Management of Nystagmus the Ophthalmologists perspective

Dr. R.R.Battu Consultant Pediatric Ophthalmologist Narayana Nethralaya Bangalore

Historically
What is the presenting feature?
Nystagmus - Wobbly eyes Anomalous Head Posture Poor vision Photophobia

Informant:::

Night blindness Oscillopsia Vertigo Diplopia Head nodding

Many times a combination of the above !!

Historically
Family history
Poor vision Nystagmus Neurological disease

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

Historically
Medication
Anticonvulsants Sedatives Psychiatric medications

Occupation [ - and hobbies? ] Epilepsy Head Trauma Neurological abnormalities.. Craniofacial anomalies

 Is there a visual defect?

If so, qualify and quantify

Is this likely to be an Ocular nystagmus

Sensory defect nystagmus [ SDN ] Latent nystagmus [ LN/ MLN ]

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

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

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

Visual Acuity
Behaviour

Eye poking Fix and follow Other techniques

Pre verbal child or infant

 Special problems with Latent

Fogging Polarised glasses Vectograph Neutral density filter Remote occlusion The Spielman Occluder

nystagmus - Infantile Esotropia

The Eye
Microphthalmos Obvious malformations
AFFERENT PUPILLARY DEFECT

The Eye
Iris
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

The Eye
Optic nerve abnormalities
Hypoplasia Atrophy Coloboma

Retinal abnormalities
Albinism Macular hypoplasia Cicatricial ROP Dysplasia Coloboma Pigmentary retinopathy

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

The Nystagmus

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

How long to observe ?

Single concentrated effort of observation of
at least 3 minutes !!!

Periodic Alternating Nystagmus

Serious neurological disease?

Asymmetric

nystagmus Monocular nystagmus

Visual pathway disorders !

Vertical nystagmus Purely torsional

nystagmus

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

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.

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

Spasmus nutans
Head nodding Anomalous head position Monocular/asymmetric nystagmus
Shimmering

RULE OUT CNS TUMOUR [ glioma ]

Latent nystagmus/ Manifest Latent Nystagmus

Probably the only cause of Infantile nystagmus which does not need Electrophysiologic study or Neuro imaging

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

Anomalous Head Position

Null point Usually in an eccentric gaze position Head is positioned AWAY from the null
point
Beware PAN Wandering Null point

Mostly lateral turn, occasionally vertical

and cyclovertical head turns

i.e. Null point to left, face turn to right

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

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

TREATMENT
Drug treatment Optical treatment Chemodenervation Surgical treatment

Drug Therapy - Specific

Pendular Nystagmus Gabapentin and
Memantine

PAN Baclofen Superior Oblique Myokymia

Carbemazipine, Gabapentin

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

Optical treatment

CORRECT REFRACTIVE ERROR

Refraction in nystagmus
1. Binocular UCVA in forced pp 2. Binocular UCVA in preferred AHP

Refraction in nystagmus
1. Binocular retinoscopy with patient fixing either
in AHP or forced PP
1. Put the lenses in front of both eyes, fog one eye by 1-3 lines 2. Subjectively refract other eye 3. Repeat on the other side 4. If there is no strabismus ( orthophoric), then add upto 7pd BO prism and -1.0DS to the prescription, observe nystagmus and check binocular acuity 5. Repeat all steps with cycloplegia

Factors which can be improved

Visual acuity
VA, contrast sensitivity, colour, motion sensitivity, gaze angle

Anomalous Head Position Oscillopsia

Congenital nystagmus, acquired nystagmus, convergence damping, adduction null in LN/MLN

Acquired nystagmus, decompensated congenital nystagmus

Hypo accommodation Photophobia

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

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

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

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 !!

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

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

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

Chemodenervation
Complications include
Ptosis Diplopia Filamentary keratitis

Electronystagmography

Nystagmovideography

Surgical principles
Decrease the amplitude of nystagmus
Maximal recession of horizontal muscles Tenotomy

Increase foveation time

Tenotomy

Broaden the null zone

 Rotate the null zone

Anderson Goto Kestenbaum Parks modification of Kestenbaum Augmented Kestenbaum 40% 60%

Induce an attempt to converge

Artificial divergence surgery

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] Parks modification of Kestenbaums
5-6-7-8 rule [both eyes get 13 mm ] Very rarely corrects more than 10 -15 degrees

Surgery to correct AHP

Augmented K-A procedure
Classic + 40% - For > 30 deg of face turn Classic +60% - for > 45 deg of face turn

Problems
Intractable diplopia

Surgery to correct AHP

Vertical AHP
Chin up IR recess SR resect Chin down IR resect SR recess

Anteriorisation of IO

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, 587592. doi:10.1038/sj.eye.6700431

Surgery to correct AHP

Cyclovertical AHP

As an adaptation to torsional nystagmus Surgery to recreate the torsional direction

created by the patients head tilt Several methods
Strengthen or weaken obliques Slanting recti insertions Vertical recti slanting

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

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

DellOsso & 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;

110:2097-105. 8:539-48.

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;

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.

Thank you