Research in Vestibular Science

Posterior Inferior Cerebellar Artery Stenosis Presenting as Recurrent Orthostatic Dizziness: Seongjin Jeon, Ji-Yun Park, Jong-min Lee, Seulgi Hong; Res Vestib Sci. 2022;21(2):53-56. Published online June 15, 2022; DOI: https://doi.org/10.21790/rvs.2022.21.2.53

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Abstract PDF: We report a unique case of vertebrobasilar transient ischemic attacks manifesting as isolated, recurrent, orthostatic dizziness with posterior inferior cerebellar artery (PICA) stenosis. A 57-year-old male patient without past medical history, presented with brief orthostatic dizziness for 2 weeks. There was no associated nausea, vomiting, diplopia, or weakness. On neuro-otologic examination, the patient did not show spontaneous, positional, or gaze-evoked nystagmus. Vestibular function tests such as caloric test, head impulse test, video-oculography, and tilt table test were normal. Brain diffusion-weighted images showed multiple small high signal intensities in the bilateral cerebellar hemispheres. Brain magnetic resonance angiography revealed hypoplasia of the right vertebral artery without focal intracranial focal stenosis. Four-vessel cerebral angiogram showed severe stenosis at the right PICA artery. Our patient’s clinical scenario appears unique hemodynamic spells without symptoms or signs of posterior circulation ischemia. Physicians should also consider cerebrovascular ischemic when the patient suffers repeated orthostatic dizziness that is not explained clinically.

Paraneoplastic Cerebellar Degeneration Presented as Acute Vertigo: Sung-Hee Kim; Res Vestib Sci. 2022;21(1):24-27. Published online March 15, 2022; DOI: https://doi.org/10.21790/rvs.2022.21.1.24

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Abstract PDF: Paraneoplastic cerebellar degeneration is a rare neurological manifestation of nonmetastatic malignancy. Its usual manifestation is subacute to chronic dizziness, gait ataxia, and dysarthria. There have been only a few cases of paraneoplastic cerebellar degeneration with acute presentation. This study describes a patient with paraneoplastic cerebellar degeneration, who presented acute vestibular syndrome and then episodically developed horizontal gaze-evoked nystagmus and gait ataxia.

Understanding Vestibular Information Processing in Velocity-Storage Circuit and Application to Interpreting Clinical Manifestation of Vestibular Disorders: Jeong-Yoon Choi; Res Vestib Sci. 2021;20(3):81-92. Published online September 15, 2021; DOI: https://doi.org/10.21790/rvs.2021.20.3.81

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Abstract PDF: The velocity-storage circuit comprised of bilateral vestibular nucleus complexes, commissural fiber, and nodulus and uvula functions in refining the raw vestibular signal to estimate rotational velocity, gravity direction, and inertia. In this review, we pursued the functional significance of this velocity-storage circuit and how this physiologic knowledge could help us understand the clinical symptoms and signs of patients with vestibular disorders.

Neurotological Findings in a Patient with Glufosinate Ammonium Intoxication: Ji Young Kim, Joo Yeon Ham, Seong-Hae Jeong; Res Vestib Sci. 2020;19(4):138-140. Published online December 15, 2020; DOI: https://doi.org/10.21790/rvs.2020.19.4.138

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Abstract PDF Supplementary Material: The upward deviation could be explained by loss of inhibitory inputs from the cerebellum onto the brainstem anterior semicircular canal projections for upward vestibulo-ocular reflex, which would lead to an upward bias in static eye position. Therefore, upward gaze deviation has been reported in comatose patients after resuscitation and diffuse cerebrocerebellar damage sparing the brainstem. Herein, we report a patient with ingestion of glufosinate ammonium presented with cerebellar ataxia and ocular motor findings suggestive of cerebellum involvement such as upward gaze tendency, spontaneous downbeat, gaze-evoked nystagmus, perverted head impulse test, and impaired smooth pursuit.

Positional Hemiseesaw Nystagmus in Ataxia with Oculomotor Apraxia Type 2 due to a Novel Senataxin Gene Mutation: A New Phenotype: Sung-Hee Kim, Ja-Hyun Jang, Ji-Soo Kim; Res Vestib Sci. 2020;19(1):12-15. Published online March 15, 2020; DOI: https://doi.org/10.21790/rvs.2020.19.1.12

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Abstract PDF Supplementary Material

Ataxia with ocular motor apraxia type 2 (AOA2) is an autosomal recessive disorder that is characterized by adolescent-onset gait ataxia, peripheral neuropathy, ocular apraxia, and cerebellar atrophy. A 19-year-old male with AOA2 from a novel SETX mutation showed distinct oculomotor abnormalities that included spontaneous and gaze-induced downbeat nystagmus, impaired smooth pursuit, and reversed catch-up saccades during horizontal head impulse tests, as well as peripheral neuropathy involving the lower extremities and mild slowing of frontal processing. He also showed positional hemiseesaw nystagmus in the supine and straight head-hanging positions. Positional hemiseesaw nystagmus is a new manifestation of hereditary cerebellar ataxia and may be explained by a gravity-dependent position-induced error in estimating the tilt in the roll plane due to dysfunction of the tilt-estimator circuit.

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A Case of AOA2 With Compound Heterozygous SETX Mutations
Hee Jin Chang, Ryul Kim, Minchae Kim, Jangsup Moon, Man Jin Kim, Han-Joon Kim
Journal of Movement Disorders.2022; 15(2): 178. CrossRef

Post Infectious Cerebellar Ataxia with Abnormal Brain Magnetic Resonance Imaging and Single Photon Emission Computed Tomography Findings: Hae Joo Rha, Jae Han Park; Res Vestib Sci. 2017;16(4):167-170. Published online December 15, 2017; DOI: https://doi.org/10.21790/rvs.2017.16.4.167

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Abstract PDF: A 84-year-old woman presented with a two weeks history of dizziness, slurred speech and ataxia. The neurological exam showed spontaneous left beating nystagmus, horizontal gaze evoked nystagmus and limb ataxia. A few weeks earlier, she had an upper airway infection. Brain MRI revealed diffuse leptomeningeal enhancement in the both cerebellar hemisphere and brain single photon emission computed tomography (SPECT) showed hyperperfusion in both cerebellar hemisphere. Extensive laboratory studies with cerebrospinal fluid analysis did not reveal any etiologic factors. She was started on methylprednisolone (1 g/day for 7 days), gradually improved over the weeks. Post infectious cerebellar ataxia is a neurologic complication that occasionally follows systemic viral and bacterial infections. This case demonstrates that cerebellar abnormalities can be detected by brain magnetic resonance imaging and SPECT.

Rapidly Progressive Ophthalmoplegia and Brainstem-Cerebellar Dysfunction in Rhombencephalitis Caused by Listeria monocytogenes: Sung-Sik Kim, Jong-Hun Kim, Jin-Heui Lee, Seung-Han Lee; Res Vestib Sci. 2017;16(1):29-33. Published online March 15, 2017; DOI: https://doi.org/10.21790/rvs.2017.16.1.29

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Abstract PDF: Listeria monocytogenes (L. monocytogenes) is a rare, but important bacterial pathogen causing central nervous system infection in the elderly, pregnant women, and immunocompromised patients. A 60-year-old man with diabetes presented with headache, fever and rapidly progressive ophthalmoplegia. Based on serological and MRI studies, he was diagnosed with rhombencephalitis due to L. monocytogenes. The patient recovered without complications with urgent initiation of empirical antibiotics and the pathogen-specific antibiotic treatment. L. monocytogenes should be considered as a cause of rhombencephalitis presenting as external ophthalmoplegia.

Isolated Infarction of Anterior Cerebellar Vermis: Sung-Hee Kim, Ji-Soo Kim; Res Vestib Sci. 2016;15(4):147-150. Published online December 12, 2016; DOI: https://doi.org/10.21790/rvs.2016.15.4.147

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The anterior cerebellar vermis has been known to act in coordination of gait and postural adjustment of the trunk and legs. However, oculomotor abnormalities in an isolated anterior vermian lesion have not been described in the literature. A 59-year-old man presented with acute non-rotatory dizziness and disequilibrium. Neuro-ophthalmologic examination found impaired smooth pursuit and hypometric saccades in the contralesional direction, and disconjugate ipsiversive ocular torsion, but without spontaneous or gaze-evoked nystagmus. Imaging study showed an infarction restricted to the rostral end of right cerebellar vermis involving the lingual and central lobules. The anterior cerebellar vermis participates in the maintenance of axial posture and gait, and also in the control of ocular motor and vestibular systems.

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Free water imaging unravels unique patterns of longitudinal structural brain changes in Parkinson’s disease subtypes
Abigail E. Bower, Sophia J. Crisomia, Jae Woo Chung, Justin P. Martello, Roxana G. Burciu
Frontiers in Neurology.2023;[Epub] CrossRef

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