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Acquired Nystagmus

  • Author: Christopher M Bardorf, MD, MS; Chief Editor: Edsel Ing, MD, FRCSC  more...
 
Updated: May 13, 2016
 

Background

Nystagmus may be defined as a periodic rhythmic ocular oscillation of the eyes. The oscillations may be sinusoidal and of approximately equal amplitude and velocity (pendular nystagmus) or, more commonly, with a slow initiating phase and a fast corrective phase (jerk nystagmus).[1, 2]

Nystagmus may be unilateral or bilateral, but, when the nystagmus appears unilateral, it is more often asymmetric rather than truly unilateral. Nystagmus may be conjugate or disconjugate (dissociated). It may be horizontal, vertical, torsional (rotary), or any combination of these movements superimposed upon each other.

Nystagmus may be congenital or acquired. When acquired, it most often is caused by abnormalities of vestibular input. Congenital forms may be associated with afferent visual pathway abnormalities (sensory nystagmus).[3]

To understand the mechanisms by which nystagmus may occur, it is important to discuss the means by which the nervous system maintains position of the eyes. Foveal centration of an object of regard is necessary to obtain the highest level of visual acuity. Three mechanisms are involved in maintaining foveal centration of an object of interest: fixation, the vestibulo-ocular reflex, and the neural integrator.

Fixation in the primary position involves the visual system's ability to detect drift of a foveating image and signal an appropriate corrective eye movement to refoveate the image of regard. The vestibular system is intimately and complexly involved with the oculomotor system.

The vestibulo-ocular reflex is a complex system of neural interconnections that maintains foveation of an object during changes in head position. The proprioceptors of the vestibular system are the semicircular canals of the inner ear. Three semicircular canals are present on each side, anterior, posterior, and horizontal. The semicircular canals respond to changes in angular acceleration due to head rotation.

The third mechanism is the neural integrator. When the eye is turned in an extreme position in the orbit, the fascia and ligaments that suspend the eye exert an elastic force to return toward the primary position. To overcome this force, a tonic contraction of the extraocular muscles is required. A gaze-holding network called the neural integrator generates the signal. The cerebellum, ascending vestibular pathways, and oculomotor nuclei are important components of the neural integrator.

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Pathophysiology

A disorder affecting any of the 3 mechanisms that control eye movements may result in nystagmus. Both focal and diffuse disorders may cause nystagmus. The characteristics of the nystagmus, as well as associated signs and symptoms help to localize the lesion and suggest possible etiologies.

Vestibular nystagmus

Vestibular nystagmus may be central or peripheral. Important differentiating features between central and peripheral nystagmus include the following: peripheral nystagmus is unidirectional with the fast phase opposite the lesion; central nystagmus may be unidirectional or bidirectional; purely vertical or torsional nystagmus suggests a central location; central vestibular nystagmus is not dampened or inhibited by visual fixation; tinnitus or deafness often is present in peripheral vestibular nystagmus, but it usually is absent in central vestibular nystagmus. According to Alexander's law, the nystagmus associated with peripheral lesions becomes more pronounced with gaze toward the side of the fast-beating component; with central nystagmus, the direction of the fast component is directed toward the side of gaze (eg, left-beating in left gaze, right-beating in right gaze, up-beating in upgaze).

Downbeat nystagmus

Downbeat nystagmus is defined as nystagmus with the fast phase beating in a downward direction.[4] The nystagmus usually is of maximal intensity when the eyes are deviated temporally and slightly inferiorly. With the eyes in this position, the nystagmus is directed obliquely downward. In most patients, removal of fixation (eg, by Frenzel goggles) does not influence slow phase velocity to a considerable extent; however, the frequency of saccades may diminish.[5]

The presence of downbeat nystagmus is highly suggestive of disorders of the craniocervical junction (eg, Arnold-Chiari malformation). This condition also may occur with bilateral lesions of the cerebellar flocculus and bilateral lesions of the medial longitudinal fasciculus, which carries optokinetic input from the posterior semicircular canals to the third nerve nuclei. It may also occur when the tone within pathways from the anterior semicircular canals is relatively higher than the tone within the posterior semicircular canals. Under such circumstances, the relatively unopposed neural activity from the anterior semicircular canals causes a slow upward pursuit movement of the eyes with a fast, corrective downward saccade.[6, 7]

Upbeat nystagmus

Upbeat nystagmus is defined as nystagmus with the fast phase beating in an upward direction. Daroff and Troost described 2 distinct types.[8] The first type consists of a large amplitude nystagmus that increases in intensity with upward gaze. This type is suggestive of a lesion of the anterior vermis of the cerebellum. The second type consists of a small amplitude nystagmus that decreases in intensity with upward gaze and increases in intensity with downward gaze. This type is suggestive of lesions of the medulla.[9]

This condition may occur when the tone within the pathways of the posterior semicircular canals is relatively higher than the tone within the anterior semicircular canals, and it can occur from lesions of the ventral tegmental tract or the brachium conjunctivum, which carry optokinetic input from the anterior semicircular canals to the third nerve nuclei.

Torsional (rotary) nystagmus

Torsional (rotary) nystagmus refers to a rotary movement of the globe about its anteroposterior axis. Torsional nystagmus is accentuated on lateral gaze. Most nystagmus resulting from dysfunction of the vestibular system has a torsional component superimposed on a horizontal or vertical nystagmus.

This condition occurs with lesions of the anterior and posterior semicircular canals on the same side (eg, lateral medullary syndrome). Lesions of the lateral medulla may produce a torsional nystagmus with the fast phase directed away from the side of the lesion. This type of nystagmus can be accentuated by otolithic stimulation by placing the patient on their side with the intact side down (eg, if the lesion is on the left, the nystagmus is accentuated when the patient is placed on his right side).

Pendular nystagmus

Pendular nystagmus is a multivectorial nystagmus (ie, horizontal, vertical, circular, elliptical) with an equal velocity in each direction that may reflect brain stem or cerebellar dysfunction. Often, there is marked asymmetry and dissociation between the eyes. The amplitude of the nystagmus may vary in different positions of gaze.[10, 11]

Horizontal nystagmus

Horizontal nystagmus is a well-recognized finding in patients with a unilateral disease of the cerebral hemispheres, especially with large, posterior lesions. It often is of low amplitude. Such patients show a constant velocity drift of the eyes toward the intact hemisphere with fast saccade directed toward the side of the lesion.

Seesaw nystagmus

Seesaw nystagmus is a pendular oscillation that consists of elevation and intorsion of one eye and depression and extorsion of the fellow eye that alternates every half cycle. This striking and unusual form of nystagmus may be seen in patients with chiasmal lesions, suggesting loss of the crossed visual inputs from the decussating fibers of the optic nerve at the level of the chiasm as the cause or lesions in the rostral midbrain. This type of nystagmus is not affected by otolithic stimulation.

Gaze-evoked nystagmus

Gaze-evoked nystagmus is produced by the attempted maintenance of an extreme eye position. It is the most common form of nystagmus. Gaze-evoked nystagmus is due to a deficient eye position signal in the neural integrator network. Thus, the eyes cannot be maintained at an eccentric orbital position and are pulled back toward primary position by the elastic forces of the orbital fascia. Then, corrective saccade moves the eyes back toward the eccentric position in the orbit.[12]

Gaze-evoked nystagmus may be caused by structural lesions that involve the neural integrator network, which is dispersed between the vestibulocerebellum, the medulla (region of the nucleus prepositus hypoglossi and adjacent medial vestibular nucleus [NPH/MVN]), and the interstitial nucleus of Cajal (INC). Patients recovering from a gaze palsy go through a period where they are able to gaze in the direction of the previous palsy, but they are unable to sustain gaze in that direction; therefore, the eyes drift slowly back toward primary position followed by a corrective saccade. When this is repeated, a gaze-evoked or gaze-paretic nystagmus results.

Gaze-evoked nystagmus often is encountered in healthy patients; in which case, it is called end-point nystagmus. End-point nystagmus usually can be differentiated from gaze-evoked nystagmus caused by disease, in that the former has lower intensity and, more importantly, is not associated with other ocular motor abnormalities.

Spasmus nutans

Spasmus nutans is a rare condition with the clinical triad of nystagmus, head nodding, and torticollis. Onset is from age 3-15 months with disappearance by 3 or 4 years. Rarely, it may be present to age 5-6 years. The nystagmus typically consists of small-amplitude, high frequency oscillations and usually is bilateral, but it can be monocular, asymmetric, and variable in different positions of gaze.

Periodic alternating nystagmus

Periodic alternating nystagmus is a conjugate, horizontal jerk nystagmus with the fast phase beating in one direction for a period of approximately 1-2 minutes. The nystagmus has an intervening neutral phase lasting 10-20 seconds; the nystagmus begins to beat in the opposite direction for 1-2 minutes; then, the process repeats itself. The presumed mechanism is disruption of the vestibulo-ocular tracts at the pontomedullary junction.[13]

Abducting nystagmus of internuclear ophthalmoplegia

Abducting nystagmus of internuclear ophthalmoplegia (INO) is, as the name implies, nystagmus in the abducting eye contralateral to a medial longitudinal fasciculus (MLF) lesion.

Convergence retraction nystagmoid movements

Dorsal midbrain lesions may be associated with convergence retraction nystagmoid movements. Although not a true nystagmus because it lacks slow phases, it is a potentially localizing eye movement disorder similar to nystagmus. Convergence retraction nystagmus may be elicited with upward saccades (downgoing optokinetic stimulus).

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Epidemiology

Mortality/Morbidity

Dependent upon etiology

Age

Onset of spasmus nutans is in infants aged 3-15 months with disappearance by age 3 or 4 years. Rarely, it may be present until children are aged 5-6 years.

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Contributor Information and Disclosures
Author

Christopher M Bardorf, MD, MS Ophthalmologist, Children's Eye Physicians

Christopher M Bardorf, MD, MS is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Enrique Garcia-Valenzuela, MD, PhD Clinical Assistant Professor, Department of Ophthalmology, University of Illinois Eye and Ear Infirmary; Consulting Staff, Vitreo-Retinal Surgery, Midwest Retina Consultants, SC, Parkside Center

Enrique Garcia-Valenzuela, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Society of Retina Specialists, Retina Society, Society for Neuroscience

Disclosure: Nothing to disclose.

Gregory Van Stavern, MD Attending Physician, Department of Ophthalmology and Neurology, Washington University School of Medicine

Gregory Van Stavern, MD is a member of the following medical societies: American Academy of Neurology, North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Edsel Ing, MD, FRCSC Associate Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Consulting Staff, Hospital for Sick Children and Sunnybrook Hospital

Edsel Ing, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Ophthalmic Plastic and Reconstructive Surgery, Royal College of Physicians and Surgeons of Canada, Canadian Ophthalmological Society, North American Neuro-Ophthalmology Society, Canadian Society of Oculoplastic Surgery, European Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Medical Association, Ontario Medical Association, Statistical Society of Canada, Chinese Canadian Medical Society

Disclosure: Nothing to disclose.

Additional Contributors

Michael J Bartiss, OD, MD Medical Director, Ophthalmology, Family Eye Care of the Carolinas and Surgery Center of Pinehurst

Michael J Bartiss, OD, MD is a member of the following medical societies: American Academy of Ophthalmology, North Carolina Medical Society, American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus

Disclosure: Nothing to disclose.

Acknowledgements

Brian R Younge, MD Professor of Ophthalmology, Mayo Clinic School of Medicine

Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

References
  1. Rucker JC. An update on acquired nystagmus. Semin Ophthalmol. 2008 Mar-Apr. 23(2):91-7. [Medline].

  2. Shaikh AG, Thurtell MJ, Optican LM, Leigh RJ. Pharmacological tests of hypotheses for acquired pendular nystagmus. Ann N Y Acad Sci. 2011 Sep. 1233:320-6. [Medline]. [Full Text].

  3. Sarvananthan N, Surendran M, Roberts E, et al. The prevalence of nystagmus: The Leicestershire nystagmus survey. Invest Ophthalmol Vis Sci. 2009 May 20. [Medline].

  4. Wagner JN, Glaser M, Brandt T, Strupp M. Downbeat nystagmus: aetiology and comorbidity in 117 patients. J Neurol Neurosurg Psychiatry. 2008 Jun. 79(6):672-7. [Medline].

  5. Thurtell MJ, Leigh RJ. Nystagmus and saccadic intrusions. Handb Clin Neurol. 2011. 102:333-78. [Medline].

  6. Schniepp R, Wuehr M, Huth S, Pradhan C, Schlick C, Brandt T, et al. The gait disorder in downbeat nystagmus syndrome. PLoS One. 2014. 9(8):e105463. [Medline]. [Full Text].

  7. Feil K, Claaßen J, Bardins S, Teufel J, Krafczyk S, Schneider E, et al. Effect of chlorzoxazone in patients with downbeat nystagmus: a pilot trial. Neurology. 2013 Sep 24. 81(13):1152-8. [Medline].

  8. Daroff RB, Troost BT. Upbeat nystagmus. JAMA. 1973 Jul 16. 225(3):312. [Medline].

  9. Gonzalez C, Seth RK, Ramos-Esteban JC. Change in head posture and character of nystagmus in a patient with neurological upbeat nystagmus. Binocul Vis Strabismus Q. 2007. 22(3):179-84. [Medline].

  10. Spielmann AC. Large recession of the four vertical rectus muscles for acquired pendular vertical nystagmus and oscillopsia without a null zone. J AAPOS. 2009 Feb. 13(1):102-4. [Medline].

  11. Beh SC, Tehrani AS, Kheradmand A, Zee DS. Damping of monocular pendular nystagmus with vibration in a patient with multiple sclerosis. Neurology. 2014 Apr 15. 82(15):1380-1. [Medline]. [Full Text].

  12. Thurtell MJ, Weber KP, Halmagyi GM. Teaching video NeuroImage: acquired or congenital gaze-evoked nystagmus?. Neurology. 2008 Jun 3. 70(23):e96. [Medline].

  13. Murofushi T, Chihara Y, Ushio M, Iwasaki S. Periodic alternating nystagmus in Meniere's disease: the peripheral type?. Acta Otolaryngol. 2008 Jul. 128(7):824-7. [Medline].

  14. Tilikete C, Jasse L, Pelisson D, Vukusic S, Durand-Dubief F, Urquizar C, et al. Acquired pendular nystagmus in multiple sclerosis and oculopalatal tremor. Neurology. 2011 May 10. 76(19):1650-7. [Medline].

  15. Thurtell MJ, Leigh RJ. Treatment of nystagmus. Curr Treat Options Neurol. 2012 Feb. 14(1):60-72. [Medline].

  16. Menon GJ, Thaller VT. Therapeutic external ophthalmoplegia with bilateral retrobulbar botulinum toxin- an effective treatment for acquired nystagmus with oscillopsia. Eye. 2002 Nov. 16(6):804-6. [Medline].

  17. Castillo IG, Reinecke RD, Sergott RC, Wizov S. Surgical treatment of trauma-induced periodic alternating nystagmus. Ophthalmology. 2004 Jan. 111(1):180-3. [Medline].

  18. Greven MA, Nelson LB. Four-muscle tenotomy surgery for nystagmus. Curr Opin Ophthalmol. 2014 Sep. 25 (5):400-5. [Medline].

  19. Kumar A, Thomas S, McLean R, et al. Treatment of acquired periodic alternating nystagmus with memantine: a case report. Clin Neuropharmacol. 2009 Mar-Apr. 32(2):109-10. [Medline].

  20. Cestari DM, Chan K, Tajouri N, Rizzo JF 3rd. The use of onabotulinum toxin a in the treatment of see-saw nystagmus. J Pediatr Ophthalmol Strabismus. 2010 Jul 22. 47 Online:e1-3. [Medline].

  21. American Academy of Ophthalmology. Basic and Clinical Science Course. Neuro-ophthalmology. 1999-2000. 5: 139-41.

  22. American Academy of Ophthalmology. Basic and Clinical Science Course. Pediatric. 1999-2000. 69(3): 129-35.

  23. American Medical Association. Drug Evaluations. 1995.

  24. Arnoldi KA, Tychsen L. Prevalence of intracranial lesions in children initially diagnosed with disconjugate nystagmus (spasmus nutans). J Pediatr Ophthalmol Strabismus. 1995 Sep-Oct. 32(5):296-301. [Medline].

  25. Averbuch-Heller L, Tusa RJ, Fuhry L, et al. A double-blind controlled study of gabapentin and baclofen as treatment for acquired nystagmus. Ann Neurol. 1997 Jun. 41(6):818-25. [Medline].

  26. Baloh RW, Spooner JW. Downbeat nystagmus: a type of central vestibular nystagmus. Neurology. 1981 Mar. 31(3):304-10. [Medline].

  27. Breen LA. Nystagmus and related ocular oscillations. Neuroophthalmology: Clinical Signs and Symptoms. 1997. Vol 4: 504-20.

  28. Cross SA, Smith JL, Norton EW. Periodic alternating nystagmus clearing after vitrectomy. J Clin Neuroophthalmol. 1982 Mar. 2(1):5-11. [Medline].

  29. Donin JF. Acquired monocular nystagmus in children. Can J Ophthalmol. 1967 Jul. 2(3):212-5. [Medline].

  30. Glaser JS. Neuro-Ophthalmology. 1990.

  31. Leigh RJ, Robinson DA, Zee DS. A hypothetical explanation for periodic alternating nystagmus: instability in the optokinetic-vestibular system. Ann N Y Acad Sci. 1981. 374:619-35. [Medline].

  32. Martin JH. Neuroanatomy Atlas. 2nd ed. McGraw-Hill Co; 1996. 155-61.

  33. May EF, Truxal AR. Loss of vision alone may result in seesaw nystagmus. J Neuroophthalmol. 1997 Jun. 17(2):84-5. [Medline].

  34. Neely DE, Sprunger DT. Nystagmus. Curr Opin Ophthalmol. 1999 Oct. 10(5):320-6. [Medline].

  35. Nolte J. The Human Brain. 1993. 218-23.

  36. Norton EW, Cogan DG. Spasmus nutans; a clinical study of twenty cases followed two years or more since onset. AMA Arch Ophthalmol. 1954 Sep. 52(3):442-6. [Medline].

  37. Pratt-Johnson JA, Tillson G. Management of Strabismus and Amblyopia: A Practical Guide. 1994.

  38. Ruben ST, Lee JP, O'Neil D, Dunlop I, Elston JS. The use of botulinum toxin for treatment of acquired nystagmus and oscillopsia. Ophthalmology. 1994 Apr. 101(4):783-7. [Medline].

  39. Sharpe JA, Lo AW, Rabinovitch HE. Control of the saccadic and smooth pursuit systems after cerebral hemidecortication. Brain. 1979 Jun. 102(2):387-403. [Medline].

  40. Stahl JS, Averbuch-Heller L, Leigh RJ. Acquired nystagmus. Arch Ophthalmol. 2000 Apr. 118(4):544-9. [Medline].

  41. Troost BT. Nystagmus and Related Ocular Oscillations. Available at http://www.waduicenter.com/?page_id=1341.

  42. Tusa RJ. Nystagmus: diagnostic and therapeutic strategies. Semin Ophthalmol. 1999 Jun. 14(2):65-73. [Medline].

  43. Tychsen L. Pediatric Ocular Motility Disorders of Neuro-ophthalmic Significance. 1991. Vol. 4: 615-43.

  44. Van Stavern GP, Biousse V, Newman NJ, Leingang JC. Downbeat nystagmus from heat stroke. J Neurol Neurosurg Psychiatry. 2000 Sep. 69(3):403-4. [Medline]. [Full Text].

  45. Sprenger A, Zils E, Rambold H, Sander T, Helmchen C. Effect of 3,4-diaminopyridine on the postural control in patients with downbeat nystagmus. Ann N Y Acad Sci. 2005 Apr. 1039:395-403. [Medline].

 
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