Alternating monocular adducting saccadic pulses and dissociated adducting nystagmus during lateral gazes in spinocerebellar ataxia type 3

  • Sun-Uk Lee
  • Hyo-Jung Kim
  • Jeong-Yoon Choi
  • Ji-Soo KimEmail author
Letter to the Editors

Dear Sirs,

We report alternating monocular adducting saccades in the primary gaze and dissociated adducting nystagmus during lateral gazes in a patient with spinocerebellar ataxia type 3 (SCA3).

A 65-year-old man presented progressive unsteadiness and binocular diplopia since his 40s. His father, two brothers and a sister had similar symptoms since their 40s, and had a previous diagnosis of SCA3 at other hospitals.

Examination showed exotropia of the eyes (video 1). Alternating monocular adducting eye movements interrupted the steady fixation in both eyes. Theses spontaneous eye movements increased during attempted convergence for a near target, and decreased without visual fixation in darkness (Fig.  1a). He also showed dissociated adducting nystagmus during lateral gazes (video 2). Convergence was impaired and bedside head-impulse tests (HITs) were positive in both horizontal directions. Ocular ductions and versions were otherwise full in both eyes.


Author contributions

Dr. Lee analyzed and interpreted the data and wrote the manuscript. Drs. H.J. Kim, and Choi analyzed and interpreted the data, and revised the manuscript. Drs. J.S. Kim designed and conceptualized the study, interpreted the data, and revised the manuscript.


This study was supported by Basic Science Research Program through the National Research Foundation of Korea funded by the Ministry of Education, Science and Technology (No. NRF-2016R1D1A1B04935568).

Compliance with ethical standards

Conflicts of interest

Drs. S.U. Lee, H.J. Kim, and J.Y. Choi report no disclosures. Dr. J.S. Kim serves as an associate editor of Frontiers in Neuro-otology and on the editorial boards of the Journal of Clinical Neurology, Frontiers in Neuro-ophthalmology, Journal of Neuro-ophthalmology, Journal of Vestibular Research, Journal of Neurology, and Medicine.

Ethical approval

This study followed the tenets of the Declaration of Helsinki and was performed according to the guidelines of Institutional Review Board of Seoul National University Bundang Hospital (B-1906-549-701).

Supplementary material

415_2019_9609_MOESM1_ESM.docx (34 kb)
Supplementary material 1 (DOCX 34 kb)

Video 1 Exotropia is evident during alternating cover test (MP4 21470 kb)

Video 2 The patient shows alternating monocular adducting saccades in both eyes followed by glissadic eye movements (MP4 1768 kb)

Video 3 The patient shows a pulse-step mismatch, especially in the adducting eye, and dissociated adducting nystagmus during horizontal saccades. Vertical saccades are slow in both directions and show a pulse-step mismatch upward (MP4 42619 kb)


  1. 1.
    Kawaguchi Y, Okamoto T, Taniwaki M, Aizawa M, Inoue M, Katayama S, Kawakami H, Nakamura S, Nishimura M, Akiguchi I (1994) CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32. 1. Nat Genet 8(3):221CrossRefGoogle Scholar
  2. 2.
    Leigh RJ, Zee DS (2015) The neurology of eye movements. Oxford University Press, New YorkCrossRefGoogle Scholar
  3. 3.
    Otero-Millan J, Serra A, Leigh RJ, Troncoso XG, Macknik SL, Martinez-Conde S (2011) Distinctive features of saccadic intrusions and microsaccades in progressive supranuclear palsy. J Neurosci 31(12):4379–4387CrossRefGoogle Scholar
  4. 4.
    Oh EH, Choi S-Y, Choi K-D, Choi J-H (2019) Monocular single saccadic pulses. J Neuroopthalmol 39(2):237–239CrossRefGoogle Scholar
  5. 5.
    Herishanu YO, Sharpe JA (1983) Saccadic intrusions in internuclear ophthalmoplegia. Ann Neurol 14(1):67–72CrossRefGoogle Scholar
  6. 6.
    Lee ES, Choi JY, Kim JS (2017) Teaching video neuroimages: alternating horizontal single saccadic pulses in progressive supranuclear palsy. Neurology 88(3):e32–e33CrossRefGoogle Scholar
  7. 7.
    Lemos J, Novo A, Duque C, Castelhano J, Eggenberger E, Januário C (2018) “Pinball” intrusions in spinocerebellar ataxia type 3. Neurology 90(1):36–37CrossRefGoogle Scholar
  8. 8.
    Bahill AT, Troost BT (1979) Types of saccadic eye movements. Neurology 29(8):1150–1152CrossRefGoogle Scholar
  9. 9.
    Clendaniel RA, Mays LE (1994) Characteristics of antidromically identified oculomotor internuclear neurons during vergence and versional eye movements. J Neurophysiol 71(3):1111–1127CrossRefGoogle Scholar
  10. 10.
    Christova P, Anderson JH, Gomez CM (2008) Impaired eye movements in presymptomatic spinocerebellar ataxia type 6. Arch Neurol 65(4):530–536CrossRefGoogle Scholar
  11. 11.
    Jacobi H, Reetz K, du Montcel ST, Bauer P, Mariotti C, Nanetti L, Rakowicz M, Sulek A, Durr A, Charles P (2013) Biological and clinical characteristics of individuals at risk for spinocerebellar ataxia types 1, 2, 3, and 6 in the longitudinal RISCA study: analysis of baseline data. Lancet Neurol 12(7):650–658CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of NeurologyKorea University Medical CenterSeoulSouth Korea
  2. 2.Department of NeurologySeoul National University College of MedicineSeoulSouth Korea
  3. 3.Research Administration TeamSeoul National University Bundang HospitalSeongnamSouth Korea
  4. 4.Department of Neurology, Dizziness Center, Clinical Neuroscience CenterSeoul National University Bundang HospitalSeongnamSouth Korea

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