Abnormal Oculomotor Functions in Amyotrophic Lateral Sclerosis.
10.3988/jcn.2018.14.4.464
- Author:
Bong Hui KANG
1
;
Jae Il KIM
;
Young Min LIM
;
Kwang Kuk KIM
Author Information
1. Department of Neurology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. kkkim@amc.seoul.kr
- Publication Type:Original Article
- Keywords:
central nystagmus;
oculomotor dysfunction;
amyotrophic lateral sclerosis;
perverted head-shaking nystagmus;
central positional nystagmus;
direction-changing head-shaking nystagmus
- MeSH:
Amyotrophic Lateral Sclerosis*;
Brain;
Cerebellar Ataxia;
Cerebellum;
Eye Movements;
Head;
Humans;
Longitudinal Studies;
Motor Neurons;
Neurodegenerative Diseases;
Nystagmus, Physiologic;
Observational Study;
Pursuit, Smooth
- From:Journal of Clinical Neurology
2018;14(4):464-471
- CountryRepublic of Korea
- Language:English
-
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.