Research in Vestibular Science

Fourth Nerve Paresis plus Crossed Horner Syndrome in Acute Leukemia: Sumin Kim, Sung-Hee Kim; Res Vestib Sci. 2022;21(3):86-88. Published online September 15, 2022; DOI: https://doi.org/10.21790/rvs.2022.21.3.86

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Abstract PDF: A 29-year-old patient with diplopia showed a unilateral fourth nerve palsy combined with a Horner syndrome on the opposite side. Magnetic resonance images disclosed a focal hemorrhage in the left dorsal midbrain, affecting the nucleus of the fourth nerve and adjacent oculosympathetic fibers. Further evaluations including a bone marrow biopsy confirmed acute lymphoblastic leukemia. The combination of ocular motor and oculosympathetic palsy has a distinct localizing value. Fourth nerve palsy ipsilateral to Horner syndrome locates lesion in the cavernous sinus, while contralateral fourth nerve palsy locates the lesion in the midbrain.

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.

Differential Diagnosis of the Acute Vestibular Syndrome: Sung-Hee Kim; Res Vestib Sci. 2021;20(1):7-16. Published online March 11, 2021; DOI: https://doi.org/10.21790/rvs.2021.20.1.7

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Abstract PDF: Acute vestibular syndrome refers to the rapid onset of vertigo, nausea, vomiting, and unsteady gait. Acute unilateral vestibular neuritis is the most common cause of acute vestibular syndrome. However, vascular lesions involving the brainstem and cerebellum also produce acute vestibular syndrome even without other neurologic deficits. The vestibular nucleus or nucleus prepositus hypoglossi in the dorsal portion of the brainstem, cerebellar structures including flocculus, tonsil, and nodulus, and cerebellar peduncle can produce isolated vertigo and imbalance when damaged. Early recognition of the pseudo-vestibular neuritis of the vascular etiology is warranted for clinicians.

Persistent Postural-Perceptual Dizziness: Overview and Diagnostic Criteria: Sung-Hee Kim; Res Vestib Sci. 2020;19(2):42-48. Published online June 15, 2020; DOI: https://doi.org/10.21790/rvs.2020.19.2.42

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Abstract PDF: Persistent postural perceptual dizziness (PPPD) is a chronic functional vestibular disorder that manifests with 3 or more months of dizziness, nonspinning vertigo, and unsteadiness. These main symptoms are exacerbated by upright posture, active or passive self-motion, and exposure to visual stimuli. PPPD is usually precipitated by illnesses that cause vertigo, dizziness, or unsteadiness. The common precipitants are acute or episodic peripheral vestibular diseases including vestibular neuritis, Meniere disease, or benign paroxysmal positional vertigo. PPPD is not a diagnosis of exclusion. An abnormal finding on examination or laboratory testing does not necessarily exclude a diagnosis of PPPD. This article reviewed the Bárány Society’s diagnostic criteria for PPPD in detail and discussed directions of future investigations.

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

Positional Hemiseesaw Nystagmus in Ataxia with Oculomotor Apraxia Type 2 due to a Novel Senataxin Gene Mutation: A New Phenotype: Sung-Hee Kim, Ji-Soo Kim; Received July 24, 2018 Accepted September 14, 2018 Published online September 14, 2018; [Accepted]

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Abstract: 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 hemi-seesaw 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.

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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Abstract PDF

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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