J Clin Neurol.  2018 Oct;14(4):464-471. 10.3988/jcn.2018.14.4.464.

Abnormal Oculomotor Functions in Amyotrophic Lateral Sclerosis

Affiliations
  • 1Department of Neurology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. kkkim@amc.seoul.kr
  • 2Department of Neurology, Dankook University College of Medicine, Dankook University Hospital, Cheonan, Korea.

Abstract

BACKGROUND AND PURPOSE
Although traditionally regarded as spared, a range of oculomotor dysfunction has been recognized in amyotrophic lateral sclerosis (ALS) patients. ALS is nowadays considered as a neurodegenerative disorder of a third compartment comprising widespread areas of extra-motor brain including cerebellum. Our objective was to perform an observational study to examine for ocular motor dysfunction in patients with ALS and for any differences between bulbar-onset and spinal-onset patients.
METHODS
Thirty two ALS patients (bulbar onset: 10, spinal onset: 22) underwent the standardized systemic evaluations using video-oculography.
RESULTS
Oculomotor dysfunctions such as square wave jerks, saccadic dysmetria, abnormal cogwheeling smooth pursuits and head shaking and positional nystagmus of central origin have been observed in the ALS patients at a relatively early stage. Abnormal smooth pursuits and saccadic dysmetria were increased in the bulbar-onset compared to the spinal-onset (p < 0.05).
CONCLUSIONS
These oculomotor abnormalities may be a marker of neuro-degeneration beyond motor neurons in ALS, especially in bulbar-onset disease. Future longitudinal studies of eye movement abnormalities have provided insights into the distribution and nature of the disease process.

Keyword

central nystagmus; oculomotor dysfunction; amyotrophic lateral sclerosis; perverted head-shaking nystagmus; central positional nystagmus; direction-changing head-shaking nystagmus

MeSH Terms

Amyotrophic Lateral Sclerosis*
Brain
Cerebellar Ataxia
Cerebellum
Eye Movements
Head
Humans
Longitudinal Studies
Motor Neurons
Neurodegenerative Diseases
Nystagmus, Physiologic
Observational Study
Pursuit, Smooth

Figure

  • Fig. 1 Examples of abnormal oculomotor functions in our patients with amyotrophic lateral sclerosis. A: Square-wave jerks, B: Abnormal smooth pursuits, and C: Saccadic hypometria and hypermetria.


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