral turn, occasionally vertical and cyclovertical head turns

26. Electrophysiology ERG, EOG and VER
Would probably be indicated in most situations as an initial ‘workup’
May allow to avoid neuroimaging

27. Neuro imaging
Again, would probably be required as an initial workup, unless there is unequivocally ophthalmic cause of nystagmus evident on examination and Electrophysiology

28. TREATMENT Drug treatment Optical treatment Chemodenervation
Surgical treatment

29. Drug Therapy - Specific
Pendular Nystagmus – Gabapentin and Memantine
PAN – Baclofen
Superior Oblique Myokymia – Carbemazipine, Gabapentin

30. Drug Therapy – Less specific
Pendular – Valproate, Trihexyphenidyl, Isoniazid, Cannabis
Downbeat nystagmus – 3,4 diaminopyridine, 4 aminopyridine, gabapentin, clonazepam, baclofen
Any form of Nystagmus – Clonazepam, baclofen

31. CORRECT REFRACTIVE ERROR
Optical treatment
CORRECT REFRACTIVE ERROR

32. Refraction in nystagmus
Binocular UCVA in forced pp
Binocular UCVA in preferred AHP

33. Refraction in nystagmus
Binocular retinoscopy with patient fixing either in AHP or forced PP
Put the lenses in front of both eyes, fog one eye by 1-3 lines
Subjectively refract other eye
Repeat on the other side
If there is no strabismus ( orthophoric), then add upto 7pd BO prism and -1.0DS to the prescription, observe nystagmus and check binocular acuity
Repeat all steps with cycloplegia

34. Factors which can be improved
Visual acuity
VA, contrast sensitivity, colour, motion sensitivity, gaze angle
Anomalous Head Position
Congenital nystagmus, acquired nystagmus, convergence damping, adduction null in LN/MLN
Oscillopsia
Acquired nystagmus, decompensated congenital nystagmus
Hypo accommodation
Photophobia

35. Refractive Correction
In children upto 10 years, full cycloplegic refraction
In adults, subjective, try to push over time if there is a difference in sub and obj refraction

36. Amblyopia therapy May significantly decrease or eliminate MLN …… LN
Periods of occlusion have to be very prolonged in patients with LN
Alternatively fogging or penalisation may have to be used

37. Optical treatment To direct the null point centrally
Prisms placed with apex directed towards the null point.
Large power prisms may have to be used.
Fresnels
May degrade vision

38. Optical treatment To stabilize visual image on the retina
High plus spectacle with high minus contact lens[ -58 & +32 ]
Entire 30 deg field focussed to centre of eye, and CL refocuses to the retina.
Image remains stable irrespective of eye movement !!

39. Optical treatment To induce convergence Base out prisms bilaterally
Induce a convergence
Useful only if there is a convergence null
May have to compensate with a -1.0 sph for induced accommodation

40. Chemodenervation Botox 2.5 – 5 units into all horizontal recti
Retrobulbar injection of 25 – 30 units

41. Chemodenervation Useful to reduce amplitude of nystagmus
Has been shown to improve foveation time and improve visual acuity slightly.
More useful in neurological acquired nystagmus, particularly in oculopalatal myoclonus
RB injection effect lasts for several weeks

42. Chemodenervation Complications include Ptosis Diplopia
Filamentary keratitis

43. Electronystagmography
Nystagmovideography

44. Surgical principles Decrease the amplitude of nystagmus
Maximal recession of horizontal muscles
Tenotomy
Increase foveation time
Broaden the null zone

45. Induce an attempt to converge
Rotate the null zone
Anderson
Goto
Kestenbaum
Parks’ modification of Kestenbaum
Augmented Kestenbaum
40%
60%
Induce an attempt to converge
Artificial divergence surgery

46. Face turns - horizontal
Surgery to correct AHP
Face turns - horizontal
Anderson advocated bilateral recession
Eg. Null zone to left, weaken levo- ‘verters’
Kestenbaum advocated recess-recess [ pull and push]
Park’s modification of Kestenbaum’s
rule [both eyes get 13 mm ]
Very rarely corrects more than degrees

47. Surgery to correct AHP Augmented K-A procedure Problems
Classic + 40% - For > 30 deg of face turn
Classic +60% - for > 45 deg of face turn
Problems
Intractable diplopia

48. Surgery to correct AHP Vertical AHP Chin up Chin down
IR recess – SR resect
Chin down
IR resect– SR recess
Anteriorisation of IO

49. Patient with right horizontal gaze palsy and head turn of approximately 20° to the right (a); the same patient 1 year after recession of right medial rectus and left lateral rectus muscles (b). Note: the patient can use his glasses more effectively. Patient with acquired nystagmus equilibrium in upward gaze; CHP with chin-down is present (c); the same patient 1 year after surgical weakening of both superior rectus muscles (d).
E C Campos1, C Schiavi1 and C Bellusci1.
Surgical management of anomalous head posture because of horizontal gaze palsy or acquired vertical nystagmus
Eye (2003) 17, 587–592. doi: /sj.eye

50. Surgery to correct AHP Cyclovertical AHP
As an adaptation to torsional nystagmus
Surgery to recreate the torsional direction ‘created’ by the patient’s head tilt
Several methods
Strengthen or weaken obliques
Slanting recti insertions
Vertical recti slanting

51. Surgery Other problems
Management of co existent strabismus with nystagmus
Acquiring of a new head position - PAN
Creating a new strabismus

52. Surgery primarily designed to improve vision
Artificial divergence
Bimedial recession
Unilateral recess-resect to XT
4 – muscle retro equatorial recession
10 mm MR and 12 mm LR
Ideal for PAN
May induce an exotropia

53. Dell’Osso & Hertle
Based on the principle of enthesial proprioceptive input to nystagmus at the insertion of the horizontal recti
Dell'Osso LF. Extraocular muscle tenotomy, dissection, and suture: A hypothetical therapy for congenital nystagmus. J Pediatr Ophthalmol Strab 1998; 35:232-3.
Hertle RW, Dell'Osso LF, FitzGibbon EJ, Thompson D, Yang D, Mellow SD. Horizontal rectus tenotomy in patients with congenital nystagmus. Results in 10 adults. Ophthalmology 2003; 110:
Hertle RW, Dell'Osso LF, FitzGibbon EJ, Thompson D, Yang D, Mellow SD. Horizontal rectus muscle tenotomy in patients with infantile nystagmus syndrome: a pilot study. JAAPOS 2004; 8:

54. Summary Evaluation of nystagmus is multidisciplinary
However, it is possible to improve the quality of life with drugs/optical devices/surgical procedures
No single procedure has shown to be consistently predictive of success
This does not mean we cannot try.

55. Thank you