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Original article
‘Staircase’ square-wave jerks in early Parkinson's disease
  1. Aasef G Shaikh1,
  2. Minnan Xu-Wilson2,
  3. Stephen Grill3,
  4. David S Zee1
  1. 1Department of Neurology, The Johns Hopkins University, Baltimore, Maryland, USA
  2. 2Department of Biomedical Engineering, The Johns Hopkins University, Baltimore, Maryland, USA
  3. 3Parkinson's & Movement Disorders Center of Maryland, Elkridge, Maryland, USA
  1. Correspondence to Dr Aasef G Shaikh, Department of Neurology, The Johns Hopkins University, 600 North Wolfe Street, Path. 2-210, Baltimore, MD 21287, USA; ashaikh{at}dizzy.med.jhu.edu

Abstract

Background/aims Visually guided saccades and gaze-fixation ability were recorded in patients with early Parkinson's disease (PD).

Methods Magnetic search coil system was used to measure horizontal and vertical eye positions.

Results ‘Staircase’ visually guided saccades (multiple hypometric saccades separated by an intersaccadic interval) and ‘staircase’ square-wave jerks (SWJ) were present in all patients. The SWJ amplitude (total amplitude of the series of hypometric saccades comprising the SWJ) was abnormally large (mean 2°). SWJ frequency was also abnormally high (50–70 intrusions/min).

Conclusion Loss of dopaminergic neurons in the substantia nigra pars compacta leading to phasic excitation of the substantia nigra pars reticulata and, in turn, phasic inhibition of the superior colliculus (SC), may account for ‘staircase’ visually guided saccades in these patients. This mechanism, however, does not explain abnormally large SWJ, which in the traditional view results from decreased inhibition upon SC. The abnormally large SWJ could be due to a compensatory increase in frontal eye field activity secondary to the increased inhibition upon the SC. Abnormally large and frequent SWJ are often used clinically to distinguish multi-system atrophy from idiopathic PD. Our study, however, suggests that idiopathic PD cannot be excluded if the patient has large-amplitude SWJ.

  • Saccade
  • intrusion
  • superior colliculus
  • basal ganglia
  • movement disorder
  • vision
  • visual pathway
  • visual (cerebral) cortex
  • physiology
  • degeneration

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Footnotes

  • Funding National Institutes of Health; gift funds to the JHH vestibular laboratory; the Leon Levy Foundation.

  • Competing interests None to declare.

  • Patient consent Obtained.

  • Ethics approval This study was conducted with the approval of the The Johns Hopkins University.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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