Research in Vestibular Science

Arnold-Chiari Malformation Presented with Spontaneous Down-Beating Nystagmus and Gait Disturbance: Minbum Kim, Youn Jin Cho; Res Vestib Sci. 2023;22(4):132-136. Published online December 15, 2023; DOI: https://doi.org/10.21790/rvs.2023.22.4.132

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Abstract PDF Supplementary Material: Arnold Chiari malformation is a disease which is characterized by herniation of a portion of the cerebellum through the foramen magnum. Symptoms vary depending on the extent of the affected area, including posterior neck pain, upper limb pain, paralysis, paresthesia, weakness, dizziness, and ataxia. Among the patients presenting with dizziness, nystagmus is frequently observed, which is primarily characterized by down-beating nystagmus. We experienced a 42- years-old female patient presented with vertigo and gait disturbance, who were diagnosed with type 1 Arnold-Chiari malformation and treated by surgical decompression.

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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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.

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

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Abstract 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.

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