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The incidence and characteristics of acute vestibular symptoms, responsible structures, and lateralization of the causative lesions in supratentorial stroke remain unknown. This study aimed to determine the incidence, clinical features, and anatomical correlation of acute vestibular symptoms in supratentorial stroke. We performed a prospective, multicenter, observational study that had recruited patients with supratentorial stroke from the neurology clinics of referral-based four university hospitals in Korea. All patients received a constructed neuro-otological evaluations, and neuroimaging. We analyzed the incidence of acute vestibular symptoms, abnormal ocular motor and vestibular function tests, and stroke lesions. Of 1301 patients with supratentorial stroke, 48 (3.7%) presented with acute vestibular symptoms, and 13 of them (1%) had the vestibular symptoms in isolation. In patients with acute vestibular symptoms, abnormal findings included spontaneous nystagmus (5%), impaired horizontal smooth pursuit (41%), and abnormal tilt of the subjective visual vertical (SVV) (20%). Video head impulse and caloric tests were normal in all the patients. There was no clear correlation between acute vestibular symptoms and involvement of specific vestibular cortex. In patients with unilateral stroke, there was also no lateralization of the causative lesions of acute vestibular symptoms (left vs. right; 52 vs. 48%), even in patients with vertigo (left vs. right; 58 vs. 42%). This study demonstrates that the incidence of acute vestibular symptoms in supratentorial stroke is 3.7%, with being isolated in 1%. The widespread lesions responsible for acute vestibular symptoms implicate diffuse multisensory cortical–subcortical networks in the cerebral hemispheres without a lateralization. [ABSTRACT FROM AUTHOR]

Differentiation of spinocerebellar ataxia type 17 (SCA17) from Huntington's disease (HD) is often challenging since they share the clinical features of chorea, parkinsonism, and dystonia. The ocular motor findings remain to be elucidated in SCA17, and may help differentiating SCA17 from HD. We retrospectively compared the ocular motor findings of 11 patients with SCA17 with those of 10 patients with HD. In SCA17, abnormal ocular motor findings included impaired smooth pursuit (9/11, 82%), dysmetric saccades (9/11, 82%), central positional nystagmus (CPN, 7/11, 64%), abnormal head-impulse tests (4/11, 36%), and horizontal gaze-evoked nystagmus (GEN, 3/11, 27%). Among these, CPN was more frequently observed in SCA17 than in HD (7/11 (64%) vs. 0/10 (0%), p = 0.004) while saccadic slowing was more frequently observed in HD than in SCA17 (8/10 (80%) vs. 2/11 (18%), p = 0.009). Of six patients with follow-up evaluation, five later developed bilateral saccadic hypermetria (n = 4), GEN (n = 1), CPN (n = 1), bilaterally abnormal smooth pursuit (n = 1), and hyperactive head-impulse responses (n = 1) along with a clinical decline. Ocular motor abnormalities can be utilized as a diagnostic marker for differentiation of SCA17 from HD as well as a surrogate marker for clinical decline in SCA17. [ABSTRACT FROM AUTHOR]

In central as well as peripheral vestibular lesions, right-beating horizontal nystagmus is almost always associated with clockwise (top poles of the eyes beating to the right ear) torsional nystagmus when observed and vice versa (concordant nystagmus). This study aimed to determine the etiologies and mechanisms of horizontal and torsional nystagmus beating in the opposite directions (discordant nystagmus). We reviewed the medical records of 16 consecutive patients with discordant horizontal–torsional nystagmus who had been evaluated at the dizziness clinics of Seoul National University Bundang Hospital (n = 11, from March 2003 to March 2021) and Korea University Medical Center (n = 5, from March 2019 to March 2021). The underlying etiologies included inferior vestibular neuritis (n = 7), Meniere's disease (n = 4), internuclear ophthalmoplegia (n = 3), medullary hemorrhage (n = 1), and normal pressure hydrocephalus (n = 1). The torsional nystagmus decreased during the gaze in the same direction (for instance, during rightward gaze in clockwise nystagmus) and increased during the gaze in the opposite direction. Head-impulse tests (HITs) were positive for the ipsilesional posterior canal (PC) in all 11 patients with unilateral peripheral vestibulopathy and two of the three patients with unilateral central vestibulopathy. Discordant horizontal–torsional nystagmus may be observed in peripheral as well as central lesions. Given the findings of HITs and modulation of spontaneous nystagmus during lateral gazes, discordant horizontal–torsional nystagmus may be ascribed to selective damage of the excitatory or inhibitory pathway from the PC that innervates the ipsilateral superior oblique and contralateral inferior rectus muscles. [ABSTRACT FROM AUTHOR]

Fixation nystagmus refers to the nystagmus that appears or markedly increases with fixation. While relatively common in infantile (congenital) nystagmus, acquired fixation nystagmus is unusual and has been ascribed to lesions involving the cerebellar nuclei or the fibers projecting from the cerebellum to the brainstem. We aimed to report the clinical features of patients with acquired fixation nystagmus and discuss possible mechanisms using a model simulation and diagnostic significance. We describe four patients with acquired fixation nystagmus that appears or markedly increases with visual fixation. All patients had lesions involving the cerebellum or dorsal medulla. All patients showed direction-changing gaze-evoked nystagmus, impaired smooth pursuit, and decreased vestibular responses on head-impulse tests. The clinical implication of fixation nystagmus is that it may occur in central lesions that impair both smooth pursuit and the vestibulo-ocular reflex (VOR) but without creating a spontaneous nystagmus in the dark. We develop a mathematical model that hypothesizes that fixation nystagmus reflects a central tone imbalance due to abnormal function in cerebellar circuits that normally optimize the interaction between visual following (pursuit) and VOR during attempted fixation. Patients with fixation nystagmus have central lesions involving the cerebellar circuits that are involved in visual–vestibular interactions and normally eliminate biases that cause a spontaneous nystagmus. [ABSTRACT FROM AUTHOR]

Caloric tests are useful for disease surveillance of Meniere's disease (MD), although the interpretation of caloric tests remains controversial during the attacks of MD. Therefore, we aimed to characterize the findings of caloric tests during the attacks of MD. We analyzed the results of bithermal caloric tests during and between the attacks in 43 patients with unilateral definite MD. Evaluation was performed during the irritative/recovery phase in 22 and during the paretic phase in 26 patients. During the irritative/recovery phase, less than half of patients (10/22, 45%) showed a caloric asymmetry of more than 25%, lesser responses in the affected ear in six and in the healthy ear in the other four. In contrast, patients usually showed a caloric asymmetry of more than 25% during the paretic phase (19/26, 73%) with a decreased response almost always in the affected ear (18/19, 95%). Between the attacks, caloric asymmetry was found in less than half of the patients (20/43, 47%) with a decreased response almost always in the affected ear (19/20, 95%) when observed. The caloric asymmetry was less during the irritative/recovery phases than between the attacks (p = 0.007). In contrast, the caloric asymmetry was larger during the paretic phase than between the attacks (p = 0.041). The caloric asymmetry decreased during the irritative/recovery phases with an estimated mean difference of 34% (adjusted 95% CI 16–53%, p < 0.001) and 23% (adjusted 95% CI 8–38%, p = 0.002) compared to the paretic and interictal phases. Caloric responses fluctuate during and between the attacks of MD depending on the electrophysiologic status of the vestibular afferents. The results of caloric tests in MD, thus should be interpreted with consideration of each phase when the evaluation was done. [ABSTRACT FROM AUTHOR]

We aimed to characterize the central positional nystagmus (CPN) observed in lesions involving the inferior cerebellar peduncle (ICP). We analyzed the clinical and neurotologic findings in six patients with an isolated unilateral ICP infarction that had been diagnosed at a university hospital in South Korea from 2003 to 2019. Patients usually presented with acute vestibular syndrome in isolation (4/6, 67%). Ipsilesional spontaneous nystagmus was observed in five while supine (5/6, 83%). Four (4/6, 67%) patients showed CPN which included apogeotropic (n = 3) or geotropic (n = 1) during head turning to either side while supine, and vertical nystagmus during straight-head hanging, Dix-Hallpike maneuvers, or up-righting (n = 3). Four patients showed contraversive ocular tilt reaction or tilt of the subjective visual vertical. Bedside and video head-impulse tests were normal in all patients. CPN is a usual finding in ICP lesions, and may be ascribed to damage of the fibers running from the nodulus/uvula onto the vestibular nucleus via the ICP. [ABSTRACT FROM AUTHOR]

The bilateral lateral medullary lesions disrupt both the inhibitory Purkinje outflow to bilateral vestibular nuclei and the climbing fibers participating in the cerebellar VOR adaptation, resulting in phenotypically identical HIS compared to the global cerebellar lesions. During vertical HITs for left or bilateral anterior canal semicircular canals, the VOR gain was slightly increased and small corrective saccades were followed in the anti-compensatory direction (Fig. Normally, vertical eye movements derived from each vertical canal are offset but not when the anterior canal gain is increased due to the vestibulocerebellar lesions [[9]]. [Extracted from the article]

Paroxysmal positional nystagmus frequently occurs in lesions involving the cerebellum, and has been ascribed to disinhibition and enhanced canal signals during positioning due to cerebellar dysfunction. This study aims to elucidate the mechanism of central positional nystagmus (CPN) by determining the effects of baclofen on the intensity of paroxysmal positional downbeat nystagmus due to central lesions. Fifteen patients with paroxysmal downbeat CPN were subjected to manual straight head-hanging before administration of baclofen, while taking baclofen 30 mg per day for at least one week, and two weeks after discontinuation of baclofen. The maximum slow phase velocity (SPV) and time constant (TC) of the induced paroxysmal downbeat CPN were analyzed. The positional vertigo was evaluated using an 11-point numerical rating scale (0 to 10) in 9 patients. After treatment with baclofen, the median of the maximum SPV of paroxysmal downbeat CPN decreased from 30.1°/s [interquartile range (IQR) = 19.6—39.0°/s] to 15.2°/s (IQR = 11.2—22.0°/s, Wilcoxon signed rank test, p < 0.001) with the median decrement ratio at 40.2% (IQR = 28.2—50.6%). After discontinuation of baclofen, the maximum SPV re-increased to 24.6°/s (IQR = 13.1—34.4°/s, Wilcoxon signed rank test, p = 0.001) with the median increment ratio at 23.5% (IQR = 5.2—87.9%). In contrast, the TCs of paroxysmal downbeat CPN remained unchanged at approximately 3.0 s throughout the evaluation. The positional vertigo also decreased with the medication (Wilcoxon signed rank test, p = 0.020), and remained unchanged even after discontinuation of medication (Wilcoxon signed rank test, p = 0.737). The results of this study support the prior presumption that paroxysmal CPN is caused by enhanced responses of the semicircular canals during positioning due to cerebellar disinhibition. Baclofen may be tried in symptomatic patients with paroxysmal CPN. [ABSTRACT FROM AUTHOR]