Nystagmus is a condition of involuntary (or voluntary, in some cases)
eye movement
Eye movement includes the voluntary or involuntary movement of the eyes. Eye movements are used by a number of organisms (e.g. primates, rodents, flies, birds, fish, cats, crabs, octopus) to fixate, inspect and track visual objects of interest ...
. Infants can be born with it but more commonly acquire it in infancy or later in life. In many cases it may result in
reduced or limited vision.
Due to the involuntary movement of the eye, it has been called "dancing eyes".
In normal eyesight, while the
head
A head is the part of an organism which usually includes the ears, brain, forehead, cheeks, chin, eyes, nose, and mouth, each of which aid in various sensory functions such as sight, hearing, smell, and taste. Some very simple animals may ...
rotates about an
axis
An axis (plural ''axes'') is an imaginary line around which an object rotates or is symmetrical. Axis may also refer to:
Mathematics
* Axis of rotation: see rotation around a fixed axis
* Axis (mathematics), a designator for a Cartesian-coordinat ...
, distant visual images are sustained by rotating eyes in the opposite direction of the respective axis.
The
semicircular canals
The semicircular canals or semicircular ducts are three semicircular, interconnected tubes located in the innermost part of each ear, the inner ear. The three canals are the horizontal, superior and posterior semicircular canals.
Structure
The ...
in the
vestibule of the ear
The vestibule is the central part of the bony labyrinth in the inner ear, and is situated medial to the eardrum, behind the cochlea, and in front of the three semicircular canals.
The name comes from the Latin ', literally an entrance hall.
Stru ...
sense angular acceleration, and send signals to the nuclei for eye movement in the brain. From here, a signal is relayed to the
extraocular muscles
The extraocular muscles (extrinsic ocular muscles), are the seven extrinsic muscles of the human eye. Six of the extraocular muscles, the four recti muscles, and the superior and inferior oblique muscles, control movement of the eye and the ot ...
to allow one's gaze to fix on an object as the head moves. Nystagmus occurs when the
semicircular canals
The semicircular canals or semicircular ducts are three semicircular, interconnected tubes located in the innermost part of each ear, the inner ear. The three canals are the horizontal, superior and posterior semicircular canals.
Structure
The ...
are stimulated (e.g., by means of the
caloric test
In medicine, the caloric reflex test (sometimes termed vestibular caloric stimulation) is a test of the vestibulo-ocular reflex that involves irrigating cold or warm water or air into the external auditory canal. This method was developed by Rob ...
, or by disease) while the head is stationary. The direction of ocular movement is related to the semicircular canal that is being stimulated.
There are two key forms of nystagmus:
pathological
Pathology is the study of the causal, causes and effects of disease or injury. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when us ...
and
physiological
Physiology (; ) is the scientific study of functions and mechanisms in a living system. As a sub-discipline of biology, physiology focuses on how organisms, organ systems, individual organs, cells, and biomolecules carry out the chemical ...
, with variations within each type. Nystagmus may be caused by
congenital disorder
A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can ...
or
sleep deprivation
Sleep deprivation, also known as sleep insufficiency or sleeplessness, is the condition of not having adequate duration and/or quality of sleep to support decent alertness, performance, and health. It can be either chronic or acute and may vary ...
, acquired or
central nervous system
The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain and spinal cord. The CNS is so named because the brain integrates the received information and coordinates and influences the activity of all par ...
disorders,
toxicity
Toxicity is the degree to which a chemical substance or a particular mixture of substances can damage an organism. Toxicity can refer to the effect on a whole organism, such as an animal, bacterium, or plant, as well as the effect on a subst ...
,
pharmaceutical drug
A medication (also called medicament, medicine, pharmaceutical drug, medicinal drug or simply drug) is a drug used to diagnose, cure, treat, or prevent disease. Drug therapy (pharmacotherapy) is an important part of the medical field and re ...
s,
alcohol
Alcohol most commonly refers to:
* Alcohol (chemistry), an organic compound in which a hydroxyl group is bound to a carbon atom
* Alcohol (drug), an intoxicant found in alcoholic drinks
Alcohol may also refer to:
Chemicals
* Ethanol, one of sev ...
, 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
Vertigo is a condition where a person has the sensation of movement or of surrounding objects moving when they are not. Often it feels like a spinning or swaying movement. This may be associated with nausea, vomiting, sweating, or difficulties w ...
.
Causes
The cause of pathological nystagmus may be
congenital
A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can ...
,
idiopathic
An idiopathic disease is any disease with an unknown cause or mechanism of apparent wikt:spontaneous, spontaneous origin. From Ancient Greek, Greek ἴδιος ''idios'' "one's own" and πάθος ''pathos'' "suffering", ''idiopathy'' means approxi ...
, or secondary to a pre-existing
neurological disorder
A neurological disorder is any disorder of the nervous system. Structural, biochemical or electrical abnormalities in the brain, spinal cord or other nerves can result in a range of symptoms. Examples of symptoms include paralysis, muscle weakn ...
. It also may be induced temporarily by
disorientation
Orientation is a function of the mind involving awareness of three dimensions: time, Location (geography), place and person. Problems with orientation lead to ''dis''orientation, and can be due to various conditions, from delirium to Substance int ...
(such as on roller coaster rides or when a person has been spinning in circles) or by some
drug
A drug is any chemical substance that causes a change in an organism's physiology or psychology when consumed. Drugs are typically distinguished from food and substances that provide nutritional support. Consumption of drugs can be via insuffla ...
s (
alcohol
Alcohol most commonly refers to:
* Alcohol (chemistry), an organic compound in which a hydroxyl group is bound to a carbon atom
* Alcohol (drug), an intoxicant found in alcoholic drinks
Alcohol may also refer to:
Chemicals
* Ethanol, one of sev ...
,
lidocaine
Lidocaine, also known as lignocaine and sold under the brand name Xylocaine among others, is a local anesthetic of the amino amide type. It is also used to treat ventricular tachycardia. When used for local anaesthesia or in nerve blocks, lidoca ...
, and other
central nervous system
The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain and spinal cord. The CNS is so named because the brain integrates the received information and coordinates and influences the activity of all par ...
depressants, inhalant drugs,
stimulant
Stimulants (also often referred to as psychostimulants or colloquially as uppers) is an overarching term that covers many drugs including those that increase activity of the central nervous system and the body, drugs that are pleasurable and inv ...
s,
psychedelic
Psychedelics are a subclass of hallucinogenic drugs whose primary effect is to trigger non-ordinary states of consciousness (known as psychedelic experiences or "trips").Pollan, Michael (2018). ''How to Change Your Mind: What the New Science of ...
s, and
dissociative
Dissociatives, colloquially dissos, are a subclass of hallucinogens which distort perception of sight and sound and produce feelings of detachment – dissociation – from the environment and/or self. Although many kinds of drugs are capable of ...
drugs).
Early-onset nystagmus
![Rapid horizontal congenital nystagmus](https://upload.wikimedia.org/wikipedia/commons/9/94/Rapid_horizontal_congenital_nystagmus.gif)
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
Down syndrome or Down's syndrome, also known as trisomy 21, is a genetic disorder caused by the presence of all or part of a third copy of chromosome 21. It is usually associated with physical growth delays, mild to moderate intellectual dis ...
). 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:
**
Albinism
Albinism is the congenital absence of melanin in an animal or plant resulting in white hair, feathers, scales and skin and pink or blue eyes. Individuals with the condition are referred to as albino.
Varied use and interpretation of the term ...
**
Aniridia
Aniridia is the absence of the iris, a muscular structure that opens and closes the pupil to allow light into the eye. It is also responsible for eye color. Without it, the central eye appears all black. It can be congenital, in which both eye ...
**Bilateral
congenital cataract
Congenital cataracts refers to a lens opacity which is present at birth. Congenital cataracts cover a broad spectrum of severity: whereas some lens opacities do not progress and are visually insignificant, others can produce profound visual impa ...
**Bilateral
optic nerve hypoplasia
Optic nerve hypoplasia (ONH) is a medical condition arising from the underdevelopment of the optic nerve(s). This condition is the most common congenital optic nerve anomaly. The optic disc appears abnormally small, because not all the optic nerve ...
**
Idiopathic
An idiopathic disease is any disease with an unknown cause or mechanism of apparent wikt:spontaneous, spontaneous origin. From Ancient Greek, Greek ἴδιος ''idios'' "one's own" and πάθος ''pathos'' "suffering", ''idiopathy'' means approxi ...
**
Leber's congenital amaurosis
Leber congenital amaurosis (LCA) is a rare inherited eye disease that appears at birth or in the first few months of life.
It affects about 1 in 40,000 newborns. LCA was first described by Theodor Leber in the 19th century. It should not be co ...
**
Optic nerve
In neuroanatomy, the optic nerve, also known as the second cranial nerve, cranial nerve II, or simply CN II, is a paired cranial nerve that transmits visual system, visual information from the retina to the brain. In humans, the optic nerve i ...
or
macula
The macula (/ˈmakjʊlə/) or macula lutea is an oval-shaped pigmented area in the center of the retina of the human eye and in other animals. The macula in humans has a diameter of around and is subdivided into the umbo, foveola, foveal avas ...
r disease
**
Persistent tunica vasculosa lentis
Persistent tunica vasculosa lentis is a congenital ocular anomaly. It is a form of persistent hyperplastic primary vitreous (PHPV).
It is a developmental disorder of the vitreous. It is usually unilateral and first noticed in the neonatal pe ...
**
Rod monochromatism
**Visual-motor syndrome of functional monophthalmus
*Latent nystagmus
*
Noonan syndrome
Noonan syndrome (NS) is a genetic disorder that may present with mildly unusual facial features, short height, congenital heart disease, bleeding problems, and skeletal malformations. Facial features include widely spaced eyes, light-colored ...
*Nystagmus blockage syndrome
X-linked infantile nystagmus is associated with mutations of the gene
FRMD7
FERM domain-containing protein 7 is a protein that in humans is encoded by the ''FRMD7'' gene
In biology, the word gene (from , ; "... Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generat ...
, which is located on the
X chromosome
The X chromosome is one of the two sex-determining chromosomes (allosomes) in many organisms, including mammals (the other is the Y chromosome), and is found in both males and females. It is a part of the XY sex-determination system and XO sex-d ...
.
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
Nyctalopin is a protein located on the surface of Retina bipolar cell, photoreceptor-to-ON bipolar cell synapse in the retina. It is composed of 481 amino acids. and is encoded in human by the ''NYX'' gene. This gene is found on the X chromosome, c ...
). CSNB-2 involves mutations of CACNA1F, a
voltage-gated calcium channel
Voltage-gated calcium channels (VGCCs), also known as voltage-dependent calcium channels (VDCCs), are a group of voltage-gated ion channels found in the membrane of excitable cells (''e.g.'', muscle, glial cells, neurons, etc.) with a permeabi ...
that, when mutated, does not conduct ions.
Acquired nystagmus
Nystagmus that occurs later in childhood or in adulthood is called acquired nystagmus. The cause is often unknown, or
idiopathic
An idiopathic disease is any disease with an unknown cause or mechanism of apparent wikt:spontaneous, spontaneous origin. From Ancient Greek, Greek ἴδιος ''idios'' "one's own" and πάθος ''pathos'' "suffering", ''idiopathy'' means approxi ...
, and thus referred to as idiopathic nystagmus. Other common causes include diseases and disorders of the central nervous system,
metabolic disorder
A metabolic disorder is a disorder that negatively alters the body's processing and distribution of macronutrients, such as proteins, fats, and carbohydrates. Metabolic disorders can happen when abnormal chemical reactions in the body alter the ...
s and alcohol and drug toxicity. In the elderly,
stroke
A stroke is a medical condition in which poor blood flow to the brain causes cell death. There are two main types of stroke: ischemic, due to lack of blood flow, and hemorrhagic, due to bleeding. Both cause parts of the brain to stop functionin ...
is the most common cause.
General diseases and conditions
Some of the diseases that present nystagmus as a pathological sign or symptom are as follows:
:*
Aniridia
Aniridia is the absence of the iris, a muscular structure that opens and closes the pupil to allow light into the eye. It is also responsible for eye color. Without it, the central eye appears all black. It can be congenital, in which both eye ...
:*
Benign paroxysmal positional vertigo
Benign paroxysmal positional vertigo (BPPV) is a disorder arising from a problem in the inner ear. Symptoms are repeated, brief periods of vertigo with movement, characterized by a spinning sensation upon changes in the position of the head.
* T ...
:*
Brain tumor
A brain tumor occurs when abnormal cells form within the brain. There are two main types of tumors: malignant tumors and benign (non-cancerous) tumors. These can be further classified as primary tumors, which start within the brain, and seconda ...
s (
medulloblastoma
Medulloblastoma is a common type of primary brain cancer in children. It originates in the part of the brain that is towards the back and the bottom, on the floor of the skull, in the cerebellum, or posterior fossa.
The brain is divided into two ...
,
astrocytoma
Astrocytomas are a type of brain tumor. They originate in a particular kind of glial cells, star-shaped brain cells in the cerebrum called astrocytes. This type of tumor does not usually spread outside the brain and spinal cord and it does not usu ...
, or other tumors in the
posterior fossa.)
:*
Canavan disease
Canavan disease, or Canavan-Van Bogaert-Bertrand disease, is a rare and fatal autosomal recessive degenerative disease that causes progressive damage to nerve cells and loss of white matter in the brain. It is one of the most common degenerative ...
:*
COVID-19
Coronavirus disease 2019 (COVID-19) is a contagious disease caused by a virus, the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The first known case was COVID-19 pandemic in Hubei, identified in Wuhan, China, in December ...
:*
Head trauma
A head injury is any injury that results in trauma to the skull or brain. The terms ''traumatic brain injury'' and ''head injury'' are often used interchangeably in the medical literature. Because head injuries cover such a broad scope of inju ...
:*
Lateral medullary syndrome
Lateral medullary syndrome is a neurological disorder causing a range of symptoms due to ischemia in the lateral part of the medulla oblongata in the brainstem. The ischemia is a result of a blockage most commonly in the vertebral artery or the p ...
:*
Ménière's disease
Ménière's disease (MD) is a disease of the inner ear that is characterized by potentially severe and incapacitating episodes of vertigo, tinnitus, hearing loss, and a feeling of fullness in the ear. Typically, only one ear is affected initial ...
and other
balance disorder
A balance disorder is a disturbance that causes an individual to feel unsteady, for example when standing or walking. It may be accompanied by feelings of giddiness, or wooziness, or having a sensation of movement, spinning, or floating. Balance ...
s
:*
Multiple sclerosis
Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This d ...
:*
Optic nerve hypoplasia
Optic nerve hypoplasia (ONH) is a medical condition arising from the underdevelopment of the optic nerve(s). This condition is the most common congenital optic nerve anomaly. The optic disc appears abnormally small, because not all the optic nerve ...
:*
Pelizaeus–Merzbacher disease
Friedrich Christoph Pelizaeus, Pelizaeus–Ludwig Merzbacher, Merzbacher disease is an X-linked neurological disorder that damages oligodendrocytes in the central nervous system. It is caused by mutations in proteolipid protein 1 (''PLP1''), a maj ...
:*
Superior canal dehiscence syndrome
Superior semicircular canal dehiscence syndrome is a set of hearing and balance symptoms, related to a rare medical condition of the inner ear, known as ''superior canal dehiscence''. The symptoms are caused by a thinning or complete absence of ...
:*
Tullio phenomenon Tullio phenomenon, sound-induced vertigo, dizziness, nausea or eye movement (nystagmus) was first described in 1929 by the Italian biologist Prof. Pietro Tullio. (1881–1941)Tullio, Pietro: Some experiments and considerations on experimental otolog ...
:*
Whipple's disease
Whipple's disease is a rare systemic infectious disease caused by the bacterium '' Tropheryma whipplei''. First described by George Hoyt Whipple in 1907 and commonly considered as a gastrointestinal disorder, Whipple's disease primarily causes m ...
Toxicity or intoxication, metabolic disorders and combination
Sources of toxicity that could lead to nystagmus:
:*
Alcohol intoxication
Alcohol intoxication, also known as alcohol poisoning, commonly described as drunkenness or inebriation, is the negative behavior and physical effects caused by a recent consumption of alcohol. In addition to the toxicity of ethanol, the main p ...
:*
Amphetamines
Substituted amphetamines are a class of compounds based upon the amphetamine structure; it includes all derivative compounds which are formed by replacing, or substituting, one or more hydrogen atoms in the amphetamine core structure with sub ...
:*
Barbiturates
:*
Benzodiazepines
:*
Ketamine
Ketamine is a dissociative anesthetic used medically for induction and maintenance of anesthesia. It is also used as a recreational drug. It is one of the safest anesthetics, as, in contrast with opiates, ether, and propofol, it suppresses ne ...
:*
Pregabalin
Pregabalin, sold under the brand name Lyrica among others, is an anticonvulsant, analgesic and anxiolytic medication used to treat epilepsy, neuropathic pain, fibromyalgia, restless leg syndrome, opioid withdrawal and generalized anxiety disord ...
:*
Lithium
Lithium (from el, λίθος, lithos, lit=stone) is a chemical element with the symbol Li and atomic number 3. It is a soft, silvery-white alkali metal. Under standard conditions, it is the least dense metal and the least dense solid el ...
:*
MDMA
3,4-Methylenedioxymethamphetamine (MDMA), commonly seen in Tablet (pharmacy), tablet form (ecstasy) and crystal form (molly or mandy), is a potent empathogen–entactogen with stimulant properties primarily used for Recreational dru ...
:*
Nitrous Oxide
Nitrous oxide (dinitrogen oxide or dinitrogen monoxide), commonly known as laughing gas, nitrous, or nos, is a chemical compound, an oxide of nitrogen with the formula . At room temperature, it is a colourless non-flammable gas, and has a ...
:*
Phencyclidine
Phencyclidine or phenylcyclohexyl piperidine (PCP), also known as angel dust among other names, is a dissociative anesthetic mainly used recreationally for its significant mind-altering effects. PCP may cause hallucinations, distorted perceptio ...
(PCP)
:*
Phenytoin
Phenytoin (PHT), sold under the brand name Dilantin among others, is an anti-seizure medication. It is useful for the prevention of tonic-clonic seizures (also known as grand mal seizures) and focal seizures, but not absence seizures. The intr ...
(Dilantin)
:*
Salicylates
Salicylic acid is an organic compound with the formula HOC6H4CO2H. A colorless, bitter-tasting solid, it is a precursor to and a metabolite of aspirin (acetylsalicylic acid). It is a plant hormone, and has been listed by the EPA Toxic Substa ...
:*
Selective serotonin reuptake inhibitors (SSRIs)
:* Other anticonvulsants or sedatives
:*
Thiamine
Thiamine, also known as thiamin and vitamin B1, is a vitamin, an essential micronutrient, that cannot be made in the body. It is found in food and commercially synthesized to be a dietary supplement or medication. Phosphorylated forms of thi ...
deficiency
:**
Wernicke's encephalopathy
:**
Wernicke–Korsakoff syndrome
Wernicke–Korsakoff syndrome (WKS) is the combined presence of Wernicke encephalopathy (WE) and Korsakoff syndrome. Due to the close relationship between these two disorders, people with either are usually diagnosed with WKS as a single syndrome ...
Thiamine deficiency
Risk factors for
thiamine deficiency
Thiamine deficiency is a medical condition of low levels of thiamine (Vitamin B1). A severe and chronic form is known as beriberi. The two main types in adults are wet beriberi and dry beriberi. Wet beriberi affects the cardiovascular system, r ...
, or beri beri, in turn include a diet of mostly
white rice
White rice is milled rice that has had its husk, bran, and germ removed. This alters the flavor, texture and appearance of the rice and helps prevent spoilage, extend its storage life, and makes it easier to digest. After milling (hulling), the ...
, as well as
alcoholism
Alcoholism is, broadly, any drinking of alcohol (drug), alcohol that results in significant Mental health, mental or physical health problems. Because there is disagreement on the definition of the word ''alcoholism'', it is not a recognize ...
,
dialysis, chronic
diarrhea
Diarrhea, also spelled diarrhoea, is the condition of having at least three loose, liquid, or watery bowel movements each day. It often lasts for a few days and can result in dehydration due to fluid loss. Signs of dehydration often begin wi ...
, and taking high doses of
diuretics
A diuretic () is any substance that promotes diuresis, the increased production of urine. This includes forced diuresis. A diuretic tablet is sometimes colloquially called a water tablet. There are several categories of diuretics. All diuretics in ...
.
[ Rarely it may be due to a genetic condition that results in difficulties absorbing thiamine found in food.][ ]Wernicke encephalopathy
Wernicke encephalopathy (WE), also Wernicke's encephalopathy, or wet brain is the presence of neurological symptoms caused by biochemical lesions of the central nervous system after exhaustion of B-vitamin reserves, in particular thiamine (vit ...
and Korsakoff syndrome
Korsakoff syndrome (KS) is a disorder of the central nervous system characterized by amnesia, deficits in explicit memory, and confabulation. This neurological disorder is caused by a deficiency of thiamine (vitamin B1) in the brain, and it is ...
are forms of dry beriberi.
Central nervous system (CNS) diseases and disorders
Central nervous system
The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain and spinal cord. The CNS is so named because the brain integrates the received information and coordinates and influences the activity of all par ...
disorders such as with a cerebellar
The cerebellum (Latin for "little brain") is a major feature of the hindbrain of all vertebrates. Although usually smaller than the cerebrum, in some animals such as the mormyrid fishes it may be as large as or even larger. In humans, the cereb ...
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
Phenytoin (PHT), sold under the brand name Dilantin among others, is an anti-seizure medication. It is useful for the prevention of tonic-clonic seizures (also known as grand mal seizures) and focal seizures, but not absence seizures. The intr ...
toxicity. Other causes of toxicity that may result in nystagmus include:
:*Brain abscess
Brain abscess (or cerebral abscess) is an abscess caused by inflammation and collection of infected material, coming from local (ear infection, dental abscess, infection of paranasal sinuses, infection of the mastoid air cells of the temporal bone ...
(Cerebellar)
:*Cerebellar ataxia
Cerebellar ataxia is a form of ataxia originating in the cerebellum. Non-progressive congenital ataxia (NPCA) is a classical presentation of cerebral ataxias.
Cerebellar ataxia can occur as a result of many diseases and may present with symptom ...
:*Chiari Malformation
Chiari malformation (CM) is a structural defect in the cerebellum, characterized by a downward displacement of one or both cerebellar tonsils through the foramen magnum (the opening at the base of the skull). CMs can cause headaches, difficu ...
:*Multiple sclerosis
Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This d ...
:*Stroke
A stroke is a medical condition in which poor blood flow to the brain causes cell death. There are two main types of stroke: ischemic, due to lack of blood flow, and hemorrhagic, due to bleeding. Both cause parts of the brain to stop functionin ...
:* Thalamic hemorrhage
:*Trauma
:*Tumor
A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
:* Infantile cerebellar retinal degeneration
Infantile cerebellar retinal degeneration is a rare hereditary neurological disorder which primarily affects the eyes and the brain.
Presentation
Babies with this condition usually appear normal at birth, but start showing symptoms when they a ...
Other causes
:*Non-physiological
:*Trochlear nerve
The trochlear nerve (), ( lit. ''pulley-like'' nerve) also known as the fourth cranial nerve, cranial nerve IV, or CN IV, is a cranial nerve that innervates just one muscle: the superior oblique muscle of the eye, which operates through the pul ...
malfunction
:*Vestibular Pathology (Ménière's disease
Ménière's disease (MD) is a disease of the inner ear that is characterized by potentially severe and incapacitating episodes of vertigo, tinnitus, hearing loss, and a feeling of fullness in the ear. Typically, only one ear is affected initial ...
, SCDS (superior canal dehiscence syndrome
Superior semicircular canal dehiscence syndrome is a set of hearing and balance symptoms, related to a rare medical condition of the inner ear, known as ''superior canal dehiscence''. The symptoms are caused by a thinning or complete absence of ...
), BPPV
Benign paroxysmal positional vertigo (BPPV) is a disorder arising from a problem in the inner ear. Symptoms are repeated, brief periods of vertigo with movement, characterized by a spinning sensation upon changes in the position of the head.
* T ...
, Labyrinthitis
Labyrinthitis is inflammation of the labyrinth – a maze of fluid-filled channels in the inner ear. Vestibular neuritis is inflammation of the vestibular nerve – the nerve in the inner ear that sends messages related to motion and position t ...
)
:*Exposure to strong magnetic fields (as in MRI machines)
:*Long-term exposure to low light conditions or darkness, called ''miner's nystagmus'' after 19th-century coal miners who developed nystagmus from working in the dark.
:*A slightly different form of nystagmus may be produced voluntarily by some people.
Diagnosis
Nystagmus is highly noticeable but rarely recognized. Nystagmus can be clinically investigated by using a number of non-invasive
A medical procedure is defined as ''non-invasive'' when no break in the skin is created and there is no contact with the mucosa, or skin break, or internal body cavity beyond a natural or artificial body orifice. For example, deep palpation and p ...
standard tests. The simplest one is the caloric reflex test
In medicine
Medicine is the science and practice of caring for a patient, managing the diagnosis, prognosis, prevention, treatment, palliation of their injury or disease, and promoting their health. Medicine encompasses a variety of ...
, 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
The semicircular canals or semicircular ducts are three semicircular, interconnected tubes located in the innermost part of each ear, the inner ear. The three canals are the horizontal, superior and posterior semicircular canals.
Structure
The ...
and the consequent nystagmus.
Nystagmus is often very commonly present with Chiari malformation
Chiari malformation (CM) is a structural defect in the cerebellum, characterized by a downward displacement of one or both cerebellar tonsils through the foramen magnum (the opening at the base of the skull). CMs can cause headaches, difficu ...
.
The resulting movement of the eyes may be recorded and quantified by a special device called an electronystagmograph (ENG), a form of electrooculography
Electrooculography (EOG) is a technique for measuring the corneo-retinal standing potential that exists between the front and the back of the human eye. The resulting signal is called the electrooculogram. Primary applications are in ophthalmol ...
(an electrical method of measuring eye movements using external electrodes
An electrode is an electrical conductor used to make contact with a nonmetallic part of a circuit (e.g. a semiconductor, an electrolyte, a vacuum or air). Electrodes are essential parts of batteries that can consist of a variety of materials de ...
), or an even less invasive device called a videonystagmograph (VNG), a form of video-oculography
Video-oculography (VOG) is a non-invasive, video-based method of measuring horizontal, vertical and torsional position components of the movements of both eyes (eye tracking) using a head-mounted mask that is equipped with small cameras. VOG is usu ...
(VOG) (a video-based method of measuring eye movements using external small cameras built into head masks), administered by an audiologist
Audiology (from Latin , "to hear"; and from Greek , ''-logia'') is a branch of science that studies hearing, balance, and related disorders. Audiologists treat those with hearing loss and proactively prevent related damage. By employing vario ...
. Special swinging chairs with electrical controls can be used to induce rotatory nystagmus.
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.
Orthoptist
Orthoptics is a profession allied to the eye care profession. Orthoptists are the experts in diagnosing and treating defects in eye movements and problems with how the eyes work together, called binocular vision. These can be caused by issues with ...
s may also use an optokinetic drum
An optokinetic drum—also called catford drum—is a rotating instrument to test vision in which individuals are seated facing the wall of the drum. The interior surface of the drum is normally striped; thus, as the drum rotates, the subject's eye ...
, or electrooculography
Electrooculography (EOG) is a technique for measuring the corneo-retinal standing potential that exists between the front and the back of the human eye. The resulting signal is called the electrooculogram. Primary applications are in ophthalmol ...
or Frenzel goggles
Frenzel goggles are a diagnostic tool used in ophthalmology, otolaryngology and audiovestibular medicine for the medical evaluation of involuntary eye movement (nystagmus). They are named after Frenzel, a German physician.
Usage
The purpose of the ...
to assess a patient's eye movements.
Nystagmus can be caused by subsequent foveation of moving objects, pathology
Pathology is the study of the causes and effects of disease or injury. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when used in ...
, 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
Opsoclonus refers to uncontrolled, irregular, and nonrhythmic eye movement. Opsoclonus consists of rapid, involuntary, multivectorial (horizontal and vertical), unpredictable, conjugate fast eye movements without inter-saccadic intervals. It is a ...
or ocular flutter Ocular flutter is an opsoclonic disorder that results in horizontal saccades.
It is caused by damage to the brainstem paramedian pontine reticular formation cells or the cerebellar neurons controlling those cells.
See also
* Nystagmus
* Opsoclo ...
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
Medicine is the science and practice of caring for a patient, managing the diagnosis, prognosis, prevention, treatment, palliation of their injury or disease, and promoting their health. Medicine encompasses a variety of health care pract ...
, the presence of nystagmus can be benign
Malignancy () is the tendency of a medical condition to become progressively worse.
Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...
, or it can indicate an underlying visual or neurological problem.
Pathologic nystagmus
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)".
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
The vestibular system, in vertebrates, is a sensory system that creates the sense of balance and spatial orientation for the purpose of coordinating movement with balance. Together with the cochlea, a part of the auditory system, it constitutes ...
, including the semicircular canal
In mathematics (and more specifically geometry), a semicircle is a one-dimensional locus of points that forms half of a circle. The full arc of a semicircle always measures 180° (equivalently, radians, or a half-turn). It has only one line of ...
s, otolith
An otolith ( grc-gre, ὠτο-, ' ear + , ', a stone), also called statoconium or otoconium or statolith, is a calcium carbonate structure in the saccule or utricle of the inner ear, specifically in the vestibular system of vertebrates. The sa ...
organs, and the vestibulocerebellum
The anatomy of the cerebellum can be viewed at three levels. At the level of gross anatomy, the cerebellum consists of a tightly folded and crumpled layer of cortex, with white matter underneath, several deep nuclei embedded in the white matte ...
.
Pathological nystagmus generally causes a degree of vision impairment
Visual impairment, also known as vision impairment, is a medical definition primarily measured based on an individual's better eye visual acuity; in the absence of treatment such as correctable eyewear, assistive devices, and medical treatment ...
, although the severity of such impairment varies widely. Also, many blind people have nystagmus, which is one reason that some wear dark glasses.
Variations
*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.
**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.
*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''.
**Positional nystagmus occurs when a person's head is in a specific position. An example of disease state in which this occurs is Benign paroxysmal positional vertigo
Benign paroxysmal positional vertigo (BPPV) is a disorder arising from a problem in the inner ear. Symptoms are repeated, brief periods of vertigo with movement, characterized by a spinning sensation upon changes in the position of the head.
* T ...
(BPPV).
**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.
**Spontaneous nystagmus is nystagmus that occurs randomly, regardless of the position of the patient's head.
Physiological nystagmus
Physiological nystagmus is a form of involuntary eye movement
Eye movement includes the voluntary or involuntary movement of the eyes. Eye movements are used by a number of organisms (e.g. primates, rodents, flies, birds, fish, cats, crabs, octopus) to fixate, inspect and track visual objects of interest ...
that is part of the vestibulo-ocular reflex (VOR), characterized by alternating smooth pursuit
In the scientific study of vision, smooth pursuit describes a type of eye movement in which the eyes remain fixated on a moving object. It is one of two ways that visual animals can voluntarily shift gaze, the other being saccadic eye movemen ...
in one direction and saccadic movement in the other direction.
Variations
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
Oscillation is the repetitive or periodic variation, typically in time, of some measure about a central value (often a point of equilibrium) or between two or more different states. Familiar examples of oscillation include a swinging pendulum ...
may occur in the vertical, horizontal or torsional
In the field of solid mechanics, torsion is the twisting of an object due to an applied torque. Torsion is expressed in either the pascal (Pa), an SI unit for newtons per square metre, or in pounds per square inch (psi) while torque is expressed ...
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
A saccade ( , French for ''jerk'') is a quick, simultaneous movement of both eyes between two or more phases of fixation in the same direction.Cassin, B. and Solomon, S. ''Dictionary of Eye Terminology''. Gainesville, Florida: Triad Publishi ...
in the opposite direction. The process proceeds indefinitely. This is optokinetic nystagmus, and is a source for understanding the fixation reflex.
* 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.
Treatment
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 called baclofen
Baclofen, sold under the brand name Lioresal among others, is a medication used to treat muscle spasticity such as from a spinal cord injury or multiple sclerosis. It may also be used for hiccups and muscle spasms near the end of life. It is ta ...
could stop periodic alternating nystagmus. Subsequently, gabapentin
Gabapentin, sold under the brand name Neurontin among others, is an anticonvulsant medication primarily used to treat partial seizures and neuropathic pain. It is a first-line medication for the treatment of neuropathic pain caused by diabet ...
, an anticonvulsant, led to improvement in about half the patients who took it. Other drugs found to be effective against nystagmus in some patients include memantine
Memantine is a medication used to slow the progression of moderate-to-severe Alzheimer's disease. It is taken by mouth.
Common side effects include headache, constipation, sleepiness, and dizziness. Severe side effects may include blood clots ...
, levetiracetam
Levetiracetam, sold under the brand name Keppra among others, is a medication used to treat epilepsy. It is used for partial-onset, myoclonic, or tonic–clonic seizures and is taken either by mouth as an immediate or extended release formu ...
, 3,4-diaminopyridine (available in the US to eligible patients with downbeat nystagmus at no cost under an expanded access program[Muscular Dystrophy Association Press Release](_blank)
/ref>), 4-aminopyridine, and acetazolamide
Acetazolamide, sold under the trade name Diamox among others, is a medication used to treat glaucoma, epilepsy, altitude sickness, periodic paralysis, idiopathic intracranial hypertension (raised brain pressure of unclear cause), urine alkalin ...
. Several therapeutic approaches, such as contact lenses
Contact lenses, or simply contacts, are thin lenses placed directly on the surface of the eyes. Contact lenses are ocular prosthetic devices used by over 150 million people worldwide, and they can be worn to correct vision or for cosmetic ...
, drugs, surgery
Surgery ''cheirourgikē'' (composed of χείρ, "hand", and ἔργον, "work"), via la, chirurgiae, meaning "hand work". is a medical specialty that uses operative manual and instrumental techniques on a person to investigate or treat a pat ...
, and low vision
Visual impairment, also known as vision impairment, is a medical definition primarily measured based on an individual's better eye visual acuity; in the absence of treatment such as correctable eyewear, assistive devices, and medical treatment ...
rehabilitation
Rehabilitation or Rehab may refer to:
Health
* Rehabilitation (neuropsychology), therapy to regain or improve neurocognitive function that has been lost or diminished
* Rehabilitation (wildlife), treatment of injured wildlife so they can be retur ...
have also been proposed. For example, it has been proposed that mini-telescopic eyeglasses suppress nystagmus.
Surgical treatment of congenital nystagmus is aimed at improving head posture, simulating artificial divergence, or weakening the horizontal recti muscles. Clinical trials of a surgery to treat nystagmus (known as tenotomy
A tenotomy is a surgical act which involves the division of a tendon. It and related procedures are also referred to as tendon release, tendon lengthening, and heel-cord release.
When it involves the Achilles tendon, it is called "Achillotenotomy ...
) 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.
Acupuncture
Acupuncture is a form of alternative medicine and a component of traditional Chinese medicine (TCM) in which thin needles are inserted into the body. Acupuncture is a pseudoscience; the theories and practices of TCM are not based on scientifi ...
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 sternocleidomastoid
The sternocleidomastoid muscle is one of the largest and most superficial cervical muscles. The primary actions of the muscle are rotation of the head to the opposite side and flexion of the neck. The sternocleidomastoid is innervated by the access ...
muscle. 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.[ By the standards of ]evidence-based medicine
Evidence-based medicine (EBM) is "the conscientious, explicit and judicious use of current best evidence in making decisions about the care of individual patients". The aim of EBM is to integrate the experience of the clinician, the values of t ...
, 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, 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.
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.
Epidemiology
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.[ 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.
]
Law enforcement
In the United States, testing for ''horizontal gaze nystagmus'' is one of a battery of field sobriety tests
Field may refer to:
Expanses of open ground
* Field (agriculture), an area of land used for agricultural purposes
* Airfield, an aerodrome that lacks the infrastructure of an airport
* Battlefield
* Lawn, an area of mowed grass
* Meadow, a grass ...
used by police
The police are a constituted body of persons empowered by a state, with the aim to enforce the law, to ensure the safety, health and possessions of citizens, and to prevent crime and civil disorder. Their lawful powers include arrest and t ...
officers to determine whether a suspect is driving under the influence
Driving under the influence (DUI)—also called driving while impaired, impaired driving, driving while intoxicated (DWI), drunk driving, operating while intoxicated (OWI), operating under the influence (OUI), operating vehicle under the infl ...
of alcohol
Alcohol most commonly refers to:
* Alcohol (chemistry), an organic compound in which a hydroxyl group is bound to a carbon atom
* Alcohol (drug), an intoxicant found in alcoholic drinks
Alcohol may also refer to:
Chemicals
* Ethanol, one of sev ...
. The test involves observation of the suspect's pupil
The pupil is a black hole located in the center of the iris of the eye that allows light to strike the retina.Cassin, B. and Solomon, S. (1990) ''Dictionary of Eye Terminology''. Gainesville, Florida: Triad Publishing Company. It appears black ...
as it follows a moving object, noting
# lack of smooth pursuit,
# distinct and sustained nystagmus at maximum deviation, and
# 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. However, the validity of the horizontal gaze nystagmus test for use as a field sobriety test for persons with a blood alcohol level
Blood alcohol content (BAC), also called blood alcohol concentration or blood alcohol level, is a measurement of alcohol intoxication used for legal or medical purposes; it is expressed as mass of alcohol per volume or mass of blood. For example ...
between 0.04 and 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.
See also
* Bruns nystagmus
* Myoclonus
Myoclonus is a brief, involuntary, irregular (lacking rhythm) twitching of a muscle or a group of muscles, different from clonus, which is rhythmic or regular. Myoclonus (myo "muscle", clonic "jerk") describes a medical sign and, generally, is ...
* Oscillopsia Oscillopsia is a visual disturbance in which objects in the visual field appear to oscillate. The severity of the effect may range from a mild blurring to rapid and periodic jumping. Oscillopsia is an incapacitating condition experienced by many p ...
* Opsoclonus
Opsoclonus refers to uncontrolled, irregular, and nonrhythmic eye movement. Opsoclonus consists of rapid, involuntary, multivectorial (horizontal and vertical), unpredictable, conjugate fast eye movements without inter-saccadic intervals. It is a ...
* Optokinetic nystagmus
The optokinetic response is a combination of a slow-phase and fast-phase eye movements. It is seen when an individual tracks (pursuit movement) a moving object with their eyes, which then moves out of the field of vision, a point at which their ...
Explanatory notes
References
{{DEFAULTSORT:Nystagmus
Eye diseases
Medical signs
Neurological disorders