Research in Vestibular Science

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

A Case of Patient with Acute Peripheral Vestibulopathy Involving Isolated Posterior Semicircular Canal: Sang Kwon Im, Mee Hyun Song, Ja Won Gu, Dae Bo Shim; Res Vestib Sci. 2019;18(4):128-132. Published online December 15, 2019; DOI: https://doi.org/10.21790/rvs.2019.18.4.128

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Abstract PDF Supplementary Material: Down-beat nystagmus in peripheral vestibulopathy may rarely occur in selective involvement of inferior division. Diagnosis of isolated inferior vestibular neuritis is based on torsional down-beating spontaneous nystagmus, abnormal head-impulse test for the posterior semicircular canal, and abnormal cervical vestibular evoked myogenic potentials (cVEMPs) with normal bithermal caloric tests and ocular vestibular evoked myogenic potentials (oVEMPs). Herein we report a 50-year-old male patient with spontaneous down-beat nystagmus who demonstrated vestibular dysfunction involving only the posterior semicircular canal. The patient showed normal results in the bithermal caloric test, the oVEMPs and cVEMPs test. Video head impulse test showed decreased gain and corrective saccades only in the posterior semicircular canal. This case is significant in showing that peripheral dizziness with spontaneous down-beating nystagmus can occur as a result of an abnormality involving only a single semicircular canal.

Various Nystagmus Patterns and Their Clinical Significance in Benign Paroxysmal Positional Vertigo of Anterior Semicircular Canal: Jin Woo Park, Yong Gook Shin, Ja Won Gu, Mee Hyun Song, Dae Bo Shim; Res Vestib Sci. 2016;15(4):126-131. Published online December 12, 2016; DOI: https://doi.org/10.21790/rvs.2016.15.4.126

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Abstract PDF

Objective: The purpose of this study was to identify the diverse patterns of nystagmus and analyze their clinical significance in benign paroxysmal positional vertigo (BPPV) of the anterior semicircular canal.
Methods
Fifty-three patients diagnosed with anterior canal BPPV (AC-BPPV) were analyzed retrospectively. Patients were classified according to the presence or absence of the torsional component of the nystagmus and the direction of Dix-Hallpike test which induced the nystagmus. We compared the clinical characteristics and treatment outcomes among the different patient groups.
Results
There were 11 patients with unilateral down beat (DB) nystagmus, 11 patients with bilateral DB nystagmus, 14 patients with ipsilateral torsional down beat (TDB) nystagmus, 7 patients with contralateral TDB nystagmus, and 7 patients with bilateral TDB nystagmus. There were no differences between the unilateral and the bilateral DB groups in terms of the duration of nystagmus or vertigo and the number of treatment sessions. In addition, the ipsilateral TDB group showed no significant clinical difference compared to the contralateral or bilateral TDB group.
Conclusion
Various nystagmus patterns can be seen in AC-BPPV. There was no statistically significant difference in the clinical characteristics according to the different nystagmus patterns. This information may be helpful for clinicians in counseling and managing the patients with AC-BPPV.

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Benign Paroxysmal Positional Vertigo: Diagnostic Criteria and Updated Practice Guideline in Diagnosis
Dae Bo Shim
Research in Vestibular Science.2020; 19(4): 111. CrossRef

Downbeat, Gaze-evoked and Perverted Head-shaking Nystagmus Associated with Dapsone Toxicity: Tae Woo Kim, Sun Young Oh, Ha Cheol Choi, Byoung Soo Shin, Man Wook Seo, Young Hyun Kim; Res Vestib Sci. 2009;8(2):161-163.

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Abstract PDF: Dapsone is an agent commonly used to treat leprosy and its most common adverse effect is a hemolytic anemia. We present a patient who showed transient downbeat, bilateral horizontal gaze-evoked nystagmus (GEN), and perverted head-shaking nystagmus after taking a large dose of dapsone. These oculomotor abnormalities are assumed to be a reversible vestibulocerebellar dysfunction caused by over-dose of dapsone.

Lithium-Induced Downbeat Nystagmus with Reversible Splenial Lesion: Dong Uk Kim, Seung Han Lee, Hyun Jung Jung, Kyung Wook Kang; J Korean Bal Soc. 2007;6(2):150-154.

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Abstract PDF: Lithium is recognized as a cause of reversible or permanent downbeat nystagmus. Many patients who were treated with lithium for psychiatric illness developed downbeat nystagmus and other neurological manifestations. Reversible splenial lesions of corpus callosum are commonly seen on diffusion-weighted imaging (DWI) in various neurological disorders including metabolic/toxic encephalopathy, encephalitis, patients with epilepsy receiving antiepileptic drugs. Herein, we report a patient with reversible downbeat nystagmus, disturbance of smooth pursuit, and gait ataxia who was treated with lithium. Also, we can observe reversible splenial lesion of corpus callosum on DWI in this patient.

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