Advertisement

Perverted Downward Corrective Saccades During Horizontal Head Impulses in Chiari Malformation

  • Sung-Hee Kim
  • Hyo-Jung Kim
  • Ji-Soo KimEmail author
Original Paper
  • 13 Downloads

Abstract

The mechanism of perverted vertical responses during horizontal head impulse tests (HITs) requires further elucidation. A 47-year-old woman with a Chiari malformation showed alternating skew deviation, downbeat nystagmus with an increasing slow phase velocity, impaired smooth pursuit, and upward ocular deviation during horizontal HITs and corrective downward saccades in the presence of normal bithermal caloric tests and intact tilt suppressions of the post-rotatory nystagmus. These findings suggest dysfunction of the inferior cerebellum including the tonsil, nodulus, and uvula. We propose that disruption of signals from the medial part of the vestibulocerebellum, which normally inhibits the lateral and anterior canal pathways, may elicit an upward misdirection of the eye velocity during rapid horizontal head rotation. Otherwise, the Chiari malformation may have directly affected the brainstem structures involved in the direction matrix of the vestibulo-ocular reflex.

Keywords

Chiari malformation Head impulse test Downbeat nystagmus 

Notes

Author Contributions

S.-H. K., as the first author, designed the study, interpreted and analyzed the data, and drafted the manuscript. H. J. K. acquired the data and made revisions of the manuscript and figures. J.-S. K., as the corresponding author, designed the study, interpreted the data, and made critical revisions of the manuscript.

Funding

This research was supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Education, Science and Technology (NRF-2016R1D1A1B04935568).

Compliance with Ethical Standards

Disclosure

S.-H. K and H-J. K report no disclosure.

J.-S. K. 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, and Journal of Neurology and Medicine.

Conflict of Interest

The authors declare that they have no conflict of interest.

References

  1. 1.
    Halmagyi GM, Curthoys IS. A clinical sign of canal paresis. Arch Neurol. 1988;45:737–9.CrossRefGoogle Scholar
  2. 2.
    Kumar A, Patni AH, Charbel F. The Chiari I malformation and the neurotologist. Otol Neurotol. 2002;23:727–35.CrossRefGoogle Scholar
  3. 3.
    Park HK, Kim JS, Strupp M, Zee DS. Isolated floccular infarction: impaired vestibular responses to horizontal head impulse. J Neurol. 2013;260:1576–82.CrossRefGoogle Scholar
  4. 4.
    Jeong SH, Kim JS, Baek IC, Shin JW, Jo H, Lee AY, et al. Perverted head impulse test in cerebellar ataxia. Cerebellum. 2013;12:773–5.CrossRefGoogle Scholar
  5. 5.
    Kim SH, Zee DS, du Lac S, Kim HJ, Kim JS. Nucleus prepositus hypoglossi lesions produce a unique ocular motor syndrome. Neurology. 2016;87:2026–33.CrossRefGoogle Scholar
  6. 6.
    Choi JY, Kim JS, Jung JM, Kwon DY, Park MH, Kim C, et al. Reversed corrective saccades during head impulse test in acute cerebellar dysfunction. Cerebellum. 2014;13:243–7.CrossRefGoogle Scholar
  7. 7.
    Walker MF, Zee DS. Cerebellar disease alters the axis of the high-acceleration vestibuloocular reflex. J Neurophysiol. 2005;94:3417–29.CrossRefGoogle Scholar
  8. 8.
    Choi JY, Kim HJ, Kim JS. Recent advances in head impulse test findings in central vestibular disorders. Neurology. 2018;90:602–12.CrossRefGoogle Scholar
  9. 9.
    Walker MF, Zee DS. Directional abnormalities of vestibular and optokinetic responses in cerebellar disease. Ann N Y Acad Sci. 1999;871:205–20.CrossRefGoogle Scholar
  10. 10.
    Weber PC, Cass SP. Neurotologic manifestations of Chiari 1 malformation. Otolaryngol Head Neck Surg. 1993;109:853–60.CrossRefGoogle Scholar
  11. 11.
    Guerra Jiménez G, Mazón Gutiérrez Á, Marco de Lucas E, Valle San Román N, Martín Laez R, Morales Angulo C. Audio-vestibular signs and symptoms in Chiari malformation type I. case series and literature review. Acta Otorrinolaringol Esp. 2015;66:28–35.CrossRefGoogle Scholar
  12. 12.
    Salman MS, Sharpe JA, Lillakas L, Dennis M, Steinbach MJ. The vestibulo-ocular reflex during active head motion in Chiari II malformation. Can J Neurol Sci. 2008;35:495–500.CrossRefGoogle Scholar
  13. 13.
    Choi KD, Oh SY, Park SH, Kim JH, Koo JW, Kim JS. Head-shaking nystagmus in lateral medullary infarction: patterns and possible mechanisms. Neurology. 2007;68:1337–44.CrossRefGoogle Scholar
  14. 14.
    Choi KD, Oh SY, Kim HJ, Kim JS. The vestibulo-ocular reflexes during head impulse in Wernicke’s encephalopathy. J Neurol Neurosurg Psychiatry. 2007;78:1161–2.CrossRefGoogle Scholar
  15. 15.
    Choi KD, Kim JS. Head-shaking nystagmus in central vestibulopathies. Ann N Y Acad Sci. 2009;1164:338–43.CrossRefGoogle Scholar
  16. 16.
    Kim HJ, Lee SH, Park JH, Choi JY, Kim JS. Isolated vestibular nuclear infarction: report of two cases and review of the literature. J Neurol. 2014;261:121–9.CrossRefGoogle Scholar
  17. 17.
    Choi SY, Lee SH, Kim HJ, Kim JS. Impaired modulation of the otolithic function in acute unilateral cerebellar infarction. Cerebellum. 2014;13:362–71.CrossRefGoogle Scholar
  18. 18.
    Wearne S, Raphan T, Cohen B. Control of spatial orientation of the angular vestibuloocular reflex by the nodulus and uvula. J Neurophysiol. 1998;79:2690–715.CrossRefGoogle Scholar
  19. 19.
    Goldschagg N, Feil K, Ihl F, Krafczyk S, Kunz M, Tonn JC, et al. Decompression in Chiari malformation: clinical, ocular motor, cerebellar, and vestibular outcome. Front Neurol. 2017;8:292.  https://doi.org/10.3389/fneur.2017.00292.CrossRefPubMedPubMedCentralGoogle Scholar
  20. 20.
    Lee SH, Park SH, Kim JS, Kim HJ, Yunusov F, Zee DS. Isolated unilateral infarction of the cerebellar tonsil: ocular motor findings. Ann Neurol. 2014;75:429–34.CrossRefGoogle Scholar
  21. 21.
    Zee DS, Leigh RJ, Mathieu-Millaire F. Cerebellar control of ocular gaze stability. Ann Neurol. 1980;7:37–40.CrossRefGoogle Scholar
  22. 22.
    Radtke A, Bronstein AM, Gresty MA, Faldon M, Taylor W, Stevens JM, et al. Paroxysmal alternating skew deviation and nystagmus after partial destruction of the uvula. J Neurol Neurosurg Psychiatry. 2001;70:790–3.CrossRefGoogle Scholar
  23. 23.
    Barton JJ, Sharpe JA. Oscillopsia and horizontal nystagmus with accelerating slow phases following lumbar puncture in the Arnold-Chiari malformation. Ann Neurol. 1993;33:418–21.CrossRefGoogle Scholar
  24. 24.
    Choi JY, Kim JH, Kim HJ, Glasauer S, Kim JS. Central paroxysmal positional nystagmus: characteristics and possible mechanisms. Neurology. 2015;84:2238–46.CrossRefGoogle Scholar
  25. 25.
    Mossman S, Halmagyi GM. Partial ocular tilt reaction due to unilateral cerebellar lesion. Neurology. 1997;49:491–3.CrossRefGoogle Scholar
  26. 26.
    Tarnutzer AA, Shaikh AG, Palla A, Straumann D, Marti S. Vestibulo-cerebellar disease impairs the central representation of self-orientation. Front Neurol. 2011;2:11.  https://doi.org/10.3389/fneur.2011.00011.CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Waespe W, Cohen B, Raphan T. Dynamic modification of the vestibulo-ocular reflex by the nodulus and uvula. Science. 1985;228:199–202.CrossRefGoogle Scholar
  28. 28.
    Hain TC, Zee DS, Maria BL. Tilt suppression of vestibulo-ocular reflex in patients with cerebellar lesions. Acta Otolaryngol. 1988;105:13–20.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Neurology, School of MedicineKyungpook National University, Kyungpook National University Chilgok HospitalDaeguSouth Korea
  2. 2.Research Administration TeamSeoul National University Bundang HospitalSeongnamSouth Korea
  3. 3.Department of NeurologySeoul National University College of MedicineSeoulSouth Korea
  4. 4.Dizziness Center, Clinical Neuroscience Center, and Department of NeurologySeoul National University Bundang HospitalSeongnam-siSouth Korea

Personalised recommendations