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Isolated vestibular nuclear infarction: report of two cases and review of the literature

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Abstract

Cerebral infarction presenting with isolated vertigo remains a diagnostic challenge. To define the clinical characteristics of unilateral infarctions restricted to the vestibular nuclei, two patients with isolated unilateral vestibular nuclear infarction had bedside and laboratory evaluation of the ocular motor and vestibular function, including video-oculography, bithermal caloric irrigation, the head impulse test (HIT) using magnetic scleral coils, and cervical and ocular vestibular-evoked myogenic potentials (VEMPs). We also reviewed the literature on isolated vertigo from lesions restricted to the vestibular nuclei, and analyzed the clinical features of seven additional patients. Both patients showed spontaneous torsional-horizontal nystagmus that beat away from the lesion side, and direction-changing gaze-evoked nystagmus. Recording of HIT using a magnetic search coil system documented decreased gains of the vestibular-ocular reflex for the horizontal and posterior semicircular canals on both sides, but more for the ipsilesional canals. Bithermal caloric tests showed ipsilesional canal paresis in both patients. Cervical and ocular VEMPs showed decreased or absent responses during stimulation of the ipsilesional ear. Initial MRIs including diffusion-weighted images were normal or equivocal, but follow-up imaging disclosed a circumscribed acute infarction in the area of the vestibular nuclei. Infarctions restricted to the vestibular nuclei may present with isolated vertigo with features of both peripheral and central vestibulopathies. Central signs should be sought even in patients with spontaneous horizontal–torsional nystagmus and positive HIT. In patients with combined peripheral and central vestibulopathy, a vestibular nuclear lesion should be considered especially when hearing is preserved.

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Acknowledgments

This study was supported by a grant of Korea Medical Devices Industrial Cooperative Association and Small & Medium Business Administration (08-2011-065).

Conflicts of interest

The authors declare that they have no conflict of interest. Dr. Kim serves as an Associate Editor of Frontiers in Neuro-otology and on the editorial boards of the Journal of Korean Society of Clinical Neurophysiology, Research in Vestibular Science, Journal of Clinical Neurology, Frontiers in Neuro-ophthalmology, Journal of Neuro-ophthalmology, and Case Reports in Ophthalmological Medicine; and received research support from SK Chemicals, Co. Ltd. HJ Kim, SH Lee, JH Park, JY Choi report no disclosure.

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Authors and Affiliations

  1. Biomedical Research Institute, Seoul National University Bundang Hospital, Seongnam, Korea

    Hyo-Jung Kim

  2. Department of Neurology, Seoul National University College Medicine, Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, Seongnam, Gyeonggi, 463-707, Korea

    Hyo-Jung Kim, Jae Han Park & Ji-Soo Kim

  3. Department of Neurology, Chonnam National University Medical School, Gwangju, Korea

    Seung-Han Lee

  4. Department of Neurology, Korea University College of Medicine, Korea University Ansan Hospital, Ansan, Korea

    Jung-Yoon Choi

Authors
  1. Hyo-Jung Kim
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  2. Seung-Han Lee
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Corresponding author

Correspondence to Ji-Soo Kim.

Additional information

H.-J. Kim and S.-H. Lee equally contributed to this study.

Electronic supplementary material

Below is the link to the electronic supplementary material.

415_2013_7139_MOESM1_ESM.wmv

Supplementary material 1 Video 1: Spontaneous and gaze-evoked nystagmus in patient 1. Spontaneous left-beating horizontal-torsional nystagmus increases with removal of visual fixation and during left eccentric gaze, and changes into right beating during rightward gaze. (WMV 18130 kb)

415_2013_7139_MOESM2_ESM.wmv

Supplementary material 2 Video 2: Bedside head impulse tests in patient 1. The patient shows a corrective catch-up saccade during head impulse to the right. (WMV 9943 kb)

415_2013_7139_MOESM3_ESM.wmv

Supplementary material 3 Video 3: Spontaneous and gaze-evoked nystagmus in patient 2. Spontaneous left beating horizontal-torsional nystagmus increases without fixation and during left eccentric gaze, and changes into right beating during rightward gaze. (WMV 14333 kb)

415_2013_7139_MOESM4_ESM.wmv

Supplementary material 4 Video 4: Bedside head impulse tests in patient 2. The patient shows a corrective catch-up saccade during head impulses for the horizontal and posterior canals on both sides, more marked for the ipsilesional ones.(WMV 15013 kb)

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Kim, HJ., Lee, SH., Park, J.H. et al. Isolated vestibular nuclear infarction: report of two cases and review of the literature. J Neurol 261, 121–129 (2014). https://doi.org/10.1007/s00415-013-7139-0

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