Klin Monbl Augenheilkd 2021; 238(11): 1186-1195
DOI: 10.1055/a-1525-0030
Übersicht

Nystagmus: Diagnosis, Topographic Anatomical Localization and Therapy

Article in several languages: English | deutsch
1   Neurologische Klinik der Ludwig-Maximilians-Universität München, Deutschland
2   Deutsches Schwindel- und Gleichgewichtszentrum der Ludwig-Maximilians-Universität München, Deutschland
,
Dominik Straumann
3   Klinik für Neurologie, Universitätsspital Zürich, Schweiz
,
Christoph Helmchen
4   Neurologische Klinik der Universität zu Lübeck, Deutschland
› Author Affiliations

Abstract

Nystagmus is defined as rhythmic, most often involuntary eye movements. It normally consists of a slow (pathological) drift of the eyes, followed by a fast central compensatory movement back to the primary position (refixation saccade). The direction, however, is reported according to the fast phase. The cardinal symptoms are, on the one hand, blurred vision, jumping images (oscillopsia), reduced visual acuity and, sometimes, double vision; many of these symptoms depend on the eye position. On the other hand, depending on the etiology, patients may suffer from the following symptoms: 1. permanent dizziness, postural imbalance, and gait disorder (typical of downbeat and upbeat nystagmus); 2. if the onset of symptoms is acute, the patient may experience spinning vertigo with a tendency to fall to one side (due to ischemia in the area of the brainstem or cerebellum with central fixation nystagmus or as acute unilateral vestibulopathy with spontaneous peripheral vestibular nystagmus); or 3. positional vertigo. There are two major categories: the first is spontaneous nystagmus, i.e., nystagmus which occurs in the primary position as upbeat or downbeat nystagmus; and the second includes various types of nystagmus which are induced or modified by certain factors. Examples are gaze-evoked nystagmus, head-shaking nystagmus, positional nystagmus, and hyperventilation-induced nystagmus. In addition, there are disorders similar to nystagmus, such as ocular flutter or opsoclonus. The most common central types of spontaneous nystagmus are downbeat and upbeat, infantile, pure torsional, pendular fixation, periodic alternating, and seesaw nystagmus. Many types of nystagmus allow a precise neuroanatomical localization: for instance, downbeat nystagmus, which is most often caused by a bilateral floccular lesion or dysfunction, or upbeat nystagmus, which is caused by a lesion in the midbrain or medulla. Examples of drug treatment are the use of 4-aminopyridine for downbeat and upbeat nystagmus, memantine or gabapentin for pendular fixation nystagmus, or baclofen for periodic alternating nystagmus. In this article we are focusing on nystagmus. In a second article we will focus on central ocular motor disorders, such as saccade or gaze palsy, internuclear ophthalmoplegia, and gaze-holding deficits. Therefore, these types of eye movements will not be described here in detail.



Publication History

Received: 22 May 2021

Accepted: 09 July 2021

Article published online:
16 November 2021

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  • References/Literatur

  • 1 Strupp M, Brandt T, Dieterich M. Vertigo and Dizziness – common Complaints. 3rd ed.. ed. London: Springer; 2022
  • 2 Eggers SDZ, Bisdorff A, von Brevern M. et al. Classification of vestibular signs and examination techniques: Nystagmus and nystagmus-like movements. J Vestib Res 2019; 29: 57-87
  • 3 Leigh RJ, Zee D. The Neurology of Eye Movements. 5th ed. Oxford, New York: Oxford University Press; 2015
  • 4 Strupp M, Thurtell MJ, Shaikh AG. et al. Pharmacotherapy of vestibular and ocular motor disorders, including nystagmus. J Neurol 2011; 258: 1207-1222
  • 5 Strupp M, Kremmyda O, Adamczyk C. et al. Central ocular motor disorders, including gaze palsy and nystagmus. J Neurol 2014; 261 (Suppl. 02) S542-S558
  • 6 Strupp M, Hüfner K, Sandmann R. et al. Central oculomotor disturbances and nystagmus: a window into the brainstem and cerebellum. Dtsch Arztebl Int 2011; 108: 197-204
  • 7 Strupp M, Fischer C, Hanss L. et al. The takeaway Frenzel goggles: a Fresnel-based device. Neurology 2014; 83: 1241-1245
  • 8 Halmagyi GM, McGarvie LA, Strupp M. Nystagmus goggles: how to use them, what you find and what it means. Pract Neurol 2020; 20: 446-450
  • 9 Strupp M. Nystagmus – Schritt für Schritt. Neurologie up2date 2018; 1: 17-21
  • 10 von Brevern M, Bertholon P, Brandt T. et al. Benign paroxysmal positional vertigo: Diagnostic criteria. J Vestib Res 2015; 25: 105-117
  • 11 Buttner U, Brandt T, Helmchen C. The direction of nystagmus is important for the diagnosis of central paroxysmal positioning nystagmus (cPPV). Neuroophthalmology 2000; 21: 97-104
  • 12 Feil K, Strobl R, Schindler A. et al. What Is Behind Cerebellar Vertigo and Dizziness?. Cerebellum 2019; 18: 320-332
  • 13 Wagner JN, Glaser M, Brandt T. et al. Downbeat nystagmus: aetiology and comorbidity in 117 patients. J Neurol Neurosurg Psychiatry 2008; 79: 672-677
  • 14 Hüfner K, Frenzel C, Kremmyda O. et al. Esophoria or esotropia in adulthood: a sign of cerebellar dysfunction?. J Neurol 2015; 262: 585-592
  • 15 Zee DS, Friendlich AR, Robinson DA. The mechanism of downbeat nystagmus. Arch Neurol 1974; 30: 227-237
  • 16 Kalla R, Deutschlander A, Hufner K. et al. Detection of floccular hypometabolism in downbeat nystagmus by fMRI. Neurology 2006; 66: 281-283
  • 17 Wagner J, Lehnen N, Glasauer S. et al. Downbeat nystagmus caused by a paramedian ponto-medullary lesion. J Neurol 2009; 256: 1572-1574
  • 18 Strupp M, Schüler O, Krafczyk S. et al. Treatment of downbeat nystagmus with 3,4-diaminopyridine: a placebo-controlled study. Neurology 2003; 61: 165-170
  • 19 Kalla R, Glasauer S, Büttner U. et al. 4-aminopyridine restores vertical and horizontal neural integrator function in downbeat nystagmus. Brain 2007; 130: 2441-2451
  • 20 Helmchen C, Sprenger A, Rambold H. et al. Effect of 3,4-diaminopyridine on the gravity dependence of ocular drift in downbeat nystagmus. Neurology 2004; 63: 752-753
  • 21 Claassen J, Spiegel R, Kalla R. et al. A randomised double-blind, cross-over trial of 4-aminopyridine for downbeat nystagmus – effects on slowphase eye velocity, postural stability, locomotion and symptoms. J Neurol Neurosurg Psychiatry 2013; 84: 1392-1399
  • 22 Bense S, Best C, Buchholz HG. et al. 18F-fluorodeoxyglucose hypometabolism in cerebellar tonsil and flocculus in downbeat nystagmus. Neuroreport 2006; 17: 599-603
  • 23 Etzion Y, Grossman Y. Highly 4-aminopyridine sensitive delayed rectifier current modulates the excitability of guinea pig cerebellar Purkinje cells. Exp Brain Res 2001; 139: 419-425
  • 24 Feil K, Claassen J, Bardins S. et al. Effect of chlorzoxazone in patients with downbeat nystagmus: a pilot trial. Neurology 2013; 81: 1152-1158
  • 25 Glasauer S, Kalla R, Büttner U. et al. 4-aminopyridine restores visual ocular motor function in upbeat nystagmus. J Neurol Neurosurg Psychiatry 2005; 76: 451-453
  • 26 Schlindwein P, Schreckenberger M, Dieterich M. Visual-motion suppression in congenital pendular nystagmus. Ann N Y Acad Sci 2009; 1164: 458-460
  • 27 Hüfner K, Stephan T, Flanagin VL. et al. Cerebellar and visual gray matter brain volume increases in congenital nystagmus. Front Neurol 2011; 2: 60
  • 28 Frühwald MC, Rutkowski S. Tumors of the central nervous system in children and adolescents. Dtsch Arztebl Int 2011; 108: 390-397
  • 29 Shaikh AG, Hong S, Liao K. et al. Oculopalatal tremor explained by a model of inferior olivary hypertrophy and cerebellar plasticity. Brain 2010; 133: 923-940