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Nystagmus

From Wikipedia, the free encyclopedia

Nystagmus
Other namesDancing eyes,Instabilitas oculorum
Horizontaloptokinetic nystagmus,a normal (physiological) form of nystagmus
SpecialtyNeurology,ophthalmology,optometry

Nystagmusis a condition of involuntary (or voluntary, in some cases)[1]eye movement.[2]People can be born with it but more commonly acquire it in infancy or later in life. In many cases it may result inreduced or limited vision.[3]

In normal eyesight, while theheadrotates about anaxis,distant visual images are sustained by rotating eyes in the opposite direction of the respective axis.[4]Thesemicircular canalsin thevestibule of the earsense angular acceleration, and send signals to the nuclei for eye movement in the brain. From here, a signal is relayed to theextraocular musclesto allow one's gaze to fix on an object as the head moves. Nystagmus occurs when thesemicircular canalsare stimulated (e.g., by means of thecaloric test,or by disease) while the head is stationary. The direction of ocular movement is related to the semicircular canal that is being stimulated.[5]

There are two key forms of nystagmus:pathologicalandphysiological,with variations within each type. Physiological nystagmus occurs under normal conditions in healthy subjects. Nystagmus may be caused bycongenital disorderorsleep deprivation,acquired orcentral nervous systemdisorders,toxicity,pharmaceutical drugs,alcohol,or rotational movement. Previously considered untreatable, in recent years several drugs have been identified for treatment of nystagmus.[6]Nystagmus is also occasionally associated withvertigo.

Causes

[edit]
Nystagmus as seen in a case ofocular albinism

The cause of pathological nystagmus may becongenital,idiopathic,or secondary to a pre-existingneurological disorder.It also may be induced temporarily bydisorientation(such as on roller coaster rides or when a person has been spinning in circles) or by somedrugs(alcohol,lidocaine,and othercentral nervous systemdepressants, inhalant drugs,stimulants,psychedelics,anddissociativedrugs).

Early-onset nystagmus

[edit]

Early-onset nystagmus occurs more frequently than acquired nystagmus. It can be insular or accompany other disorders (such as micro-ophthalmic anomalies orDown syndrome). Early-onset nystagmus itself is usually mild and non-progressive. The affected persons are usually unaware of their spontaneous eye movements, but vision can be impaired depending on the severity of the eye movements.

Types of early-onset nystagmus include the following, along with some of their causes:

X-linked infantile nystagmus is associated with mutations of the geneFRMD7,which is located on theX chromosome.[7][8]

Infantile nystagmus is also associated with two X-linked eye diseases known as complete congenital stationary night blindness (CSNB) and incomplete CSNB (iCSNB or CSNB-2), which are caused by mutations of one of two genes located on the X chromosome. In CSNB, mutations are found in NYX (nyctalopin).[9][10]CSNB-2 involves mutations of CACNA1F, avoltage-gated calcium channelthat, when mutated, does not conduct ions.[11]

Acquired nystagmus

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Nystagmus that occurs later in childhood or in adulthood is called acquired nystagmus. The cause is often unknown, oridiopathic,and thus referred to as idiopathic nystagmus. Other common causes include diseases and disorders of the central nervous system,metabolic disordersand alcohol and drug toxicity. In the elderly,strokeis the most common cause.

General diseases and conditions

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Some of the diseases that present nystagmus as a pathological sign or symptom are as follows:

Toxicity or intoxication, metabolic disorders and combination

[edit]

Sources of toxicity that could lead to nystagmus:

Thiamine deficiency

[edit]

Risk factors forthiamine deficiency,or beriberi, in turn include a diet of mostlywhite rice,as well asalcoholism,dialysis,chronicdiarrhea,and taking high doses ofdiuretics.[14][15]Rarely it may be due to ageneticcondition that results in difficulties absorbing thiamine found in food.[14]Wernicke encephalopathyandKorsakoff syndromeare forms of dry beriberi.[15]

Central nervous system (CNS) diseases and disorders

[edit]

Central nervous systemdisorders such as with acerebellarproblem, the nystagmus can be in any directionincludinghorizontal. Purely vertical nystagmus usually originates in the central nervous system, but it is also an adverse effect commonly seen in highphenytointoxicity. Other causes of toxicity that may result in nystagmus include:

Other causes

[edit]

Diagnosis

[edit]
Fast-phase horizontal eye movement vision
Fast-phase vertical eye movement vision

Nystagmus is highly noticeable but rarely recognized. Nystagmus can be clinically investigated by using a number ofnon-invasivestandard tests. The simplest one is thecaloric reflex test,in which oneear canalis irrigated with warm or cold water or air. The temperature gradient provokes the stimulation of thehorizontal semicircular canaland the consequent nystagmus. Nystagmus is often very commonly present withChiari malformation.

The resulting movement of the eyes may be recorded and quantified by a special device called anelectronystagmograph(ENG), a form ofelectrooculography(an electrical method of measuring eye movements using externalelectrodes),[22]or an even less invasive device called avideonystagmograph(VNG),[23]a form ofvideo-oculography(VOG) (a video-based method of measuring eye movements using external small cameras built into head masks), administered by anaudiologist.Special swinging chairs with electrical controls can be used to induce rotatory nystagmus.[24]

Over the past forty years, objective eye-movement-recording techniques have been applied to the study of nystagmus, and the results have led to greater accuracy of measurement and understanding of the condition.

Orthoptistsmay also use anoptokinetic drum,orelectrooculographyorFrenzel gogglesto assess a patient's eye movements.

Nystagmus can be caused by subsequentfoveationof moving objects,pathology,sustained rotation orsubstance use.Nystagmus is not to be confused with other superficially similar-appearing disorders of eye movements (saccadicoscillations) such asopsoclonusorocular flutterthat are composed purely of fast-phase (saccadic) eye movements, while nystagmus is characterized by the combination of a smooth pursuit, which usually acts to take the eye off the point of focus, interspersed with the saccadic movement that serves to bring the eye back on target. Without the use of objective recording techniques, it may be very difficult to distinguish among these conditions.

Inmedicine,the presence of nystagmus can bebenign,or it can indicate an underlying visual orneurological problem.[25]

Pathological nystagmus

[edit]

Pathological nystagmus is characterized by "excessive drifts of stationary retinal images that degrades vision and may produce illusory motion of the seen world: oscillopsia (an exception is congenital nystagmus)".[26]

Bechterew's phenomenon was discovered byVladimir Bekhterevin 1883 in animal experiments.[27]Specifically, if one side of the vestibular system is damaged, then due to the lack of vestibular signal from that side, the animal behaves with nystagmus and vertigo. After a while, due tovestibular compensation,nystagmus and vertigo stops. However, if then the other vestibular system is damaged, then nystagmus and vertigo occurs in the opposite direction. This is an early evidence of sensorimotor adaptation in the brain. It is rarely reported in humans.[28]

When nystagmus occurs without fulfilling its normal function, it is pathologic (deviating from the healthy or normal condition). Pathological nystagmus is the result of damage to one or more components of thevestibular system,including thesemicircular canals,otolithorgans, and thevestibulocerebellum.[contradictory]

Pathological nystagmus generally causes a degree ofvision impairment,although the severity of such impairment varies widely. Also, manyblindpeople have nystagmus, which is one reason that some wear dark glasses.[29]

Variations

[edit]
  • Central nystagmusoccurs as a result of either normal or abnormal processes not related to the vestibular organ. For example, lesions of the midbrain or cerebellum can result in up- and down-beat nystagmus.
    • Gaze induced nystagmusoccurs or is exacerbated as a result of changing one's gaze toward or away from a particular side which has an affected central apparatus.[30]
  • Peripheral nystagmusoccurs as a result of either normal or diseased functional states of the vestibular system and may combine a rotational component with vertical or horizontal eye movements and may bespontaneous,positional,orevoked.
    • Positional nystagmusoccurs when a person's head is in a specific position.[31]An example of disease state in which this occurs isbenign paroxysmal positional vertigo(BPPV).
    • Post rotational nystagmusoccurs after an imbalance is created between a normal side and a diseased side by stimulation of the vestibular system by rapid shaking or rotation of the head.
    • Spontaneous nystagmusis nystagmus that occurs randomly, regardless of the position of the patient's head.

Physiological nystagmus

[edit]
A diagram of thecaloric reflex test,which tests thevestibulo-ocular reflex(VOR) by irrigating the ear canal with warm or cold water to induce physiological nystagmus.

Physiological nystagmus is a form of involuntaryeye movementthat is part of thevestibulo-ocular reflex(VOR), characterized by alternatingsmooth pursuitin one direction andsaccadic movementin the other direction.

Variations

[edit]

The direction of nystagmus is defined by the direction of its quick phase (e.g. aright-beating nystagmusis characterized by a rightward-moving quick phase, and aleft-beating nystagmusby a leftward-moving quick phase). Theoscillationsmay occur in the vertical,[32]horizontal ortorsionalplanes, or in any combination. The resulting nystagmus is often named as a gross description of the movement, e.g.downbeat nystagmus,upbeat nystagmus,seesaw nystagmus,periodic alternating nystagmus.

These descriptive names can be misleading, however, as many were assigned historically, solely on the basis of subjective clinical examination, which is not sufficient to determine the eyes' true trajectory.

  • Optokinetic(syn. opticokinetic)nystagmus:a nystagmus induced by looking at moving visual stimuli, such as moving horizontal or vertical lines, and/or stripes. For example, if one fixates on a stripe of a rotating drum with alternating black and white, the gaze retreats to fixate on a new stripe as the drum moves. This is first a rotation with the same angular velocity, then returns in asaccadein the opposite direction. The process proceeds indefinitely. This is optokinetic nystagmus, and is a source for understanding thefixation reflex.[33]
  • Postrotatory nystagmus:if one spins in a chair continuously and stops suddenly, the fast phase of nystagmus is in the opposite direction of rotation, known as the "post-rotatory nystagmus", while slow phase is in the direction of rotation.[34]

Treatment

[edit]

Congenital nystagmus has long been viewed as untreatable, but medications have been discovered that show promise in some patients. In 1980, researchers discovered that a drug calledbaclofencould stop periodic alternating nystagmus. Subsequently,gabapentin,an anticonvulsant, led to improvement in about half the patients who took it. Other drugs found to be effective against nystagmus in some patients includememantine,[35]levetiracetam,3,4-diaminopyridine (available in the US to eligible patients with downbeat nystagmus at no cost under an expanded access program[36][37]),4-aminopyridine,andacetazolamide.[38]Several therapeutic approaches, such ascontact lenses,[39]drugs,surgery,andlow visionrehabilitationhave also been proposed. For example, it has been proposed that mini-telescopic eyeglasses suppress nystagmus.[40]

Surgical treatment of congenital nystagmus is aimed at improving head posture, simulating artificial divergence, or weakening the horizontal recti muscles.[41]Clinical trials of a surgery to treat nystagmus (known astenotomy) concluded in 2001. Tenotomy is now being performed regularly at numerous centres around the world. The surgery aims to reduce the eye oscillations, which in turn tends to improve visual acuity.[42]

Acupuncturetests have produced conflicting evidence on its beneficial effects on the symptoms of nystagmus. Benefits have been seen in treatments in which acupuncture points of the neck were used, specifically points on thesternocleidomastoidmuscle.[43][44]Benefits of acupuncture for treatment of nystagmus include a reduction in frequency and decreased slow phase velocities, which led to an increase in foveation duration periods both during and after treatment.[44]By the standards ofevidence-based medicine,the quality of these studies is poor (for example, Ishikawa's study had sample size of six subjects, was unblinded, and lacked proper controls), and given high quality studies showing that acupuncture has no effect beyond placebo,[citation needed]the results of these studies have to be considered clinically irrelevant until higher quality studies are performed.

Physical or occupational therapy is also used to treat nystagmus. Treatment consists of learning strategies to compensate for the impaired system.[45]

ACochrane Reviewon interventions for eye movement disorders due to acquired brain injury, updated in June 2017, identified three studies of pharmacological interventions for acquired nystagmus but concluded that these studies provided insufficient evidence to guide treatment choices.[46]

Epidemiology

[edit]

Nystagmus is a relatively common clinical condition, affecting one in several thousand people. A survey conducted in Oxfordshire, United Kingdom, found that by the age of two, one in every 670 children had manifested nystagmus.[3]Authors of another study in the United Kingdom estimated an incidence of 24 in 10,000 (c. 0.240%), noting an apparently higher rate amongst white Europeans than in individuals of Asian origin.[47]

Law enforcement

[edit]
Main article:Field sobriety testing § Horizontal Gaze Nystagmus Test (HGN)
See also:Positional alcohol nystagmus

In the United States, testing forhorizontal gaze nystagmusis one of a battery offield sobriety testsused bypoliceofficers to determine whether a suspect isdriving under the influenceofalcohol.Horizontal gaze nystagmus will show if a subject is under the influence of a central nervous system depressant, an inhalant, or a dissociative anesthetic. The test involves observation of the suspect'spupilas it follows a moving object, noting

  1. lack of smooth pursuit,
  2. distinct and sustained nystagmus at maximum deviation, and
  3. the onset of nystagmus prior to 45 degrees.

The horizontal gaze nystagmus test has been highly criticized and major errors in the testing methodology and analysis found.[48][49]However, the validity of the horizontal gaze nystagmus test for use as a field sobriety test for persons with ablood alcohol levelbetween 0.04 and 0.08[clarification needed]is supported by peer-reviewed studies and has been found to be a more accurate indication of blood alcohol content than other standard field sobriety tests.[50]

Media

[edit]

My Dancing Eyes,a documentary by filmmaker Matt Morris, had participants explain what it is like to live with the eye condition, and was released for free. It was featured on NBN News, and ABC Radio Newcastle, in Australia.

See also

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References

[edit]
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  2. ^Weil A (2013)."Dealing with dancing eyes".Weil Lifestyle, LLC. Archived fromthe originalon 2016-08-01.Retrieved2014-04-16.
  3. ^ab"General Information about Nystagmus".American Nystagmus Network. February 21, 2002. Archived fromthe originalon 2016-03-03.Retrieved2011-11-09.
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  6. ^Rowe FJ, Hanna K, Evans JR, Noonan CP, Garcia-Finana M, Dodridge CS, et al. (Cochrane Eyes and Vision Group) (March 2018)."Interventions for eye movement disorders due to acquired brain injury".The Cochrane Database of Systematic Reviews.2018(3): CD011290.doi:10.1002/14651858.CD011290.pub2.PMC6494416.PMID29505103.
  7. ^Self J, Lotery A (December 2007). "A review of the molecular genetics of congenital Idiopathic Nystagmus (CIN)".Ophthalmic Genetics.28(4): 187–91.doi:10.1080/13816810701651233.PMID18161616.S2CID46052164.
  8. ^Li N, Wang L, Cui L, Zhang L, Dai S, Li H, et al. (April 2008)."Five novel mutations of the FRMD7 gene in Chinese families with X-linked infantile nystagmus".Molecular Vision.14:733–8.PMC2324116.PMID18431453.
  9. ^Poopalasundaram S, Erskine L, Cheetham ME, Hardcastle AJ (December 2005). "Focus on molecules: nyctalopin".Experimental Eye Research.81(6): 627–8.doi:10.1016/j.exer.2005.07.017.PMID16157331.
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