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Oculomotor Dysfunction in Motor Neuron Disease.

Clover E Youn1, Christine Lu1, Jonathan Cauchi1

  • 1Icahn School of Medicine at Mount Sinai Downtown, Department of Neurology, New York, New York, USA.

Journal of Neuromuscular Diseases
|March 6, 2023
PubMed
Summary
This summary is machine-generated.

Oculomotor dysfunction (OD) is present in 64.2% of motor neuron disease (MND) patients, indicating it may serve as a clinical marker for advanced disease progression. Further research is needed to explore OD in MND.

Keywords:
Motor neuron diseaseamyotrophic lateral sclerosiseye movement data analysisophthalmoplegia

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Area of Science:

  • Neurology
  • Ophthalmology

Background:

  • Motor neuron disease (MND) typically spares eye movements, but emerging evidence suggests potential oculomotor dysfunction (OD).
  • Frontal lobe involvement is hypothesized due to oculomotor pathway anatomy and overlap between amyotrophic lateral sclerosis (ALS) and frontotemporal dementia.
  • This study investigated oculomotor characteristics in MND patients, particularly those with upper motor neuron (UMN) involvement or pseudobulbar affect (PBA).

Purpose of the Study:

  • To examine oculomotor characteristics in patients diagnosed with motor neuron disease (MND).
  • To determine the prevalence of oculomotor dysfunction (OD) in MND patients.
  • To explore the association between OD and UMN involvement or PBA in MND.

Main Methods:

  • A single-center prospective observational study involving bedside examinations of MND patients.
  • Administration of the Center for Neurologic Study-Liability Scale (CNS-LS) to screen for PBA.
  • Statistical analyses included Wilcoxon rank-sum and Fisher's exact tests to assess outcomes.

Main Results:

  • Oculomotor dysfunction (OD) was identified in 34 out of 53 (64.2%) MND patients during bedside evaluation.
  • No significant associations were found between the location of MND at presentation and the presence or type of OD.
  • OD was significantly associated with increased disease severity, indicated by reduced forced vital capacity (FVC).

Conclusions:

  • While no significant association was found between OD and UMN versus lower motor neuron disease at presentation, OD may serve as a valuable clinical marker for advanced stages of MND.
  • Oculomotor dysfunction is a notable finding in a majority of MND patients.
  • The utility of OD as an indicator of disease severity warrants further investigation.