Nystagmus is a condition of involuntary (or voluntary, in rare cases) eye movement, acquired in infancy or later in life, that may result in reduced or limited vision. Due to the involuntary movement of the eye, it has been called "dancing eyes".[a]
|Horizontal optokinetic nystagmus, a normal (physiological) form of nystagmus|
|Classification and external resources|
In a normal condition, while the head rotates about any axis, distant visual images are sustained by rotating eyes in the opposite direction on the respective axis. The semicircular canals in the vestibule sense angular acceleration. These 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 fixate on one object as the head moves. Nystagmus also occurs when the semicircular canals are being stimulated (e.g. by means of the caloric test, or by disease) while the head is not in motion. The direction of ocular movement is related to the semicircular canal that is being stimulated.
There are two key forms of nystagmus: pathological and physiological, with variations within each type. Nystagmus may be caused by congenital disorders, acquired or central nervous system disorders, toxicity, pharmaceutical drugs, alcohol, or rotational movement. Previously considered untreatable, in recent years several pharmaceutical drugs have been identified for treatment of nystagmus. Nystagmus is also occasionally associated with vertigo.
The cause for 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 certain drugs (alcohol and other central nervous system depressants, inhalant drugs, stimulants, psychedelic drugs, and dissociative drugs).
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 not normally aware of their spontaneous eye movements, but vision can be impaired depending on the severity of the movements.
Types of early-onset nystagmus include the following:
- Latent nystagmus
- Noonan syndrome
- Nystagmus blockage syndrome
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). CSNB-2 involves mutations of CACNA1F, a voltage-gated calcium channel that, when mutated, does not conduct ions.
It may be acquired from:
- Diseases. Some of the diseases that present nystagmus as a pathological sign:
- Benign paroxysmal positional vertigo
- 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
- Stroke (the most common cause in older people)
- Tullio phenomenon
- Wernicke–Korsakoff syndrome
- Whipple's disease
- Toxic or metabolic reasons could be the result of the following:
- Central nervous system (CNS) disorders, such as with a cerebellar problem, the nystagmus can be in any direction including horizontal. Purely vertical nystagmus is usually central in origin, but it is also a frequent adverse effect of high phenytoin toxicity. Causes include:
In the United States, testing for horizontal gaze nystagmus is one of a battery of field sobriety tests used 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
- 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 between 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.
Nystagmus is very 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 special devices called electronystagmograph (ENG), a form of electrooculography (an electrical method of measuring eye movements using external electrodes), or even less invasive devices called videonystagmograph (VNG), a form of video-oculography (VOG) (a video-based method of measuring eye movements using external small cameras built into head masks) by an audiologist. 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 a greater accuracy and understanding of the condition.
Nystagmus can be caused by subsequent foveation of 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 characterised by the combination of a smooth pursuit, which usually acts to take the eye off the point of regard, 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 between these conditions.
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, 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.
- 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 (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 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.
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, 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.
- 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.
- 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.
Congenital nystagmus has traditionally been viewed as non-treatable, but medications have been discovered in recent years that show promise in some patients. In 1980, researchers discovered that a drug called baclofen could effectively stop periodic alternating nystagmus. Subsequently, gabapentin, an anticonvulsant, was found to cause improvement in about half the patients who received it to relieve symptoms of nystagmus. Other drugs found to be effective against nystagmus in some patients include memantine, levetiracetam, 3,4-diaminopyridine (available in the US to eligible patients with downbeat nystagmus at no cost under an expanded access program), 4-aminopyridine, and acetazolamide. Several therapeutic approaches, such as contact lenses, drugs, surgery, and low vision rehabilitation 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 the abnormal head posture, simulating artificial divergence or weakening the horizontal recti muscles. 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 shaking (oscillations), which in turn tends to improve visual acuity.
Acupuncture has conflicting evidence as to having beneficial effects on the symptoms of nystagmus. Benefits have been seen in treatments where acupuncture points of the neck were used, specifically points on the sternocleidomastoid 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, the quality of these studies can be considered poor (for example, Ishikawa has a study sample size of just six, is unblinded and without proper control), 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 produced.
Physical or occupational therapy is also used to treat nystagmus. Treatment consist of learning compensatory strategies to take over for the impaired system.
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 (~0.240 %), noting an apparently higher rate amongst white Europeans than in individuals of Asian origin.
- Zahn JR (July 1978). "Incidence and characteristics of voluntary nystagmus". J. .jNeurol.Neurosurg. Psychiatr. 41 (7): 617–23. doi:10.1136/jnnp.41.7.617. PMC . PMID 690639.
- "General Information about Nystagmus". American Nystagmus Network. February 21, 2002. Retrieved 2011-11-09.
- Weil, Andrew (2013). "Dealing with dancing eyes". Weil Lifestyle, LLC. Retrieved 2014-04-16.
- "Nystagmus". Medline Plus. February 27, 2013. Retrieved 2012-12-12.
- Saladin, Kenneth (2012). Anatomy and Physiology: The Unity of Form and Function. New York: McGraw-Hill. pp. 597–609. ISBN 978-0-07-337825-1.
- Self, James; Lotery, Andrew (2007). "A Review of the Molecular Genetics of Congenital Idiopathic Nystagmus (CIN)". Ophthalmic Genetics. 28 (4): 187–91. doi:10.1080/13816810701651233. PMID 18161616.
- Li, N; Wang, L; Cui, L; Zhang, L; Dai, S; Li, H; Chen, X; Zhu, L; Hejtmancik, JF; Zhao, K (2008). "Five novel mutations of the FRMD7 gene in Chinese families with X-linked infantile nystagmus". Molecular Vision. 14: 733–8. PMC . PMID 18431453.
- Poopalasundaram, S; Erskine, L; Cheetham, M; Hardcastle, A (2005). "Focus on Molecules: Nyctalopin". Experimental Eye Research. 81 (6): 627–8. doi:10.1016/j.exer.2005.07.017. PMID 16157331.
- Leroy, B P; Budde, B S; Wittmer, M; De Baere, E; Berger, W; Zeitz, C (2008). "A common NYX mutation in Flemish patients with X linked CSNB". British Journal of Ophthalmology. 93 (5): 692–6. doi:10.1136/bjo.2008.143727. PMID 18617546.
- Peloquin, J.B.; Rehak, R.; Doering, C.J.; McRory, J.E. (2007). "Functional analysis of congenital stationary night blindness type-2 CACNA1F mutations F742C, G1007R, and R1049W". Neuroscience. 150 (2): 335–45. doi:10.1016/j.neuroscience.2007.09.021. PMID 17949918.
- Ganança, Fernando Freitas; Ganança, Ricardo; Ganança, Maurício M.; Korn, Gustavo P.; Dorigueto, Ricardo S. (2005). "É importante restringir a movimentação cefálica após a manobra de Epley?" [The number of procedures required to eliminate positioning nystagmus in benign paroxysmal positional vertigo]. Revista Brasileira de Otorrinolaringologia (in Portuguese). 71 (6): 769–75. doi:10.1590/S0034-72992005000600013. PMID 16878247.
- Lindgren, Stefan (1993). Kliniska färdigheter: Informationsutbytet mellan patient och läkare (in Swedish). Lund: Studentlitteratur. ISBN 91-44-37271-X.[page needed]
- Tusa, RJ (June 1999). "Nystagmus: diagnostic and therapeutic strategies". Seminars in ophthalmology. 14 (2): 65–73. doi:10.3109/08820539909056066. PMID 10758214.
- Booker JL (2004). "The Horizontal Gaze Nystagmus test: fraudulent science in the American courts". Science & Justice. 44 (3): 133–9. doi:10.1016/S1355-0306(04)71705-0. PMID 15270451.
- Booker JL (2001). "End-position nystagmus as an indicator of ethanol intoxication". Science & Justice. 41 (2): 113–6. doi:10.1016/S1355-0306(01)71862-X. PMID 11393940.
- McKnight AJ, Langston EA, McKnight AS, Lange JE (May 2002). "Sobriety tests for low blood alcohol concentrations". Accident Analysis and Prevention. 34 (3): 305–11. doi:10.1016/S0001-4575(01)00027-6. PMID 11939359.
- Markley, BA (2007). "Introduction to electronystagmography for END technologists". American Journal of Electroneurodiagnostic Technology. 47 (3): 178–89. PMID 17982846.
- Mosca, F; Sicignano, S; Leone, CA (2003). "Benign positional paroxysmal vertigo: videonystagmographic study using rotatory test". Acta Otorhinolaryngologica Italica. 23 (2): 67–72. PMID 14526552.
- Eggert, T (2007). "Eye movement recordings: methods". Developments in Ophthalmology. 40: 15–34. doi:10.1159/0000100347. PMID 17314477.
- Serra A, Leigh RJ (December 2002). "Diagnostic value of nystagmus: spontaneous and induced ocular oscillations". Journal of Neurology, Neurosurgery, and Psychiatry. 73 (6): 615–8. doi:10.1136/jnnp.73.6.615. PMC . PMID 12438459.
- "Differences Between Physiologic and Pathologic Nystagmus". Spencer S. Eccles Health Sciences Library. Retrieved 22 November 2016.
- "nystagmus". Retrieved 2007-06-07.
- Anagnostou, E (2006). "Positional nystagmus and vertigo due to a solitary brachium conjunctivum plaque". Journal of Neurology, Neurosurgery & Psychiatry. 77 (6): 790–2. doi:10.1136/jnnp.2005.084624.
- Pierrot-Deseilligny C, Milea D (June 2005). "Vertical nystagmus: clinical facts and hypotheses". Brain. 128 (Pt 6): 1237–46. doi:10.1093/brain/awh532. PMID 15872015.
- "Sensory Reception: Human Vision: Structure and function of the Human Eye" vol. 27, p. 179 Encyclopædia Britannica, 1987
- Corbett, J (2007). "Memantine/Gabapentin for the treatment of congenital nystagmus". Current Neurology and Neuroscience Reports. 7 (5): 395–6. doi:10.1007/s11910-007-0061-z. PMID 17764629.
- Muscular Dystrophy Association Press Release
- 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, Nancy (March 15, 2006). "Many options to treat nystagmus, more in development". Ophthalmology Times.
- Biousse, V; Tusa, RJ; Russell, B; Azran, MS; Das, V; Schubert, MS; Ward, M; Newman, NJ (2004). "The use of contact lenses to treat visually symptomatic congenital nystagmus". Journal of Neurology, Neurosurgery & Psychiatry. 75 (2): 314–6. doi:10.1136/jnnp.2003.010678. PMC . PMID 14742616.
- Cerman, E. "Mini-telescopic eyeglasses suppress nystagmus". World Society of Pediatric Ophthalmology and Strabismus Conference in Barcelona 2015. Retrieved 26 January 2016.
- Kumar, Anand; Shetty, S; Vijayalakshmi, P; Hertle, RW (Nov–Dec 2011). "Improvement in visual acuity following surgery for correction of head posture in infantile nystagmus syndrome". J Pediatr Ophthalmol Strabismus. 48 (6): 341–6. doi:10.3928/01913913-20110118-02. PMID 21261243.
- Wang, Z; Dell'Osso, LF; Jacobs, JB; Burnstine, RA; Tomsak, RL (December 2006). "Effects of tenotomy on patients with infantile nystagmus syndrome: foveation improvement over a broadened visual field". J AAPOS. 10 (6): 552–60. doi:10.1016/j.jaapos.2006.08.021. PMID 17189150.
- Ishikawa, S., et al. (1987). Treatment of nystagmus by acupuncture. Highlights in neuro-ophthalmology, 6th ed. pg 227–232.
- Blekher, T. (1998). "Effect of acupuncture on foveation characteristics in congenital nystagmus". British Journal of Ophthalmology. 82:115–120. Accessed May 6th, 2012: 
- Sarvananthan, N.; Surendran, M.; Roberts, E. O.; Jain, S.; Thomas, S.; Shah, N.; Proudlock, F. A.; Thompson, J. R.; McLean, R. J.; Degg, C.; Woodruff, G.; Gottlob, I. (2009). "The Prevalence of Nystagmus: The Leicestershire Nystagmus Survey". Investigative Ophthalmology & Visual Science. 50 (11): 5201–6. doi:10.1167/iovs.09-3486. PMID 19458336.
|Look up Nystagmus in Wiktionary, the free dictionary.|
- GeneReview/NIH/UW entry on FRMD7-Related Infantile Nystagmus
- Physiologic Nystagmus at the US National Library of Medicine Medical Subject Headings (MeSH)