Abnormal Oculomotor Functions in Amyotrophic Lateral Sclerosis
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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.
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.
참고문헌 (23)
- Brooks BR, "El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. subcommittee on motor neuron diseases", amyotrophic lateral sclerosis of the world federation of neurology research group on neuromuscular diseases and the El Escorial "clinical limits of amyotrophic lateral sclerosis" workshop contributors, 1994, pp.124(Suppl), 96~107
- Averbuch-Heller L, "Slow vertical saccades in motor neuron disease: correlation of structure and function", Ann Neurol, 44, pp.641~648 (1998)
- Prell T, "The involvement of the cerebellum in amyotrophic lateral sclerosis", Amyotroph Lateral Scler Frontotemporal Degener, 14, pp.507~515 (2013)
- Talbot PR, "Inter-relation between classic motor neuron disease and frontotemporal dementia: neuropsychological and single photon emission computed tomography study", J Neurol Neurosurg Psychiatry, 58, pp.541~547 (1995)
- McCluskey LF, "Amyotrophic lateral sclerosis-plus syndrome with TAR DNA-binding protein-43 pathology", Arch Neurol, 66, pp.121~124 (2009)
- Donaghy C, "Eye movements in amyotrophic lateral sclerosis and its mimics: a review with illustrative cases", J Neurol Neurosurg Psychiatry, 82, pp.110~116 (2011)
- Donaghy C, "Ocular fixation instabilities in motor neurone disease. a marker of frontal lobe dysfunction?", J Neurol, 256, pp.420~426 (2009)
- Palmowski A, "Eye movement in amyotrophic lateral sclerosis: a longitudinal study", Ger J Ophthalmol, 4, pp.355~362 (1995)
- Moon SY, "Slow vertical saccades in the frontotemporal dementia with motor neuron disease", J Neurol, 255, pp.1337~1343 (2008)
- Donaghy C, "Slow saccades in bulbar-onset motor neurone disease", J Neurol, 257, pp.1134~1140 (2010)
- Leveille A, "Eye movements in amyotrophic lateral sclerosis", Arch Neurol, 39, pp.684~686 (1982)
- Ohki M, "Ocular abnormalities in amyotrophic lateral sclerosis", Acta Otolaryngol Suppl, 511, pp.138~142 (1994)
- Abel LA, "Effects of stimulus velocity and acceleration on smooth pursuit in motor neuron disease", J Neurol, 242, pp.419~424 (1995)
- Jacobs L, "An eye movement disorder in amyotrophic lateral sclerosis", Neurology, 31, pp.1282~1287 (1981)
- Moss HE, "Cross-sectional evaluation of clinical neuro-ophthalmic abnormalities in an amyotrophic lateral sclerosis population", J Neurol Sci, 314, pp.97~101 (2012)
- Mizutani T, "Development of ophthalmoplegia in amyotrophic lateral sclerosis during long-term use of respirators", J Neurol Sci, 99, pp.311~319 (1990)
- Kushner MJ, "Nystagmus in motor neuron disease: clinicopathological study of two cases", Ann Neurol, 16, pp.71~77 (1984)
- Carvalho MD, "Awaji diagnostic algorithm increases sensitivity of El Escorial criteria for ALS diagnosis", Amyotroph Lateral Scler, 10, pp.53~57 (2009)
- Choi KD, "Head-shaking nystagmus in lateral medullary infarction: patterns and possible mechanisms", Neurology, 68, pp.1337~1344 (2007)
- Huh YE, "Patterns of spontaneous and head-shaking nystagmus in cerebellar infarction: imaging correlations", Brain, 134, pp.3662~3671 (2011)
- Choi JY, "Central paroxysmal positional nystagmus: characteristics and possible mechanisms", Neurology, 84, pp.2238~2246 (2015)
- 이태경, "중추성 체위성 현훈", Research in Vestibular Science, 10, pp.19~23 (2011), 대한평형의학회
- Schmidt D, "Myasthenia gravis: saccadic eye movement waveforms", Exp Neurol, 68, pp.346~364 (1980)