2014/12/17

Overviewofnystagmus

OfficialreprintfromUpToDate
www.uptodate.com2014UpToDate
Overviewofnystagmus
Author
JasonJSBarton,MD,PhD,
FRCPC

SectionEditor
PaulWBrazis,MD

DeputyEditor
JanetLWilterdink,MD

Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Nov2014.|Thistopiclastupdated:Oct31,2014.
INTRODUCTIONNystagmusisarhythmicregularoscillationoftheeyes.Itmayconsistofalternating
phasesofaslowdriftinonedirectionwithacorrectivequick"jerk"intheoppositedirection,orofslow,
sinusoidal,"pendular"oscillationstoandfro.Jerknystagmusismorecommonthanpendularnystagmus.
(See"Jerknystagmus"and"Pendularnystagmus".)
Nystagmuscanbecontinuousorparoxysmal,orevokedbycertainmaneuverssuchasspecificgazeor
headpositions.Nystagmusassociatedwithsomepathologiesmayonlybeseentransientlywith
hyperventilationorcoughingandsneezing.
Anoverviewofnystagmus,itstreatment,andthevestibularphysiologyrelevanttonystagmusandvertigois
presentedhere.Theapproachtovertigoisdiscussedseparately.(See"Evaluationofthepatientwith
vertigo".)
TYPESOFNYSTAGMUSThetwomajortypesofnystagmusarejerknystagmusandpendular
nystagmus.
JerknystagmusJerknystagmusissubdividedbytrajectoryandtheconditionsunderwhichitoccurs
(table1).Someformsarealwayspresent,evenwhentheeyesareintheprimaryposition.Nystagmusinthe
primarypositionisclassifiedaccordingtotrajectory:

downbeat
upbeat
horizontal
torsional
mixed

Thedirectionnamedisthedirectionofthefastphase.
Otherformsemergeonlyunderspecificconditionssuchasperipheralgaze(gazeevoked)andcertainhead
positions(positional).(See"Jerknystagmus".)
Mostacquiredjerknystagmusaretheresultofanasymmetryinvestibularinputs,ineitherthecentralor
peripheralnervoussystem.Understandingthediagnosticvalueofthetrajectoryofjerknystagmusrequires
someknowledgeofthephysiologyofthesemicircularcanals(seebelow).
PendularnystagmusPendularnystagmushasasinusoidaloscillationwithoutfastphases.The
waveformofpendularnystagmusmayoccurinanydirectionitcanbetorsional,horizontal,vertical,ora
combinationofthese,resultingincircular,oblique,orellipticaltrajectories.Itcanbedifferentinthetwoeyes,
sometimesevenmonocular.Avideodemonstratingpendularnystagmusisavailableat
http://www.neuroophthalmology.ca/caseofthemonth/eyemovements/monocularblurryvisioninmultiple
sclerosis.
Thediagnosisofpendularnystagmusisanexerciseinpatternrecognition,sincependularnystagmusis
subdividedintoanumberoftypesrecognizedmainlybyveryspecifictrajectories.Thesearedescribedin
detailseparately.(see"Pendularnystagmus"):
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Acquiredpendularnystagmus
Congenitalnystagmus
Pendularnystagmuswithvisualloss
Specificvariantsinclude:
spasmusnutans
oculopalatalmyoclonus
seesawnystagmus
oculomasticatorymyorhythmia(Whipple's)
BASICCLINICALVESTIBULARPHYSIOLOGY
ThevestibularsensoryorgansTherearetwocomponentstotheperipheralvestibularsystem:
Theotolithicorgans
Thesemicircularcanals
Theotolithicorgansarethesacculeandtheutricle,eachofwhichhasaplaneofhaircellsembeddedina
heavyslabofmaterialcontainingcalcium.Theseslabshaveasignificantamountofinertia.Anylinear
accelerationofthehead(eg,gravity)causestheheadtomoverelativetotheotoconia,resultingina
bendingofthehaircellsandachangeinneuronalactivity.
Thesemicircularcanalsdetectangularaccelerations(turns,orrotations)ofthehead.Eachcanalcontains
fluidandagelatinousstructurecalledtheampulla,inwhichhaircellsareembedded.Rotationofthehead
causesthecanaltomoverelativetothefluidwithinit,therebygeneratingaforceagainsttheampullathat
bendsthehaircells.Anincreaseordecreasefromthebaselinefiringrateoccursdependinguponthe
directionofrotation.
Therearesixcanals,twoperside,arrangedalongthreecardinalplanes.Thetwohorizontalcanalslieinone
plane,notquitehorizontalbuttilteddownward30degrees.Thevertical(anteriorandposterior)canalsareall
atapproximatelyrightanglestothehorizontalplane,butpositionedintwoplanes45degreesfromthe
sagittalplane.Thus,eachplanehasapairofcanals,theanteriorcanalofonesidepairedwiththeposterior
canaloftheotherside.
Thevestibularsystemusesthesepairsofcanalsina"pushpull"reciprocalfashion.Anangularmovementof
theheadalongoneoftheseplaneswillmaximallyactivateonecanalandmaximallyinhibititscounterpart.
Thus,thenormalrestingfiringrateofthenervefromonecanalincreaseswhilethatofitscounterpart
decreases.Thenetchangeinbothdirectionsissummedalgebraicallybythebraintorevealthechangein
headposition.Thisinformationistransmittedtotheocularmotornuclei,causingtheeyestomovesmoothly
anequalandoppositeamounttotheheadturn(thevestibuloocularreflex),enablingtheeyestoremain
stationaryinspacedespitetheheadmovement.
Thedirectionsthatexciteeachcanalaregivenbyasimplerule:"acanalisexcitedbyheadmotiontowards
thecanal,intheappropriateplane."Thus,therighthorizontalcanalisexcitedbyrightheadturns,theright
posteriorcanalisexcitedbyrightheadtiltsor"posterior"headtilts(neckextension),andtheleftanterior
canalisexcitedbyleftheadtiltsor"anterior"headtilts(neckflexion).
VestibuloocularresponsesThevestibuloocularreflexkeepsthelineofsightstableinspacewhilethe
headismoving.Thus,itusesinformationaboutheadrotationtodrivetheeyesanequalamountinthe
oppositedirection.Toaccomplishthis,eachsemicircularcanalhasexcitatoryprojectionstoapairof
extraocularmuscles,oneineacheye,andinhibitoryprojectionstoanantagonisticpair.Themuscles
maximallyactivatedbyacanalareapproximatelyalignedwiththeplaneofthatcanal.
Tounderstandtheeffectsofsuchactivationrequiresknowledgeofthedirectionofpullofeachextraocular
muscle.Thehorizontalrectiaresimple:thelateralrectiabductandthemedialrectiadducttheeye.The
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verticalrectiandobliquesaremorecomplex:theyallhaveprimary,secondary,andtertiaryactionsuponthe
globe(table2).Itisusefultothinkofthelineofpullofthesemusclesasapproximatelyalignedwiththe
planesoftheverticalsemicircularcanals.Whilethelineofpullremainsroughlyconstant,theeffectofthe
pullupontheeyeinpartdependsuponwhethertheeyeisabductedoradductedatthetime.
Contractionofthesuperiorrectuselevatestheeye,especiallywhenitisintheabductedposition.
Whentheeyeisadducted,however,thisstrongprimaryactionofthemuscleislessandthereisalso
moreofanintorsionalmotion.
Contractionoftheinferiorrectusleadstotheoppositepattern:depressionoftheeyeinabductionwith
someextorsioninadduction.
Contractionofthesuperiorobliquecausesmainlyintorsionoftheeye,asitpullsthetopoftheeye
towardsthepulleyattheanteromedialcorneroftheroofoftheorbit.Thisismaximalinabduction.
Whentheeyeisadducted,however,thepullofthetopoftheeyetowardthepulleynowcauses
depression.
Contractionoftheinferioroblique,whoseoriginisattheanteromedialcorneroftheflooroftheorbit,
leadstotheoppositepattern,extorsionoftheeyeinabduction,withelevationinadduction.
Withthesedataitiseasytoseewhichpairofmusclesisactivated(orinhibited)byeachsemicircularcanal
tokeepthelineofsightsteadywithheadturns(table3).
GenerationofnystagmusUnderstandingtheplanesofactionandocularactivationsofthesemicircular
canalsandtheirpushpullrelationshipwithopposingcanalsontheothersideisessentialtounderstanding
howjerknystagmusisgenerated.Withalesionoftherighthorizontalcanal,forexample,thereisalossof
tonicleftwardvestibularinputsincethereisalwaysabaselinelevelofactivity.Theunopposedrightward
"push"fromtheintactlefthorizontalcanalcreatesaslowrightwarddriftoftheeyes,the"slowphase,"which
iseventuallycounteractedbyaleftwardfastphase.Thus,lossoftherighthorizontalcanalcreatesleft
beatingnystagmus.
Similarly,lossoftherightanteriorcanalcreatesanystagmusthatisamixofupbeatandcounterclockwise
torsionalfastphases,andlossoftherightposteriorcanalcreatesadownbeat/counterclockwisetorsional
nystagmus.Ifallthreecanalsontherightsidearelost,theupbeatanddownbeatcomponentscanceleach
otherout,butthecounterclockwisetorsionaleffectaddtogetherandsummatewiththeleftbeatinghorizontal
nystagmustocreateamixedhorizontaltorsionalnystagmus.Thisisthetypicalfindingwith"peripheral
nystagmus,"thatis,nystagmusfromacuteunilateraldamagetothelabyrinthorvestibularnerve.
Thisalsoexplainswhypureverticalnystagmusisrarelyifevergeneratedbyperipheralvestibulardisease.
Toobtainpuredownbeatnystagmus,alesionwouldhavetoaffectthetwoposteriorcanals,summatingthe
resultinglossofupwardslowdrifts,cancelingtheopposingtorsionaldrifts,andsparingallothercanals.The
oddsareagainstsuchaselectiveyetbilaterallesionofthelabyrinth.Thesameholdsforpureupbeat
nystagmus.Ontheotherhand,thecentralvestibularpathwaysinthebrainstemareorganizedsothatsuch
deficitsarepossible.
Puretorsionalnystagmusconceivablycouldresultfromalesionthataffectedboththeanteriorandposterior
canalononeside,sparingthehorizontalcanal.However,theregionalbloodsupplyandnervesubdivisions
ofthelabyrinthsegregatetheanteriorcanalandhorizontalcanalfromtheposteriorcanal.Clinicalstudies
suggestthatvestibularneuronitis,forexample,isapartialdefectwhosenystagmussuggestseithera
combinationofhorizontalandanteriorcanaldysfunction,oralmostpurehorizontalcanalloss.Thus,partial
lesionsoftheperipheryareunlikelytocausepuretorsionalnystagmus.Purehorizontalnystagmus,
however,couldbeeithercentralorperipheral.
Somenystagmusistheresultofhyperfunctionofacanalratherthanhypofunctionofonelabyrinth.The
classicexampleisbenignparoxysmalpositionalvertigo.Movementofdebrisintheposteriorcanalleadsto
abnormalactivation,generatingslowdownwardandtorsionaldrifts,andthereforeamixedupbeat/torsional
nystagmus.(See"Benignparoxysmalpositionalvertigo".)
SYMPTOMSVertigoistheprimarysymptomassociatedwithnystagmusandismostprominentwith
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acuteunilateralperipheralvestibulardisease.Othersymptomsassociatedwithnystagmusinclude:
OscillopsiaOscillopsiaisatoandfroillusionofenvironmentalmotion.Dependinguponthe
nystagmus,thismaybecontinuous,intermittent,orgazeevoked.Thiscontrastswiththeoscillopsia
frombilateralvestibularhypofunction,whichonlyoccurswhentheheadismoving,aswhenwalkingor
ridinginacar.
BlurredvisionBlurredvisionoccursbecausetheretinalimageissmearedbystimulusmotion.
AbnormalheadpositionsPatientswhofindthattheiroscillopsiaorblurredvisionisleast
troublesomeincertaingazepositionsthatminimizenystagmusmayassumeabnormalheadpositions.
Somepatientswithnystagmusareasymptomatic.
DIFFERENTIALDIAGNOSISTherhythmicnatureandslowspeedsofnystagmusdistinguishitfroma
numberofotherabnormalinvoluntaryeyemovements.
Saccadicintrusionsareirregularburstsofrapideyemovements,almostalwaysindicatingaproblemof
themidlinecerebellum.(See"Overviewofcerebellarataxiainadults",sectionon'Clinicalandanatomic
correlations'.)
Thesemovementsincludeopsoclonus,ocularflutter,squarewavejerks,andmacrosaccadic
oscillations.Voluntarynystagmusisamisnomerittooisatypeofsaccadicintrusion,whichmost
resemblesocularflutter.
Ocularbobbinganditsvariantshaveslowdriftsandfastphasesbutaremuchmoreirregularthan
nystagmus.Theyoccurincomatoseandlockedinindividuals.(See"Stuporandcomainadults",
sectionon'Eyemovements'and"Lockedinsyndrome",sectionon'Clinicalfeatures'.)
Oculogyriccrisesareirregularprolongeddeviationsoftheeyes,usuallyupandlateral,thatlack
rhythmicityandslowphases.Theyaremostfrequentlyencounteredwithphenothiazineintoxication.
(See"Classificationandevaluationofdystonia",sectionon'Clinicalfeatures'.)
SYMPTOMATICTHERAPYThegeneralapproachtonystagmustherapyinvolvesthreebasic
considerations[1,2]:
Whatisthetypeofnystagmus?
Aretheretreatableunderlyingcauses?Considerintoxications,metabolicderangements,infections,
andoperablestructurallesions.
Whatisthepotentialbenefitofsymptomatictreatment?
Thegoalsofimprovementinthesymptomslistedabove(vertigo,oscillopsia,motiondegradedacuity,or
headturn)shouldbeclearpriortotreatment.Patientswithoutthesesymptomsdonotrequiresymptomatic
treatment.
Therearefourmaintypesofsymptomaticnystagmustherapy:

Medication
Botulinuminjections
Prismlensesandopticalsolutions
Surgery

MedicationAppropriatemedicationusedependsuponthetypeofnystagmus.(See"Pendular
nystagmus"and"Jerknystagmus".)
BotulinuminjectionsBotulinuminjectionsintomuscleortheintraconalspacehavebeenusedto
weakentheextraocularmusclesanddiminishtheamplitudeofnystagmus.Theseimprovevision,
oscillopsia,andnystagmussomewhat[35],buttheyalsoweakennormaleyemovements.Insomereports
themosttellingdetailisthatpatientshavedeclinedtorepeattheexperience[4,6].
PrismlensesandopticalsolutionsPrismlensesandopticalsolutionsmaybeusefulinthefollowing
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circumstances:
Ifpatientshaveapositionofgazeinwhichnystagmusisminimal,prismscanbeusedsothattheeyes
areinthis"nullposition"whenthepatientistryingtolookdirectlyahead.Oscillopsiaisthereby
minimizedforthismostfrequentgazeposition,butgazetothesidewillstillbringonsymptoms.
Ifconvergencedampensnystagmus,thepatientcanwearprismsthatmaketheeyesmoreconvergent
whilefixatinginthedistance[7,8].
Highhyperopicglassescanbecombinedwithhighmyopiccontactlensestocreateasituationinwhich
theretinalimagedoesnotshiftwitheyemovement[9,10].Thisisusefulforreading,butthefieldof
viewnarrowstoatunnel,andthedesirableeffectsofnormaleyemovementsareimpairedinthatthey
donotshiftorsteadygazenormally.Thus,thesystemcannotbewornwhilewalkingaround.Most
patientsfindthedevicecumbersome.
Contactlensescandampencongenitalnystagmusforunknownreasons[11].
SurgeryMostsurgerytriestoshifttheattachmentofthemusclessothatthemuscletensionsappropriate
toagazepositionwherenystagmuswasminimalorabsentnowplacetheeyesincentergaze.Inessence,
thisissimilartothestrategyofprismtherapyand,aswithprisms,oscillopsiawillstillbepresentinlateral
gaze.Surgeryhasbeenusedmainlywithcongenitalnystagmus[1214],whereithasbeenreportedto
improvevisualacuityinsomecases[12].
SUMMARYNystagmusisarhythmicregularoscillationoftheeyes.Therhythmicnatureandslowspeeds
ofnystagmusdistinguishitfromanumberofotherabnormalinvoluntaryeyemovements.
Jerknystagmusoccursmostoftenwhenthereisanimbalanceintheactivationofthesemicircular
canalsbecauseofeitherperipheralvestibulardiseaseordisruptionofcentralvestibularpathwaysin
thebrainstem(See'Generationofnystagmus'above.)
Nystagmusmaybeassociatedwiththeclinicalsymptomsofvertigo,oscillopsia,abnormalhead
position,and/orblurredvision(See'Symptoms'above.)
Jerknystagmusconsistsofalternatingphasesofaslowdriftinonedirectionwithacorrectivequick
"jerk"intheoppositedirection.Itisfurtherclassifiedaccordingtotrajectoryandtheconditionsunder
whichitoccurs(table1)(See"Jerknystagmus".)
Pendularnystagmusconsistsofslow,sinusoidal,"pendular"oscillationstoandfrosubdividedintoa
numberoftypesrecognizedmainlybyveryspecifictrajectories(eg,acquiredpendularnystagmus,
congenitalnystagmus,etc.)(See"Pendularnystagmus".)
Treatmentissymptomatic.Appropriatemedicationusedependsuponthetypeofnystagmus.Prism
lensesandotheropticalsolutionsandocularsurgerymaybehelpfulinspecificcircumstances(See
'Symptomatictherapy'above.)
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GRAPHICS
Classificationofjerknystagmus
Primaryposition
Puredirections
Upbeat
Downbeat
Torsional
Horizontal
Peripheralvestibular
Congenital
Periodicalternating
Latent
Epileptic
Mixeddirections(peripheralvestibular)
Horizontal/torsional
Vertical/torsional

Gazeevoked
Gazeholding
Rebound
Peripheralvestibular
Brun's

Positional
Paroxysmalpositional
Posteriorcanalithiasis
Anteriorcanalithiasis
Horizontalcanalithiasis
Staticpositional

Dissociated
Internuclearophthalmoplegia

Other
Convergenceretractionnystagmus
Graphic72446Version3.0

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Functionsoftheverticalandobliqueextraocularmuscles
Functions

Superior
rectus

Inferior
rectus

Superior
oblique

Inferior
oblique

Primary

Elevation

Depression

Incyclotorsion

Excyclotorsion

Secondary

Incyclotorsion

Excyclotorsion

Depression

Elevation

Tertiary

Adduction

Adduction

Abduction

Abduction

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Thecanaliculoocularreflexes
Canal

Preferredhead
motion

Eyemuscles
excited

Right
horizontal

Rightturn

Right

Neckflexion

Rightsuperiorrectus

Elevation(rightgaze)

Rightheadtilt

Leftinferioroblique

Counterclockwisetorsion
(leftgaze)

Neckextension

Leftinferiorrectus

Depression(leftgaze)

Rightheadtilt

Rightsuperior

Counterclockwisetorsion

oblique

(rightgaze)

Rightlateralrectus

Rightward

anterior

Right
posterior

Leftlateralrectus

Resultingeyemotion
Leftward

Rightmedialrectus

Left
horizontal

Leftturn

Left
anterior

Neckflexion

Leftsuperiorrectus

Elevation(leftgaze)

Leftheadtilt

Rightinferioroblique

Clockwisetorsion(right

Leftmedialrectus

gaze)

Left
posterior

Neckextension

Rightinferiorrectus

Depression(rightgaze)

Leftheadtilt

Leftsuperioroblique

Clockwisetorsion(leftgaze)

Headandeyerotationsaredescribedfromthepointofviewofthepatient.Asexamples,a
rightheadtiltisaclockwiserotationoftheheadcounterclockwisetorsionoftheeyesmeans
thatthesuperiorpoleoftheeyesaretiltedtotheleft.
Rightgaze:maximallyinrightgazeleftgaze:maximallyinleftgaze.
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Disclosures
Disclosures:JasonJSBarton,MD,PhD,FRCPCNothingtodisclose.PaulWBrazis,MDNothingtodisclose.JanetL
Wilterdink,MDEmployeeofUpToDate,Inc.
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseareaddressedby
vettingthroughamultilevelreviewprocess,andthroughrequirementsforreferencestobeprovidedtosupportthe
content.AppropriatelyreferencedcontentisrequiredofallauthorsandmustconformtoUpToDatestandardsof
evidence.
Conflictofinterestpolicy

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