Management of Nystagmus – the Ophthalmologist’s perspective

Management of Nystagmus – Management of Nystagmus – the Ophthalmologist’s the Ophthalmologist’s

perspectiveperspective

Dr. R.R.BattuDr. R.R.BattuConsultant Pediatric Consultant Pediatric

OphthalmologistOphthalmologistNarayana NethralayaNarayana Nethralaya

BangaloreBangalore

HistoricallyHistorically What is the presenting feature? What is the presenting feature? Informant:::Informant:::

– Nystagmus - Nystagmus - “Wobbly eyes” “Wobbly eyes”

– Anomalous Head Anomalous Head PosturePosture

– Poor visionPoor vision– PhotophobiaPhotophobia

– Night blindnessNight blindness– OscillopsiaOscillopsia– VertigoVertigo– DiplopiaDiplopia– Head noddingHead nodding

Many times a combination of the above !!

HistoricallyHistorically

• Family historyFamily history– Poor visionPoor vision– NystagmusNystagmus– Neurological diseaseNeurological disease


• When did this start? When did this start? – At birth or shortly thereafter At birth or shortly thereafter

[ “Congenital” or infantile nystagmus ][ “Congenital” or infantile nystagmus ]•Congenital sensory or motor nystagmusCongenital sensory or motor nystagmus

•Congenital neurological nystagmusCongenital neurological nystagmus

•Rare variantsRare variants– PANPAN– Spasmus nutansSpasmus nutans


• MedicationMedication– AnticonvulsantsAnticonvulsants– SedativesSedatives– ““Psychiatric medications” Psychiatric medications”

• OccupationOccupation [ - and hobbies? ] [ - and hobbies? ]

• EpilepsyEpilepsy

• Head TraumaHead Trauma

• Neurological abnormalities……..Neurological abnormalities……..

• Craniofacial anomaliesCraniofacial anomalies

• Is there a visual defect? Is there a visual defect? – If so, qualify and quantifyIf so, qualify and quantify

• Is this likely to be an “ Ocular Is this likely to be an “ Ocular nystagmus”nystagmus”– Sensory defect nystagmus [ SDN ]Sensory defect nystagmus [ SDN ]– Latent nystagmus [ LN/ MLN ] Latent nystagmus [ LN/ MLN ]

ObserveObserve

• One time observationOne time observation

• Multiple session observationMultiple session observation– Usually required in childrenUsually required in children– Tired adultsTired adults

What to ObserveWhat to Observe

• The eyeThe eye

• The alignmentThe alignment

• The nystagmusThe nystagmus

• Anomalous Head positionAnomalous Head position

The EyeThe Eye

• Evaluate refractive errorEvaluate refractive error

• Evaluate the anterior segmentEvaluate the anterior segment

• Evaluate the posterior segmentEvaluate the posterior segment

Visual AcuityVisual Acuity

• BehaviourBehaviour– Eye pokingEye poking

• Pre verbal child or infantPre verbal child or infant– Fix and followFix and follow– Other techniquesOther techniques

•Special problems with Latent Special problems with Latent nystagmus - Infantile Esotropianystagmus - Infantile Esotropia

– FoggingFogging– Polarised glasses – VectographPolarised glasses – Vectograph– Neutral density filterNeutral density filter– Remote occlusionRemote occlusion– The Spielman OccluderThe Spielman Occluder

The EyeThe Eye

• MicrophthalmosMicrophthalmos

• Obvious malformationsObvious malformations

• AFFERENT PUPILLARY DEFECTAFFERENT PUPILLARY DEFECT

The EyeThe Eye

• IrisIris– Obvious or subtle transillumination Obvious or subtle transillumination

defectsdefects– Ocular or oculocutaneous albinism is Ocular or oculocutaneous albinism is

usually a straightforward diagnosis. The usually a straightforward diagnosis. The anterior segment clues you onto the anterior segment clues you onto the typical posterior segment abnormalitiestypical posterior segment abnormalities

• The lensThe lens– CataractCataract

The EyeThe Eye

• Optic nerve abnormalitiesOptic nerve abnormalities– HypoplasiaHypoplasia– AtrophyAtrophy– ColobomaColoboma

• Retinal abnormalitiesRetinal abnormalities– AlbinismAlbinism– Macular hypoplasiaMacular hypoplasia– Cicatricial ROPCicatricial ROP– DysplasiaDysplasia– ColobomaColoboma– Pigmentary retinopathyPigmentary retinopathy

The AlignmentThe Alignment

• Ortho, Ortho, EsoEso or or ExoExo? ?

In an infant:In an infant:

Eso - Infantile esotropia with LN/MLNEso - Infantile esotropia with LN/MLN

Nystagmus Compensation SyndromeNystagmus Compensation Syndrome

Exo – Infantile exo, Exo – Infantile exo,

many times with neuro-developmental many times with neuro-developmental issuesissues

The NystagmusThe Nystagmus

• Pendular or JerkPendular or Jerk• DirectionDirection• Frequency and AmplitudeFrequency and Amplitude• Variation with gazeVariation with gaze• Variation with convergenceVariation with convergence• Variation with monocular Variation with monocular

occlusionocclusion• Binocular symmetricBinocular symmetric• Binocular asymmetricBinocular asymmetric• MonocularMonocular

““How long” to “observe” ?How long” to “observe” ?• Single concentrated ‘effort’ of Single concentrated ‘effort’ of

observation of at least 3 minutes !!! observation of at least 3 minutes !!!

Periodic Alternating Periodic Alternating NystagmusNystagmus

Serious neurological disease?Serious neurological disease?

• Asymmetric Asymmetric nystagmusnystagmus

• Monocular Monocular nystagmusnystagmus– Visual pathway Visual pathway

disorders ! disorders !

• Vertical nystagmusVertical nystagmus

• Purely torsional Purely torsional nystagmusnystagmus

EvaluationEvaluationAsymmetric nystagmusAsymmetric nystagmus

INOINO

Spasmus nutansSpasmus nutans

Rarely Congenital nystagmusRarely Congenital nystagmus

Parasellar tumoursParasellar tumours

Restrictive or paralytic ocular muscular Restrictive or paralytic ocular muscular disordersdisorders

Congenital Idiopathic Congenital Idiopathic NystagmusNystagmus

• ObservationObservation– Most commonly horizontalMost commonly horizontal– Pendular or jerkPendular or jerk– Horizontal nystagmus in Horizontal nystagmus in

vertical gaze positions vertical gaze positions [ Uniplanar ][ Uniplanar ]

– Null position – Eccentric Null position – Eccentric or on near gazeor on near gaze

– Usually symmetricUsually symmetric– Fulcrum of rotation in Fulcrum of rotation in

“apparently” asymmetric “apparently” asymmetric nystagmus. nystagmus.

Congenital Idiopathic Congenital Idiopathic NystagmusNystagmus

• Typically 3 phases of development [ Dr. Typically 3 phases of development [ Dr. Robert Reinecke]Robert Reinecke]– Phase 1- Broad triangular wave form [ 3-6 mths]Phase 1- Broad triangular wave form [ 3-6 mths]– Phase 2- low amp pendular waveform [6-24 Phase 2- low amp pendular waveform [6-24

months]months]– Phase 3-Typical jerk nystagmus [24-36 months]Phase 3-Typical jerk nystagmus [24-36 months]

• Historically:Historically:– No oscillopsiaNo oscillopsia– Invariably improves with ageInvariably improves with age

Spasmus nutansSpasmus nutans

• Head noddingHead nodding

• Anomalous head positionAnomalous head position

• Monocular/asymmetric nystagmus – Monocular/asymmetric nystagmus –

“ “ Shimmering”Shimmering”

• RULE OUT CNS TUMOUR [ glioma ] RULE OUT CNS TUMOUR [ glioma ]

Latent nystagmus/ Manifest Latent nystagmus/ Manifest Latent NystagmusLatent Nystagmus

Probably the only cause of Infantile Probably the only cause of Infantile nystagmus which does not need nystagmus which does not need

Electrophysiologic study or Neuro imagingElectrophysiologic study or Neuro imaging

Latent nystagmusLatent nystagmus

• Beats away from Beats away from the covered eye the covered eye [ towards the fixing [ towards the fixing eye ] eye ]

Anomalous Head PositionAnomalous Head Position

• Null point Null point – Beware PANBeware PAN– Wandering Null point Wandering Null point

• Usually in an eccentric gaze positionUsually in an eccentric gaze position• Head is positioned AWAY from the null Head is positioned AWAY from the null

pointpoint– i.e. Null point to left, face turn to righti.e. Null point to left, face turn to right

• Mostly lateral turn, occasionally Mostly lateral turn, occasionally vertical and cyclovertical head turnsvertical and cyclovertical head turns

ElectrophysiologyElectrophysiology

• ERG, EOG and VERERG, EOG and VER

• Would probably be indicated in most Would probably be indicated in most situations as an initial ‘workup’situations as an initial ‘workup’

• May allow to avoid neuroimagingMay allow to avoid neuroimaging

Neuro imagingNeuro imaging

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

TREATMENTTREATMENT

• Drug treatmentDrug treatment

• Optical treatmentOptical treatment

• ChemodenervationChemodenervation

• Surgical treatmentSurgical treatment

Drug Therapy - SpecificDrug Therapy - Specific

• Pendular Nystagmus – Gabapentin Pendular Nystagmus – Gabapentin and Memantineand Memantine

• PAN – BaclofenPAN – Baclofen

• Superior Oblique Myokymia – Superior Oblique Myokymia – Carbemazipine, GabapentinCarbemazipine, Gabapentin

Drug Therapy – Less specificDrug Therapy – Less specific

• Pendular – Valproate, Pendular – Valproate, Trihexyphenidyl, Isoniazid, CannabisTrihexyphenidyl, Isoniazid, Cannabis

• Downbeat nystagmus – 3,4 Downbeat nystagmus – 3,4 diaminopyridine, 4 aminopyridine, diaminopyridine, 4 aminopyridine, gabapentin, clonazepam, baclofengabapentin, clonazepam, baclofen

• Any form of Nystagmus – Any form of Nystagmus – Clonazepam, baclofen Clonazepam, baclofen

Optical treatmentOptical treatment

CORRECT REFRACTIVE ERRORCORRECT REFRACTIVE ERROR

Refraction in nystagmusRefraction in nystagmus

1.1. Binocular UCVA in forced ppBinocular UCVA in forced pp

2.2. Binocular UCVA in preferred AHPBinocular UCVA in preferred AHP

Refraction in nystagmusRefraction in nystagmus

1.1. Binocular retinoscopy with patient fixing Binocular retinoscopy with patient fixing either in AHP or forced PPeither in AHP or forced PP

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

2.2. Subjectively refract other eye Subjectively refract other eye

3.3. Repeat on the other sideRepeat on the other side

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

5.5. Repeat all steps with cycloplegiaRepeat all steps with cycloplegia

Factors which can be Factors which can be improvedimproved• Visual acuityVisual acuity

– VA, contrast sensitivity, colour, motion VA, contrast sensitivity, colour, motion sensitivity, gaze anglesensitivity, gaze angle

• Anomalous Head PositionAnomalous Head Position– Congenital nystagmus, acquired nystagmus, Congenital nystagmus, acquired nystagmus,

convergence damping, adduction null in LN/MLNconvergence damping, adduction null in LN/MLN• OscillopsiaOscillopsia

– Acquired nystagmus, decompensated Acquired nystagmus, decompensated congenital nystagmuscongenital nystagmus

• Hypo accommodationHypo accommodation• PhotophobiaPhotophobia

Refractive CorrectionRefractive Correction

• In children upto 10 years, full In children upto 10 years, full cycloplegic refractioncycloplegic refraction

• In adults, subjective, try to push over In adults, subjective, try to push over time if there is a difference in sub time if there is a difference in sub and obj refractionand obj refraction

Amblyopia therapyAmblyopia therapy

• May significantly decrease or May significantly decrease or eliminate MLN …… LN eliminate MLN …… LN

• Periods of occlusion have to be very Periods of occlusion have to be very prolonged in patients with LNprolonged in patients with LN

• Alternatively fogging or penalisation Alternatively fogging or penalisation may have to be used may have to be used


• To direct the null point centrallyTo direct the null point centrally– Prisms placed with apex directed Prisms placed with apex directed

towards the null point. towards the null point. – Large power prisms may have to be Large power prisms may have to be

used. used. – FresnelsFresnels– May degrade visionMay degrade vision


• To stabilize visual image on the To stabilize visual image on the retinaretina– High plus spectacle with high minus High plus spectacle with high minus

contact lens[ -58 & +32 ] contact lens[ -58 & +32 ] – Entire 30 deg field focussed to centre of Entire 30 deg field focussed to centre of

eye, and CL refocuses to the retina. eye, and CL refocuses to the retina. – Image remains stable irrespective of eye Image remains stable irrespective of eye

movement !! movement !!


• To induce convergenceTo induce convergence– Base out prisms bilaterallyBase out prisms bilaterally– Induce a convergenceInduce a convergence– Useful only if there is a convergence nullUseful only if there is a convergence null– May have to compensate with a -1.0 sph May have to compensate with a -1.0 sph

for induced accommodationfor induced accommodation

ChemodenervationChemodenervation

• BotoxBotox – 2.5 – 5 units into all horizontal recti2.5 – 5 units into all horizontal recti

– Retrobulbar injection of 25 – 30 unitsRetrobulbar injection of 25 – 30 units


• Useful to reduce amplitude of nystagmusUseful to reduce amplitude of nystagmus

• Has been shown to improve foveation time Has been shown to improve foveation time and improve visual acuity slightly. and improve visual acuity slightly.

• More useful in neurological acquired More useful in neurological acquired nystagmus, particularly in oculopalatal nystagmus, particularly in oculopalatal myoclonusmyoclonus

• RB injection effect lasts for several weeks RB injection effect lasts for several weeks


• Complications includeComplications include– PtosisPtosis– DiplopiaDiplopia– Filamentary keratitisFilamentary keratitis

Electronystagmography

Nystagmovideography

Surgical principlesSurgical principles

• Decrease the amplitude of nystagmusDecrease the amplitude of nystagmus– Maximal recession of horizontal musclesMaximal recession of horizontal muscles– TenotomyTenotomy

• Increase foveation timeIncrease foveation time– Tenotomy Tenotomy

• Broaden the null zoneBroaden the null zone

• Rotate the null zoneRotate the null zone– AndersonAnderson– GotoGoto– KestenbaumKestenbaum– Parks’ modification of KestenbaumParks’ modification of Kestenbaum– Augmented KestenbaumAugmented Kestenbaum

• 40%40%• 60%60%

• Induce an attempt to convergeInduce an attempt to converge– Artificial divergence surgeryArtificial divergence surgery

Surgery to correct AHPSurgery to correct AHP

Face turns - horizontalFace turns - horizontal• Anderson advocated bilateral recessionAnderson advocated bilateral recession

– Eg. Null zone to left, weaken levo- ‘verters’ Eg. Null zone to left, weaken levo- ‘verters’

• Kestenbaum advocated recess-recess Kestenbaum advocated recess-recess [ pull and push][ pull and push]

• Park’s modification of Kestenbaum’sPark’s modification of Kestenbaum’s– 5-6-7-8 rule [both eyes get 13 mm ]5-6-7-8 rule [both eyes get 13 mm ]

•Very rarely corrects more than 10 -15 degreesVery rarely corrects more than 10 -15 degrees


• Augmented K-A procedureAugmented K-A procedure– Classic + 40% - For > 30 deg of face Classic + 40% - For > 30 deg of face

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

• ProblemsProblems– Intractable diplopia Intractable diplopia


Vertical AHPVertical AHP– Chin upChin up

• IR recess – SR resectIR recess – SR resect

– Chin downChin down• IR resect– SR recess IR resect– SR recess

•Anteriorisation of IOAnteriorisation 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, 587–592. doi:10.1038/sj.eye.6700431

http://www.nature.com/eye/journal/v17/n5/full/6700431a.html#aff1




Cyclovertical AHPCyclovertical AHP

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

‘created’ by the patient’s head tilt‘created’ by the patient’s head tilt• Several methodsSeveral methods

– Strengthen or weaken obliquesStrengthen or weaken obliques– Slanting recti insertionsSlanting recti insertions– Vertical recti slantingVertical recti slanting

SurgerySurgery

• Other problemsOther problems– Management of co existent strabismus Management of co existent strabismus

with nystagmuswith nystagmus– Acquiring of a new head position - PANAcquiring of a new head position - PAN– Creating a new strabismusCreating a new strabismus

Surgery primarily designed to Surgery primarily designed to improve visionimprove vision

• Artificial divergenceArtificial divergence– Bimedial recession Bimedial recession – Unilateral recess-resect to XTUnilateral recess-resect to XT

• 4 – muscle retro equatorial recession4 – muscle retro equatorial recession– 10 mm MR and 12 mm LR10 mm MR and 12 mm LR– Ideal for PANIdeal for PAN– May induce an exotropiaMay induce an exotropia

Dell’Osso & HertleDell’Osso & Hertle

• Based on the principle of enthesial Based on the principle of enthesial proprioceptive input to nystagmus at proprioceptive input to nystagmus at the insertion of the horizontal rectithe insertion of the horizontal recti

• Dell'Osso LF. Extraocular muscle tenotomy, dissection, and suture: A hypothetical Dell'Osso LF. Extraocular muscle tenotomy, dissection, and suture: A hypothetical therapy for congenital nystagmustherapy for congenital nystagmus. J Pediatr Ophthalmol Strab 1998; 35:232-3. . J Pediatr Ophthalmol Strab 1998; 35:232-3.

• Hertle RW, Dell'Osso LF, FitzGibbon EJ, Thompson D, Yang D, Mellow SD. Horizontal Hertle RW, Dell'Osso LF, FitzGibbon EJ, Thompson D, Yang D, Mellow SD. Horizontal rectus tenotomy in patients with congenital nystagmus. Results in 10 adultsrectus tenotomy in patients with congenital nystagmus. Results in 10 adults. . Ophthalmology 2003; 110:2097-105.Ophthalmology 2003; 110:2097-105.

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

SummarySummary

• Evaluation of nystagmus is Evaluation of nystagmus is multidisciplinary multidisciplinary

• However, it is possible to improve However, it is possible to improve the quality of life with drugs/optical the quality of life with drugs/optical devices/surgical proceduresdevices/surgical procedures

• No single procedure has shown to be No single procedure has shown to be consistently predictive of successconsistently predictive of success

• This does not mean we cannot try. This does not mean we cannot try.

Thank you

Documents

Management of Nystagmus – the Ophthalmologist’s perspective