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The Cerebellum

, Volume 12, Issue 4, pp 557–567 | Cite as

Saccades and Eye–Head Coordination in Ataxia with Oculomotor Apraxia Type 2

  • Muriel PanouillèresEmail author
  • Solène Frismand
  • Olivier Sillan
  • Christian Urquizar
  • Alain Vighetto
  • Denis Pélisson
  • Caroline Tilikete
Original Paper

Abstract

Ataxia with oculomotor apraxia type 2 (AOA2) is one of the most frequent autosomal recessive cerebellar ataxias. Oculomotor apraxia refers to horizontal gaze failure due to deficits in voluntary/reactive eye movements. These deficits can manifest as increased latency and/or hypometria of saccades with a staircase pattern and are frequently associated with compensatory head thrust movements. Oculomotor disturbances associated with AOA2 have been poorly studied mainly because the diagnosis of oculomotor apraxia was based on the presence of compensatory head thrusts. The aim of this study was to characterise the nature of horizontal gaze failure in patients with AOA2 and to demonstrate oculomotor apraxia even in the absence of head thrusts. Five patients with AOA2, without head thrusts, were tested in saccadic tasks with the head restrained or free to move and their performance was compared to a group of six healthy participants. The most salient deficit of the patients was saccadic hypometria with a typical staircase pattern. Saccade latency in the patients was longer than controls only for memory-guided saccades. In the head-free condition, head movements were delayed relative to the eye and their amplitude and velocity were strongly reduced compared to controls. Our study emphasises that in AOA2, hypometric saccades with a staircase pattern are a more reliable sign of oculomotor apraxia than head thrust movements. In addition, the variety of eye and head movements’ deficits suggests that, although the main neural degeneration in AOA2 affects the cerebellum, this disease affects other structures.

Keywords

AOA2 Oculomotor apraxia Staircase saccade Eye–head coordination Cerebellum 

Notes

Acknowledgments

The authors thank the patients and the healthy subjects for contributing to this study. The authors thank Pr S. Thobois, Dr I. Roullet-Solignac and Dr A. Poisson for referring the patients. The authors are really grateful to Pr. D. Zee for a helpful discussion. This work was promoted by the Hospices Civils de Lyon (2002–303).

Conflict of interest

The authors declare that there is no conflict of interest

Supplementary material

12311_2013_463_MOESM1_ESM.doc (34 kb)
ESM 1 (DOC 34 kb)

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Muriel Panouillères
    • 1
    • 3
    • 4
    Email author
  • Solène Frismand
    • 2
  • Olivier Sillan
    • 1
    • 3
  • Christian Urquizar
    • 1
    • 3
  • Alain Vighetto
    • 1
    • 2
    • 3
  • Denis Pélisson
    • 1
    • 3
  • Caroline Tilikete
    • 1
    • 2
    • 3
  1. 1.INSERM U1028; CNRS UMR5292; Lyon Neuroscience Research Center, ImpAct TeamBronFrance
  2. 2.Unité de Neuro-ophtalmologie and Service de Neurologie D, Hôpital NeurologiqueHospices Civils de LyonBronFrance
  3. 3.University Lyon 1LyonFrance
  4. 4.INSERM U1028; CNRS UMR5292; Lyon Neuroscience Research Center, ImpAct TeamBron CedexFrance

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