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Related Experiment Videos

[Multiple system atrophy with macro square wave jerks and pendular nystagmus].

K Yamamoto1, T Fukusako, H Nogaki

  • 1Department of Neurology, Yamaguchi University School of Medicine.

Rinsho Shinkeigaku = Clinical Neurology
|November 1, 1992
PubMed
Summary

This study describes a patient with progressive neurological decline, developing gait issues and abnormal eye movements like macro square wave jerks. These symptoms suggest a potential dysfunction in the GABAergic system impacting eye movement control.

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Area of Science:

  • Neuroscience
  • Ophthalmology
  • Neurology

Background:

  • A 51-year-old woman presented with progressive gait disturbance and dysuria.
  • Neurological examination indicated ataxia, orthostatic hypotension, rigidity, hyperreflexia, and horizontal gaze nystagmus.

Observation:

  • The patient developed abnormal eye movements, including vertical, horizontal, or oblique macro square wave jerks and pendular nystagmus.
  • Macro square wave jerks occurred during fixation and were suppressed by eye closure or darkness.
  • Pendular nystagmus appeared with fixation changes or disappearance of the fixation point.

Findings:

  • Macro square wave jerks had a duration of ~200 msec and amplitude of 10–15 degrees.
  • Pendular nystagmus had a duration of several seconds and amplitude of 5–15 degrees.

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  • Both eye movement abnormalities showed partial suppression with phenobarbital, sodium valproate, or baclofen, and near-complete suppression with diazepam or clonazepam.

    • Implications:

    • The observed macro square wave jerks and pendular nystagmus may stem from GABAergic system dysfunction within the saccadic eye movement system.
    • This case highlights the potential role of GABAergic pathways in complex eye movement disorders.
    • Pharmacological responses suggest GABAergic modulation as a therapeutic avenue for similar conditions.