Management of Nystagmus - the Ophthalmologist's perspective

Management of Nystagmus - the Ophthalmologist's perspective

Management of Nystagmus the Ophthalmologists perspective Dr. R.R.Battu Consultant Pediatric Ophthalmologist Narayana Nethralaya Bangalore 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 !! 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 Pre verbal child or infant Fix and follow Other techniques

Special problems with Latent nystagmus - Infantile Esotropia Fogging Polarised glasses Vectograph

Neutral density filter Remote occlusion The Spielman Occluder 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 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 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 Congenital nystagmus, acquired nystagmus, convergence damping, adduction null in LN/MLN

Oscillopsia 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. 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; 8:539-48.

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

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