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Case Report
Two Cases of Myasthenia Gravis Showing Fatigibility Presenting with Decreased Gain of Smooth Pursuit
Ju-Hee Chae, Hyun-June Shin, Byoung-Soo Shin, Man-Wook Seo, Sun-Young Oh
Res Vestib Sci. 2019;18(2):54-58.   Published online June 15, 2019
DOI: https://doi.org/10.21790/rvs.2019.18.2.54
  • 5,236 View
  • 361 Download
  • 1 Crossref
AbstractAbstract PDF
Myasthenia gravis (MG) is an autoimmune disease caused by autoantibodies to the acetylcholine receptors of the neuromuscular junction characterized by weakness and abnormal fatigability of the muscles. Therefore, the diagnosis of MG depends on the recognition of this distinctive pattern of fatigable weakness. Previous studies presented the diagnostic efficacy of saccadic eye movements in patients with ocular MG. We here in report 2 patients of ocular MG showing the fatigue effects during repetitive sustained smooth pursuit, and the effects of the administration of edrophonium on myasthenic smooth pursuit. Changes in smooth pursuits reflecting peripheral and secondary central mechanisms were demonstrated.

Citations

Citations to this article as recorded by  
  • Tracking Eye Movements for Diagnosis in Myasthenia Gravis: A Comprehensive Review
    Minh N. L. Nguyen, Anneke van der Walt, Joanne Fielding, Meaghan Clough, Owen B. White
    Journal of Neuro-Ophthalmology.2022; 42(4): 428.     CrossRef
5
원활추종운동 이득감소로 근피로도를 확인한 중증근무력증 2예
Ju-Hee Chae, Hyun-June Shin, Byoung-Soo Shin, Man-Wook Seo, Sun-Young Oh
Received April 9, 2019  Accepted June 3, 2019  Published online June 3, 2019  
   [Accepted]
  • 809 View
  • 0 Download
AbstractAbstract
Myasthenia Gravis (MG) is an autoimmune disease caused by autoantibodies to the acetylcholine receptors of the neuromuscular junction characterized by weakness and abnormal fatigability of the muscles. Therefore, the diagnosis of MG depends on the recognition of this distinctive pattern of fatigable weakness. Previous studies presented the diagnostic efficacy of saccadic eye movements in patients with ocular MG. We here in report two patients of ocular MG showing the fatigue effects during repetitive sustained smooth pursuit, and the effects of the administration of edrophonium on myasthenic smooth pursuit. Changes in smooth pursuits reflecting peripheral and secondary central mechanisms were demonstrated.
Case Report
Visuospatial Dysfunction in Patients With the Right Vestibular Neuritis
Seung-Ho Jeon, Ko-Woon Kim, Hyun-June Shin, Byoung-Soo Shin, Man-Wook Seo, Sun-Young Oh
Res Vestib Sci. 2019;18(1):19-23.   Published online March 15, 2019
DOI: https://doi.org/10.21790/rvs.2019.18.1.19
  • 5,428 View
  • 85 Download
  • 1 Crossref
AbstractAbstract PDF
Acute vestibular neuritis (VN) is characterized by acute/subacute vertigo with spontaneous nystagmus and unilateral loss of semicircular canal function. Vestibular system in human is represented in the brain bilaterally with functional asymmetries of the right hemispheric dominance in the right handers. Spatial working memory entails the ability to keep spatial information active in working memory over a short period of time which is also known as the right hemispheric dominance. Three patients (patient 1, 32-year-old female; patient 2, 18-year-old male; patient 3, 63-year-old male) suffered from acute onset of severe vertigo, nausea and vomiting. Patients 1 and 2’s examination revealed VN on the right side showing spontaneous left beating nystagmus and impaired vestibular ocular reflex on the right side in video head-impulse and caloric tests. Patient 3’s finding was fit for VN on the left side. We also evaluated visuospatial memory function with the block design test in these 3 VN patients which discovered lower scores in patients 1 and 2 and the average level in patient 3 compare to those of healthy controls. Follow-up block design test after resolved symptoms showed within normal range in both patients. Our cases suggest that the patients with unilateral peripheral vestibulopathy may have an asymmetrical effect on the higher vestibular cognitive function. The right VN can be associated with transient visuospatial memory dysfunction. These findings add the evidence of significant right hemispheric dominance for vestibular and visuospatial structures in the right-handed subjects, and of predominant dysfunction in the hemisphere ipsilateral to the peripheral lesion side.

Citations

Citations to this article as recorded by  
  • The Differential Effects of Acute Right- vs. Left-Sided Vestibular Deafferentation on Spatial Cognition in Unilateral Labyrinthectomized Mice
    Thanh Tin Nguyen, Gi-Sung Nam, Jin-Ju Kang, Gyu Cheol Han, Ji-Soo Kim, Marianne Dieterich, Sun-Young Oh
    Frontiers in Neurology.2021;[Epub]     CrossRef
5
Visuospatial dysfunction in patients with the right vestibular neuronitis
Seung-Ho Jeon, Sun-Young Oh, Ko-Woon Kim, Hyun-June Shin, Byoung-soo Shin, Man-Wook Seo
Received August 3, 2018  Accepted October 1, 2018  Published online October 1, 2018  
   [Accepted]
  • 1,294 View
  • 0 Download
AbstractAbstract
Acute vestibular neuritis is characterized by acute/subacute vertigo with spontaneous nystagmus and unilateral loss of semicircular canal function. Vestibular system in human is represented in the brain bilaterally with functional asymmetries of the right hemispheric dominance in the right handers. Spatial working memory entails the ability to keep spatial information active in working memory over a short period of time which is also known as the right hemispheric dominance. Three patients (Pt 1, 32-year-old female; Pt 2, 18-year-old male; Pt 3, 63-year-old male) suffered from acute onset of vertigo. Pt 1 and 2’s examination revealed vestibular neuritis (VN) on the right side showing spontaneous left beating nystagmus and impaired vestibular ocular reflex (VOR) on the right side in video head-impulse and caloric tests. Pt 3’s finding was fit for VN on the left side. We also evaluated visuospatial memory function with the block design test in these three VN patients which discovered lower scores in Pt 1 and 2 and the average level in Pt 3 compare to those of healthy controls. Follow up block design test after resolved symptoms showed within normal range in both patients. Our cases suggest that the patients with unilateral peripheral vestibulopathy may have an asymmetrical effect on the higher vestibular cognitive function. The right VN can be associated with transient visuospatial memory dysfunction. These findings add the evidence of significant right hemispheric dominance for vestibular and visuospatial structures in the right-handed subjects, and of predominant dysfunction in the hemisphere ipsilateral to the peripheral lesion side.
Case Reports
Brain Hypometabolism in Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome
Byoung-Min Jeong, Byoung-Soo Shin, Man-Wook Seo, Ji-Yun Park, Hwan-Jeong Jeong, Sun-Young Oh
Res Vestib Sci. 2017;16(4):156-160.   Published online December 15, 2017
DOI: https://doi.org/10.21790/rvs.2017.16.4.156
  • 7,559 View
  • 141 Download
AbstractAbstract PDF
Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a slowing progressive ataxic disorder characterized by bilateral vestibulopathy, cerebellar ataxia and somatosensory impairment. Autonomic dysfunction is recently considered as a core feature in CANVAS in addition to these symptoms. In most cases, patients with CANVAS show cerebellar atrophy in brain imaging, but some cases show minimal or no atrophy of cerebellum. Brain (18F)-fluoro-2-deoxy-D-glucose positron emission tomography (18F-FDG PET) study can be a complimentary tool to diagnosis CANVAS in cases of no structural abnormality such as cerebellar atrophy. Hereby, we present a case of CANVAS with minimal atrophy of cerebellum but showing a prominent hypometabolism in cerebellum, thalamus and posterior cingulate cortex in 18F-FDG PET.
Four Cases of Wernicke’s Encephalopathy with Impaired Horizontal Vestibular Ocular Reflexes
Hyuk-Su Jang, Byoung-Soo Shin, Man-Wook Seo, Sun-Young Oh
Res Vestib Sci. 2017;16(2):57-63.   Published online June 15, 2017
DOI: https://doi.org/10.21790/rvs.2017.16.2.57
  • 8,124 View
  • 219 Download
AbstractAbstract PDF
Wernicke’s encephalopathy (WE) is a neurological disorder induced by a dietary vitamin B1 (thiamine) deficiency which is characterized by encephalopathy, gait ataxia, and variant ocular motor dysfunction. In addition to these classical signs of WE, a loss of the horizontal vestibulo-ocular reflex (VOR) is being reported as the major underdiagnosed symptoms in WE. In this retrospective single center study, we report four cases of WE initially presented with impaired horizontal VOR in addition to the classical clinical presentations, and imaging and neurotological laboratory findings were described.

Res Vestib Sci : Research in Vestibular Science