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Effect of 4-aminopyridine on gravity dependence and neural integrator function in patients with idiopathic downbeat nystagmus

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Abstract

Downbeat nystagmus (DBN) is a frequent sign in patients with cerebellar degeneration. It consists of an upward drift of the eye that does not depend on vertical head position (spontaneous drift, SD), a gravity-dependent component (GD), and a gaze-evoked drift reflecting gaze-holding impairment (deficient neural integrator function). The potassium-channel blocker 4-aminopyridine (4-AP) is reported to reduce DBN in patients with cerebellar atrophy but with little or no effect in patients with idiopathic DBN. We prospectively studied the effect of 4-AP on all three components in a large (n = 24) group of the clinically frequent idiopathic DBN. DBN was reduced by 22–31% when the head was off the head erect position. In contrast, there was no effect on vertical gaze-evoked drift. This indicates the therapeutic efficacy of 4-AP not only in patients with cerebellar atrophy but also in idiopathic DBN patients. This beneficial effect, which might be missed when gravity-dependent head positions are not tested, was not related to an improvement of gaze-holding deficit. We suggest it may be related to the restored inhibition of the overacting otolith−ocular reflex.

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Abbreviations

DBN:

Downbeat nystagmus

PC:

Purkinje cells

VOR:

Vestibulo-ocular reflex

AP:

Aminopyridines

SD:

Spontaneous vertical drift velocity on gaze straight ahead with the head erect

GD:

Gravity-dependent component

SPV:

Slow phase velocity

EPS:

Eye position sensitivity

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Acknowledgments

Dr. Sander is supported by the University Luebeck research grant (E 04-2009).

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Authors and Affiliations

  1. Department of Neurology, University Luebeck, Ratzeburger Allee 160, 23538, Luebeck, Germany

    T. Sander, A. Sprenger, T. Naumann & C. Helmchen

  2. University Hospital Zurich, Zurich, Switzerland

    S. Marti & D. Straumann

Authors
  1. T. Sander
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  2. A. Sprenger
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  3. S. Marti
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  4. T. Naumann
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  5. D. Straumann
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  6. C. Helmchen
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Correspondence to C. Helmchen.

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Sander, T., Sprenger, A., Marti, S. et al. Effect of 4-aminopyridine on gravity dependence and neural integrator function in patients with idiopathic downbeat nystagmus. J Neurol 258, 618–622 (2011). https://doi.org/10.1007/s00415-010-5806-y

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  • DOI: https://doi.org/10.1007/s00415-010-5806-y

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