Nystagmus is a condition of involuntary (or voluntary, in some cases)

[1] eye movement, acquired in infancy or later in life, that may result

in reduced or limited vision.[2] Due to the involuntary movement of the
eye, it has been called "dancing eyes".[3][a]

Nystagmus

Horizontal optokinetic nystagmus, a normal

(physiological) form of nystagmus

Specialt Neurology, otorhinolaryngology
y

In normal eyesight, while the head rotates about an axis, distant visual

images are sustained by rotating eyes in the opposite direction of the
respective axis.[4] The semicircular canals in the vestibule of the ear sense
angular acceleration, which in turn send signals to the nuclei for eye
movement in the brain. From here, a signal is relayed to the extraocular
muscles to allow one’s gaze to fix on an object as the head moves.
Nystagmus occurs when the semicircular canals are stimulated (e.g., by
means of the caloric 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: pathologicaland physiological,
with variations within each type. Nystagmus may be caused by congenital
disorder or sleep deprivation, acquired or central nervous
systemdisorders, toxicity, pharmaceutical drugs, alcohol, or rotational
movement. Previously considered untreatable, in recent years several
drugs have been identified for treatment of nystagmus. Nystagmus is also
occasionally associated with vertigo.
CausesEdit
The cause of pathological nystagmus may be congenital, idiopathic, or
secondary to a pre-existing neurological disorder. It also may be induced
temporarily by disorientation (such as on roller coaster rides) or by
some drugs (alcohol, lidocaine, and other central nervous
system depressants, inhalant drugs, stimulants, psychedelics,
and dissociative drugs).
Early-onset nystagmusEdit
Early onset nystagmus occurs more frequently than acquired nystagmus.
It can be insular or accompany other disorders (such as micro-ophthalmic
anomalies or Down 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:

 Infantile:
o Albinism
o Aniridia
o Bilateral congenital cataract
o Bilateral optic nerve hypoplasia
o Idiopathic
o Leber's congenital amaurosis
o Optic nerve or macular disease
o Persistent tunica vasculosa lentis
o Rod monochromatism
o Visual-motor syndrome of functional monophthalmus
 Latent nystagmus
 Noonan syndrome
 Nystagmus blockage syndrome

X-linked infantile nystagmus is associated with mutations of the

gene FRMD7, which is located on the X chromosome.[6][7]
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).[8][9] CSNB-2 involves mutations of CACNA1F,
a voltage-gated calcium channel that, when mutated, does not conduct
ions.[10]
Acquired nystagmusEdit
Nystagmus that occurs later in childhood or in adulthood is called
acquired nystagmus. The cause is often unknown, or idiopathic, and thus
referred to as idiopathic nystagmus. Other common causes include
diseases and disorders of the central nervous system, metabolic disorders
and alcohol and drug toxicity. In the elderly, stroke is the most common
cause.
General diseases and conditionsEdit
Some of the diseases that present nystagmus as a pathological sign or
symptom are as follows:

 Aniridia
 Benign paroxysmal positional vertigo[11]

 Brain tumors (medulloblastoma, astrocytoma, or other tumors in

the posterior fossa.)
 Canavan disease
 Head trauma
 Lateral medullary syndrome
 Ménière's disease and other balance disorders
  Multiple sclerosis
 Optic nerve hypoplasia
 Pelizaeus–Merzbacher disease
 Superior canal dehiscence syndrome
 Tullio phenomenon
 Whipple's disease

Toxicity or intoxication, metabolic disorders and combinationEdit

Sources of toxicity that could lead to nystagmus:

 Alcohol intoxication
 Amphetamines
 Barbiturates
 Benzodiazepines
 Ketamine
 Pregabalin
 Lithium
 MDMA
 Phencyclidine (PCP)
 Phenytoin (Dilantin)
 Salicylates
 Selective serotonin reuptake inhibitors (SSRIs)
 Other anticonvulsants or sedatives
 Thiamine deficiency
o Wernicke's encephalopathy
o Wernicke–Korsakoff syndrome

Thiamine deficiencyEdit
Risk factors for thiamine deficiency, or beri beri, in turn include a diet of
mostly white rice, as well as alcoholism, dialysis, chronic diarrhea, and
taking high doses of diuretics.[12][13] Rarely it may be due to
a genetic condition that results in difficulties absorbing thiamine found in
food.[12] Wernicke encephalopathyand Korsakoff syndrome are forms of
dry beriberi.[13]
Central nervous system (CNS) diseases and disordersEdit
Central nervous system disorders such as with a cerebellar problem, the
nystagmus can be in any direction including horizontal. Purely vertical
nystagmus usually originates in the central nervous system, but it is also
an adverse effect commonly seen in high phenytoin toxicity. Other causes
of toxicity that may result in nystagmus include:
 Cerebellar ataxia
 Chiari Malformation
 Multiple sclerosis
 Stroke
 Thalamic hemorrhage
 Trauma
 Tumor

Other causesEdit
 Non-physiological
  Trochlear nerve malfunction[14]
 Vestibular Pathology (Ménière's disease, SCDS (superior canal dehiscence
syndrome), BPPV, Labyrinthitis)
 Exposure to strong magnetic fields (as in MRI machines) [15]
 A slightly different form of nystagmus may be produced voluntarily by
some people.[16]

DiagnosisEdit

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 of non-invasive standard tests.
The simplest one is the caloric reflex test, in which one ear canal is
irrigated with warm or cold water or air. The temperature gradient
provokes the stimulation of the horizontal semicircular canal and the
consequent nystagmus. Nystagmus is often very commonly present
with Chiari malformation.
The resulting movement of the eyes may be recorded and quantified by a
special devices called an electronystagmograph (ENG), a form
of electrooculography (an electrical method of measuring eye movements
using external electrodes),[17] or an even less invasive device called
a videonystagmograph (VNG),[18] a form of video-oculography (VOG) (a
video-based method of measuring eye movements using external small
 cameras built into head masks), administered by an audiologist. Special
swinging chairs with electrical controls can be used to induce rotatory
nystagmus.[19]
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.

Orthoptists may also use an optokinetic drum, or electrooculography to

assess a patient's eye movements.
Nystagmus can be caused by subsequent foveationof moving
objects, pathology, sustained rotation or substance use. Nystagmus is not
to be confused with other superficially similar-appearing disorders of eye
movements (saccadic oscillations) such as opsoclonus or ocular
flutter that 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.
In medicine, the presence of nystagmus can be benign, or it can indicate
an underlying visual or neurological problem.[20]
Pathologic nystagmusEdit
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)".[21]
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
the vestibular system, including the semicircular canals, otolith organs,
and the vestibulocerebellum.[contradictory]
Pathological nystagmus generally causes a degree of vision impairment,
although the severity of such impairment varies widely. Also,
many blind people have nystagmus, which is one reason that some wear
dark glasses.[22]
VariationsEdit
 Central nystagmus occurs 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.
o Gaze induced nystagmus occurs or is exacerbated as a result of
changing one's gaze toward or away from a particular side which has an
affected central apparatus.[23]
 Peripheral nystagmus occurs 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
be spontaneous, positional, or evoked.
o Positional nystagmus occurs when a person's head is in a specific
position.[24] An example of disease state in which this occurs is Benign
paroxysmal positional vertigo (BPPV).
 o Post rotational nystagmus occurs 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.
o Spontaneous nystagmus is nystagmus that occurs randomly,
regardless of the position of the patient's head.

Physiological nystagmusEdit
Physiological nystagmus is a form of involuntary eye movement that is
part of the vestibulo-ocular reflex(VOR), characterized by
alternating smooth pursuit in one direction and saccadic movement in the
other direction.
VariationsEdit
The direction of nystagmus is defined by the direction of its quick phase
(e.g. a right-beating nystagmus is characterized by a rightward-moving
quick phase, and a left-beating nystagmus by a leftward-moving quick
phase). The oscillations may occur in the vertical,[25] horizontal
or torsional planes, 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 a saccade in the opposite direction. The process proceeds indefinitely. This is
optokinetic nystagmus, and is a source for understanding the fixation reflex.[26]
 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.[26]

TreatmentEdit
Congenital nystagmus has long been viewed as untreatable, but
medications have been discovered in recent years that show promise in
some patients. In 1980, researchers discovered that a drug
called baclofen could stop periodic alternating nystagmus.
Subsequently, gabapentin, an anticonvulsant, was led to improvement in
about half the patients who took it. Other drugs found to be effective
against nystagmus in some patients include memantine,[27]levetiracetam,
3,4-diaminopyridine (available in the US to eligible patients with downbeat
nystagmus at no cost under an expanded access program [28][29]), 4-
aminopyridine, and acetazolamide.[30] Several therapeutic approaches,
such as contact lenses,[31]drugs, surgery, and low
 vision rehabilitation have also been proposed. For example, it has been
proposed that mini-telescopic eyeglasses suppress nystagmus.[32]
Surgical treatment of congenital nystagmus is aimed at improving head
posture, simulating artificial divergence, or weakening the horizontal recti
muscles.[33] Clinical trials of a surgery to treat nystagmus (known
as tenotomy) 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.
[34]
Acupuncture tests 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 the sternocleidomastoidmuscle.[35][36] 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.[36] By the standards
of evidence-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 consist of learning strategies to compensate for the impaired
system.[citation needed]
A Cochrane Review on 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.
[37]
EpidemiologyEdit
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.[2] 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.
[38]
Law enforcementEdit
Main article: Field sobriety testing § Horizontal Gaze Nystagmus Test (HGN)

See also: Positional alcohol nystagmus

In the United States, testing for horizontal gaze nystagmus is one of a

battery of field sobriety testsused by police officers to determine whether
a suspect is driving under the influence of alcohol. The test involves
observation of the suspect's pupil as 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.[39][40] However, the
validity of the horizontal gaze nystagmus test for use as a field sobriety
test for persons with a blood alcohol levelbetween 0.04–0.08 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.
[41]
See alsoEdit
 Bruns nystagmus
 Myoclonus
 Oscillopsia
 Opsoclonus
 Optokinetic nystagmus

NotesEdit
1. ^ Note however that "dancing eyes" is also a common term
for opsoclonus myoclonus syndrome.

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