Research in Vestibular Science

Case Report

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.

Citations

Citations to this article as recorded by

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

Original Article

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.

Citations

Citations to this article as recorded by

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

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