27 nystagmus hubli

7/28/2019 27 Nystagmus Hubli

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Management of Nystagmus the

Ophthalmologists perspectiveDr. R.R.Battu

Consultant Pediatric Ophthalmologist

Narayana NethralayaBangalore


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Historically

What is the presenting feature? Informant:::

Nystagmus -Wobblyeyes

Anomalous HeadPosture

Poor vision Photophobia

Night blindness Oscillopsia

Vertigo

Diplopia

Head nodding

Many times a combination of the above !!


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Historically

Family history Poor vision

Nystagmus Neurological disease


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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


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Historically

MedicationAnticonvulsants

Sedatives Psychiatric medications

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


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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 ]


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Observe

One time observation

Multiple session observation Usually required in children

Tired adults


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What to Observe

The eye The alignment

The nystagmusAnomalous Head position


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The Eye

Evaluate refractive error Evaluate the anterior segment

Evaluate the posterior segment


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Visual Acuity

Behaviour

Eye poking

Pre verbal child or infant Fix and followOther techniques


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Special problems with Latentnystagmus - Infantile Esotropia

Fogging

Polarised glasses Vectograph

Neutral density filter

Remote occlusion

The Spielman Occluder


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The Eye

Microphthalmos Obvious malformations

AFFERENT PUPILLARY DEFECT


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The Eye

Iris Obvious or subtle transillumination defects

Ocular or oculocutaneous albinism is usually astraightforward diagnosis. The anteriorsegment clues you onto the typical posteriorsegment abnormalities

The lens Cataract


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The Eye

Optic nerve abnormalities Hypoplasia

Atrophy

Coloboma

Retinal abnormalities Albinism

Macular hypoplasia

Cicatricial ROP

Dysplasia

Coloboma

Pigmentary retinopathy


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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


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The Nystagmus

Pendular or Jerk Direction Frequency and Amplitude

Variation with gaze

Variation with convergence Variation with monocular

occlusion

Binocular symmetric

Binocular asymmetric Monocular


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How long to observe ?

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

Periodic Alternating Nystagmus


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Serious neurological disease?

Asymmetricnystagmus

Monocular nystagmusVisual pathwaydisorders !

Vertical nystagmus

Purely torsionalnystagmus


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Evaluation

Asymmetric nystagmus

INO

Spasmus nutans

Rarely Congenital nystagmus

Parasellar tumours

Restrictive or paralytic ocular muscular disorders


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Congenital Idiopathic Nystagmus

Observation Most commonly horizontal

Pendular or jerk

Horizontal nystagmus invertical gaze positions [Uniplanar ]

Null position Eccentric oron near gaze

Usually symmetric

Fulcrum of rotation inapparently asymmetricnystagmus.


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Congenital Idiopathic Nystagmus

Typically 3 phases of development [ Dr. RobertReinecke]

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


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Spasmus nutans

Head noddingAnomalous head position

Monocular/asymmetric nystagmus Shimmering

RULE OUT CNS TUMOUR [ glioma ]


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Latent nystagmus/ Manifest LatentNystagmus

Probably the only cause of Infantile nystagmus

which does not need Electrophysiologic study orNeuro imaging


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Latent nystagmus

Beats away from thecovered eye [ towardsthe fixing eye ]


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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 verticaland cyclovertical head turns


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Electrophysiology

ERG, EOG and VER Would probably be indicated in most

situations as an initial workup May allow to avoid neuroimaging


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Neuro imaging

Again, would probably be required as aninitial workup, unless there is

unequivocally ophthalmic cause ofnystagmus evident on examination andElectrophysiology


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TREATMENT

Drug treatment Optical treatment

Chemodenervation Surgical treatment


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Drug Therapy - Specific

Pendular Nystagmus Gabapentin andMemantine

PAN Baclofen

Superior Oblique MyokymiaCarbemazipine, Gabapentin


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Drug Therapy Less specific

Pendular Valproate, Trihexyphenidyl,Isoniazid, Cannabis

Downbeat nystagmus 3,4diaminopyridine, 4 aminopyridine,gabapentin, clonazepam, baclofen

Any form of Nystagmus Clonazepam,baclofen


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Optical treatment

CORRECT REFRACTIVE ERROR


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Refraction in nystagmus

1. Binocular UCVA in forced pp2. Binocular UCVA in preferred AHP


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Refraction in nystagmus

1. Binocular retinoscopy with patient fixing eitherin AHP or forced PP

1. Put the lenses in front of both eyes, fog one eye by1-3 lines

2. Subjectively refract other eye

3. Repeat on the other side

4. If there is no strabismus ( orthophoric), then addupto 7pd BO prism and -1.0DS to the prescription,observe nystagmus and check binocular acuity

5. Repeat all steps with cycloplegia


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Factors which can be improved

Visual acuityVA, contrast sensitivity, colour, motion sensitivity,

gaze angle

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

OscillopsiaAcquired nystagmus, decompensated congenital

nystagmus Hypo accommodation Photophobia


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Refractive Correction

In children upto 10 years, full cycloplegicrefraction

In adults, subjective, try to push over timeif there is a difference in sub and objrefraction


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Amblyopia therapy

May significantly decrease or eliminateMLN LN

Periods of occlusion have to be veryprolonged in patients with LN

Alternatively fogging or penalisation may

have to be used


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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


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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 eyemovement !!


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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


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Chemodenervation

Botox

2.5 5 units into all horizontal recti

Retrobulbar injection of 25 30 units


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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


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Chemodenervation

Complications include Ptosis

Diplopia Filamentary keratitis


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Electronystagmography

Nystagmovideography


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Surgical principles

Decrease the amplitude of nystagmus Maximal recession of horizontal muscles

Tenotomy

Increase foveation time

Tenotomy Broaden the null zone


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Rotate the null zoneAnderson Goto

Kestenbaum Parks modification of KestenbaumAugmented Kestenbaum

40% 60%

Induce an attempt to convergeArtificial divergence surgery


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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


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

Classic +60% - for > 45 deg of face turn

Problems

Intractable diplopia


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Vertical AHP

Chin up

IR recess SR resect Chin down

IR resect SR recess

Anteriorisation of IO


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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:10.1038/sj.eye.6700431
http://www.nature.com/eye/journal/v17/n5/full/6700431a.htmlhttp://www.nature.com/eye/journal/v17/n5/full/6700431a.htmlhttp://www.nature.com/eye/journal/v17/n5/full/6700431a.htmlhttp://www.nature.com/eye/journal/v17/n5/full/6700431a.htmlhttp://www.nature.com/eye/journal/v17/n5/full/6700431a.htmlhttp://www.nature.com/eye/journal/v17/n5/full/6700431a.html


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Cyclovertical AHP

As an adaptation to torsional nystagmus Surgery to recreate the torsional directioncreated by the patients head tilt

Several methods Strengthen or weaken obliques Slanting recti insertions

Vertical recti slanting


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Surgery

Other problems Management of co existent strabismus with

nystagmusAcquiring of a new head position - PAN

Creating a new strabismus


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Surgery primarily designed toimprove 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


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DellOsso & Hertle

Based on the principle of enthesialproprioceptive 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 rectustenotomy in patients with congenital nystagmus. Results in 10 adults. Ophthalmology 2003;110:2097-105.

Hertle RW, Dell'Osso LF, FitzGibbon EJ, Thompson D, Yang D, Mellow SD. Horizontal rectusmuscle tenotomy in patients with infantile nystagmus syndrome: a pilot study. JAAPOS 2004;8:539-48.


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Summary

Evaluation of nystagmus ismultidisciplinary

However, it is possible to improve thequality of life with drugs/opticaldevices/surgical procedures

No single procedure has shown to beconsistently predictive of success

This does not mean we cannot try.


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Thank you

27 nystagmus hubli

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