Nystagmus
Nystagmus | |
---|---|
Other names | Dancing eyes, Instabilitas oculorum |
physiological) form of nystagmus | |
Specialty | Neurology, ophthalmology, optometry |
Nystagmus is a condition of involuntary (or voluntary, in some cases)
In normal eyesight, while the
There are two key forms of nystagmus:
Causes
The cause of pathological nystagmus may be
Early-onset nystagmus
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:
- Albinism
- Aniridia
- Bilateral congenital cataract
- Bilateral optic nerve hypoplasia
- Idiopathic
- Leber's congenital amaurosis
- Optic nerve or macular disease
- Persistent tunica vasculosa lentis
- Rod monochromatism
- 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.[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, a voltage-gated calcium channel that, when mutated, does not conduct ions.[11]
Acquired nystagmus
Nystagmus that occurs later in childhood or in adulthood is called acquired nystagmus. The cause is often unknown, or
General diseases and conditions
Some of the diseases that present nystagmus as a pathological sign or symptom are as follows:
- Brain tumors (medulloblastoma, astrocytoma, or other tumors in the posterior fossa.)
- Canavan disease
- COVID-19[13]
- 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 combination
Sources of toxicity that could lead to nystagmus:
- Alcohol intoxication
- Amphetamines
- Barbiturates
- Benzodiazepines
- Ketamine
- Pregabalin
- Lithium
- MDMA
- Nitrous Oxide
- Phencyclidine (PCP)
- Phenytoin (Dilantin)
- Salicylates
- Selective serotonin reuptake inhibitors (SSRIs)
- Other anticonvulsants or sedatives
- Thiamine deficiency
- Wernicke's encephalopathy
- Wernicke–Korsakoff syndrome
Thiamine deficiency
Risk factors for
Central nervous system (CNS) diseases and disorders
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:
- Brain abscess (cerebellar)
- Cerebellar ataxia
- Chiari malformation
- Multiple sclerosis
- Stroke
- Thalamic hemorrhage
- Trauma
- Tumor
- Infantile cerebellar retinal degeneration
Other causes
- Non-physiological
- Trochlear nerve malfunction[16]
- Vestibular pathology (vestibular neuritis)
- Exposure to strong magnetic fields (as in MRI machines)[17]
- 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.[18]
- A slightly different form of nystagmus may be produced voluntarily by some people.[19]
Diagnosis
Nystagmus is highly noticeable but rarely recognized. Nystagmus can be clinically investigated by using a number of
The resulting movement of the eyes may be recorded and quantified by a special device called an
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.
to assess a patient's eye movements.Nystagmus can be caused by subsequent
In
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)".[24]
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
]Pathological nystagmus generally causes a degree of
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.[26]
- 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.[27] An example of disease state in which this occurs is benign paroxysmal positional vertigo (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
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
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.[29]
- 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.[30]
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
Surgical treatment of congenital nystagmus is aimed at improving head posture, simulating artificial divergence, or weakening the horizontal recti muscles.[37] 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.[38]
Physical or occupational therapy is also used to treat nystagmus. Treatment consists of learning strategies to compensate for the impaired system.[citation needed]
A
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.[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.[42]
Law enforcement
In the United States, testing for horizontal gaze nystagmus is one of a battery of
- 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.[43][44] However, the validity of the horizontal gaze nystagmus test for use as a field sobriety test for persons with a blood alcohol level between 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.[45]
Media
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
- Bruns nystagmus
- Myoclonus
- Oscillopsia
- Opsoclonus
- Optokinetic nystagmus
References
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- ^ a b "General Information about Nystagmus". American Nystagmus Network. February 21, 2002. Archived from the original on 2016-03-03. Retrieved 2011-11-09.
- ^ MedlinePlus Encyclopedia: Nystagmus
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- ^ "Differences Between Physiologic and Pathologic Nystagmus". Spencer S. Eccles Health Sciences Library. Retrieved 22 November 2016.
- ^ "nystagmus". Retrieved 2007-06-07.
- ^ Gold D. "Gaze-evoked and rebound nystagmus in a cerebellar syndrome". Neuro-Ophthalmology Virtual Education Library (NOVEL): Daniel Gold Collection. Spencer S. Eccles Health Sciences Library. Retrieved 9 September 2019.
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- ^ "Muscular Dystrophy Association Press Release". Archived from the original on 2016-01-18. Retrieved 2015-05-22.
- ^ Clinical trial number NCT02189720 for "Expanded Access Study of Amifampridine Phosphate in LEMS, Congenital Myasthenic Syndrome, or Downbeat Nystagmus Patients (EAP-001)" at ClinicalTrials.gov
- ^ Groves N (March 15, 2006). "Many options to treat nystagmus, more in development". Ophthalmology Times.
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- ^ Cerman E. "Mini-telescopic eyeglasses suppress nystagmus". World Society of Pediatric Ophthalmology and Strabismus Conference in Barcelona 2015. Archived from the original on 3 February 2016. Retrieved 26 January 2016.
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