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A clinical scale fully dedicated to evaluating ocular motor abnormalities is required for now. We investigated the utility of a recently developed Scale for Ocular motor Disorders in Ataxia (SODA) in patients with multiple system atrophy (MSA). We prospectively assessed SODA in consecutive patients with MSA between August 2021 and August 2023 at the Korea University Medical Center. The results of the clinical exam-based SODA were compared with those measured using video-oculography (VOG-guided SODA). We also compared the findings with other established clinical scales targeting patients with MSA, including the Unified Multiple System Atrophy Rating Scale (UMSARS) I-II, Movement Disorder Society-Unified Parkinson's Disease Rating Scale motor part (UPDRS-III), Scale for Assessment of Rating of Ataxia (SARA), Composite Autonomic Symptom Score-31 (COMPASS-31), and Composite Autonomic Severity Score (CASS). Twenty patients were enrolled in our study (17 with cerebellar-type MSA and three with Parkinson-type MSA). Scores ranged from 1 to 14 (median [interquartile range (IQR)] = 8 [5−10]). Among the subscales, saccades had a median score of 2.5 (IQR = 1–3), followed by ocular pursuit (1 [0–1]), nystagmus (1 [0–2]), saccadic intrusions (1 [0–1]), vestibulo-ocular reflex (VOR) (0.5 [0–1]), ocular alignment (0 [0–1]), and VOR cancellation (1 [0–1]). The clinical-exam-based SODA (p = 0.020) and VOG-guided SODA (p = 0.034) positively correlated with disease duration. No correlation was found between clinical exam-based SODA and other scales. Skew deviation, gaze-evoked nystagmus, VOR cancellation, and smooth pursuit had the highest precision among the items. Ocular misalignment and spontaneous and positional nystagmus were frequently false positive and were poorly detected with clinical exam-based SODA. Six patients with repeated evaluation exhibited higher scores, along with deterioration documented on other clinical scales. The SODA can reliably predict neurodegeneration as an additional clinical surrogate in MSA. [ABSTRACT FROM AUTHOR]

Opsoclonus refers to saccadic oscillations without an intersaccadic interval occurring in multiple planes. Opsoclonus mostly indicates dysfunction of the brainstem or cerebellum. We report opsoclonus induced by horizontal head-shaking without other signs of brainstem or cerebellar dysfunction in two patients with vestibular migraine (VM). The development of opsoclonus after horizontal head-shaking indicates unstable or hyperactive neural circuits between the excitatory and inhibitory saccadic premotor burst neurons in these patients with VM. [ABSTRACT FROM AUTHOR]

This document, titled "Ocular Flutter Evoked by Vestibular Stimulation," discusses a case study of a 26-year-old woman who experienced ocular flutter, a condition characterized by spontaneous bursts of eye movements, exclusively during head motion and vestibular stimulation. The patient presented with oscillopsia (the perception of objects moving) and other symptoms such as clumsiness and headache. Various tests and examinations were conducted, including video-oculography, which revealed the presence of ocular flutter during specific stimuli. The authors propose several mechanisms that may explain the occurrence of ocular flutter in this case, involving the interaction between the vestibular and saccadic systems. The patient's symptoms improved after receiving treatment with intravenous immunoglobulin and methylprednisolone. The study suggests that vestibular stimulation may play a role in generating ocular flutter. [Extracted from the article]

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]

The mechanism of vestibular syncope, the syncope occurring during the vertigo attacks, remains uncertain. This study aims to clarify the mechanism of vestibular syncope by pursuing the function of vestibular system in cardiovascular autonomic control and by defining neuro-hemodynamic changes in vestibular syncope. By integrating the velocity-storage (VS) circuit in the brainstem and cerebellum, we propose that the vestibular syncope develops as a result of dyssynergia of the vestibulosympathetic and baroreflexes in which centrally estimated downward inertial acceleration during the vertigo attacks acts as a trigger. Recognition of the vestibular disorders as a possible cause of syncope would allow proper managements for prevention of further syncope and related complications in patients with vestibular disorders. [ABSTRACT FROM AUTHOR]

Hyperventilation changes the extracellular pH modulating many central pathologies, such as tremor. The questions that remain unanswered are the following: (1) Hyperventilation modulates which aspects of the oscillations? (2) Whether the effects of hyperventilation are instantaneous and the recovery is rapid and complete? Here we study the effects of hyperventilation on eye oscillations in the syndrome of oculopalatal tremor (OPT), a disease model affecting the inferior olive and cerebellar system. These regions are commonly involved in the pathogenesis of many movement disorders. The focus on the ocular motor system also allows access to the well-known physiology and precise measurement techniques. We found that hyperventilation causes modest but insignificant changes in the intensity of oscillation displacement (i.e., how large the eye excursions are) and velocity (i.e., how fast do the eyes move during oscillations). We found the robust increase in the randomness of the oscillatory waveform during hyperventilation and it instantaneously reverts to the baseline after hyperventilation. The subsequent analysis classified the oscillations according to their waveform shape and randomness into different clusters. The hyperventilation substantially changed the cluster type in 60% of the subjects, but it reverted to the pre-hyperventilation cluster at the conclusion of the hyperventilation. In summary, hyperventilation instantaneously affects the randomness of the oscillatory waveforms but there are less substantial effects on the intensity. The deficits reverse immediately at the end of the hyperventilation. [ABSTRACT FROM AUTHOR]

We report atypical opsoclonus in a patient with multiple system atrophy and propose a mechanism based on the patterns of modulation by visual, vestibular, and saccadic and vergence stimulation. Firstly, the 6-Hz opsoclonus mostly in the vertical plane occurred only after the development of downbeat nystagmus in darkness without visual fixation. Even after a substantial build-up, visual suppression of the opsoclonus was immediate and complete. Furthermore, the latency for re-emergence of opsoclonus in darkness was greater when the duration of preceding visual fixation was longer. Secondly, the effect of preceding downbeat nystagmus on the development of opsoclonus was evaluated by changing the head position. The opsoclonus did not occur in the supine position when the downbeat nystagmus was absent. After horizontal head shaking, the opsoclonus in the vertical plane gradually evolved into horizontal plane and resumed its vertical direction again after vertical head shaking. Thirdly, any opsoclonus was not triggered by imaginary saccades in the supine position. Lastly, combined vergence and saccadic eye movements during the Müller paradigm did not induce opsoclonus. From these findings of modulation, we suggest that the opsoclonus observed in our patient was invoked by vestibular signals. When the function of the omnipause neurons and saccadic system was impaired, the centrally mediated vestibular eye velocity signals may activate the saccadic system to generate opsoclonus. These atypical patterns of opsoclonus, distinct from the classic opsoclonus frequently observed in para-neoplastic or para-infectious disorders, may be an unrevealing sign of degenerative brainstem or cerebellar disorders. [ABSTRACT FROM AUTHOR]

We investigated the vestibular perception of position, velocity, and time (duration) in humans with rotational stimuli including low velocities and small amplitudes. The participants were categorized into young, middle, and old age groups, and each consisted of 10 subjects. Position perception was assessed after yaw rotations ranged from 30 to 180° in both clockwise and counterclockwise directions. For each position, the rotation was delivered at two or more different velocities ranging from 15 to 120°/s. Position perception tended to underestimate the actual position and was similar during the slow and fast rotations. However, the trends of underestimation disappeared in the old age group. Velocity perception was evaluated by forcing the selection of the faster direction in each pair of rotations toward two positions (30° and 60°) with velocity differences from 0 to 20°/s. Velocity discrimination was similar between the rotation amplitudes or among the age groups. For duration perception, participants chose the rotation of longer duration for three test paradigms with different amplitudes (small vs. large) and durations (short vs. long) of rotation. The accuracy of discriminating duration was similar across the test paradigms or age groups, but the precision was lower in the older group and altered significantly according to the test paradigm. In conclusion, vestibular perception can be assessed using rotations of low velocities and small amplitudes. The perception of position and duration is affected by aging. The precision of duration perception can be influenced by the interactions between the amplitude and duration of motion. [ABSTRACT FROM AUTHOR]

This study aimed to determine the prevalence and mechanism of linear vertigo reported by the patients during the attacks of benign paroxysmal positional vertigo (BPPV). We prospectively evaluated the characteristics (rotational vs. linear) of positional vertigo in 70 patients with posterior and horizontal canal BPPV using a questionnaire allowing multiple choices. In patients with linear vertigo, we further assessed the directionality of linear vertigo. We adopted the velocity-storage model to explain the occurrence and direction of linear vertigo in these patients with BPPV. Patients reported only rotational vertigo in 46 (46/70, 65.7%), only linear vertigo in 10 (14.3%), and both rotational and linear vertigo in 14 (20%). The patients experienced fear from rotational vertigo in 54 (54/70, 77.1%) and from linear vertigo in 20 (20/70, 28.6%). The direction of linear vertigo was concordant with the direction of inertial acceleration predicted by the velocity-storage model. Patients with BPPV may experience linear as well as rotational vertigo during the attacks. This linear vertigo may be ascribed to centrally estimated inertial acceleration. [ABSTRACT FROM AUTHOR]

The virtual practice has made major advances in the way that we care for patients in the modern era. The culture of virtual practice, consulting, and telemedicine, which had started several years ago, took an accelerated leap as humankind was challenged by the novel coronavirus pandemic (COVID19). The social distancing measures and lockdowns imposed in many countries left medical care providers with limited options in evaluating ambulatory patients, pushing the rapid transition to assessments via virtual platforms. In this novel arena of medical practice, which may form new norms beyond the current pandemic crisis, we found it critical to define guidelines on the recommended practice in neurotology, including remote methods in examining the vestibular and eye movement function. The proposed remote examination methods aim to reliably diagnose acute and subacute diseases of the inner-ear, brainstem, and the cerebellum. A key aim was to triage patients into those requiring urgent emergency room assessment versus non-urgent but expedited outpatient management. Physicians who had expertise in managing patients with vestibular disorders were invited to participate in the taskforce. The focus was on two topics: (1) an adequate eye movement and vestibular examination strategy using virtual platforms and (2) a decision pathway providing guidance about which patient should seek urgent medical care and which patient should have non-urgent but expedited outpatient management. [ABSTRACT FROM AUTHOR]

Impaired tilt suppression of post-rotatory nystagmus and cross-coupled head-shaking nystagmus in cerebellar lesions: image mapping study. Lesions involving the ION have mostly been reported as a form of pseudo-hypertrophic olivary degeneration (HOD) that refers to vacuolar degeneration of the enlarged neurons in the ION and mostly develops several months after damage to the dentato-rubro-olivary tract (the Guillain-Mollaret triangle) [[2]]. The inferior olivary nucleus (ION) receives signals from the spinal cord and cerebellum, and regulates coordination and motor learning [[1]]. [Extracted from the article]

This study aimed to define the clinical features and involved structures that aid in differentiation of neuromyelitis optica spectrum disorder (NMOSD) from multiple sclerosis (MS) involving the brainstem and cerebellum. We analyzed the clinical and ocular motor findings, and lesion distribution on brain MRIs in 42 patients with MS (17 men, mean age ± SD = 37 ± 12) and 26 with NMOSD (3 men, mean age ± SD = 43 ± 15) that were recruited from two university hospitals in South Korea (whole study population). An additional subgroup analysis was also conducted in 41 patients presenting acute brainstem or vestibular syndrome (brainstem syndrome population). Logistic regression analysis showed that bilaterality of the lesions (p = 0.012) and presence of horizontal gaze-evoked nystagmus (hGEN, p = 0.041) were more frequently associated with NMOSD than with MS in the whole study population. In the brainstem syndrome population, only hGEN (p = 0.017) was more frequent in NMOSD than in MS. The lesions specific for NMOSD were overlapped in the medial vestibular nucleus (MVN) and nucleus prepositus hypoglossi (NPH) at the pontomedullary junction. In conclusion, presence of hGEN and bilateral lesions involving the MVN and NPH favor the diagnosis of NMOSD rather than MS. [ABSTRACT FROM AUTHOR]

The mechanism of perverted vertical responses during horizontal head impulse tests (HITs) requires further elucidation. A 47-year-old woman with a Chiari malformation showed alternating skew deviation, downbeat nystagmus with an increasing slow phase velocity, impaired smooth pursuit, and upward ocular deviation during horizontal HITs and corrective downward saccades in the presence of normal bithermal caloric tests and intact tilt suppressions of the post-rotatory nystagmus. These findings suggest dysfunction of the inferior cerebellum including the tonsil, nodulus, and uvula. We propose that disruption of signals from the medial part of the vestibulocerebellum, which normally inhibits the lateral and anterior canal pathways, may elicit an upward misdirection of the eye velocity during rapid horizontal head rotation. Otherwise, the Chiari malformation may have directly affected the brainstem structures involved in the direction matrix of the vestibulo-ocular reflex. [ABSTRACT FROM AUTHOR]

Opsoclonus often presents with other neurological symptoms and signs such as dizziness, myoclonus, ataxia, and mental confusion [[3]]. In this study, we report vestibular hyperreflexia in a patient with opsoclonus-myoclonus syndrome as a rare complication of acute hepatitis A virus (HAV) infection. Based on the clinical presentation, laboratory results, and response to the immune-modulating treatments, the patient was finally diagnosed with opsoclonus-myoclonus syndrome due to HAV-associated autoimmune encephalitis. Opsoclonus-myoclonus syndrome associated with mumps virus infection. [Extracted from the article]

We sought to determine the cerebellar structures responsible for tilt suppression of post-rotatory nystagmus. We investigated ocular motor findings and MRI lesions in 73 patients with isolated cerebellar lesions who underwent recording of the vestibulo-ocular reflex (VOR) using rotatory chair tests. Tilt suppression of post-rotatory nystagmus was diminished in 27 patients (27/73, 37.0 %). The gains of the VOR and the TCs of per- and post-rotatory nystagmus did not differ between the patients with diminished and with normal tilt suppression. The patients with impaired tilt suppression showed perverted ('cross-coupled') head-shaking nystagmus (pHSN) and central positional nystagmus (CPN) more frequently than those with normal responses. Tilt suppression was impaired in five (71.4 %) of the seven patients with isolated nodulus and uvular infarction. Probabilistic lesion-mapping analysis showed that the nodulus and uvula are responsible for tilt suppression. Impaired tilt suppression may be ascribed to disruption of cerebellar contribution to the vestibular velocity-storage mechanism, which integrates information from the semicircular canals and otolith organs to help derive the brain's estimate of the head orientation relative to the pull of gravity. [ABSTRACT FROM AUTHOR]

In spinocerebellar ataxia type 6 (SCA6), the vestibular dysfunction and its correlation with other clinical parameters require further exploration. We determined vestibular responses over a broad range of stimulus acceleration in 11 patients with SCA6 (six men, age range=33-72 years, mean age±SD=59±12 years) using bithermal caloric irrigations, rotary chair, and head impulse tests. Correlations were also pursued among disability scores, as measured using the International Cooperative Ataxia Rating Scale, disease duration, age at onset, cytosine-adenine-guanine (CAG) repeat length, and the gain of the vestibulo-ocular reflex (VOR). In response to relatively low-acceleration, low-frequency rotational and bithermal caloric stimuli, the VOR gains were normal or increased regardless of the severity of disease. On the other hand, with relatively high-acceleration, high-frequency head impulses, there was a relative increase in gain in the mildly affected patients and a decrease in gain in the more severely affected patients and gains were negatively correlated with the severity of disease (Spearman correlation, R=−0.927, p<0.001). Selective decrease of the vestibular responses during high-acceleration, high-frequency stimuli may be ascribed to degeneration of either the flocculus or vestibular nuclei. The performance of the VOR during high-acceleration, high-frequency head impulses may be a quantitative indicator of clinical decline in SCA6. [ABSTRACT FROM AUTHOR]

To define the cerebellar contribution in modulating the otolithic signals, we investigated the otolithic function in 27 patients with acute unilateral cerebellar infarctions in the territory of the posterior inferior cerebellar artery (PICA, n = 17, 63 %), combined PICA and superior cerebellar artery (SCA) ( n = 7, 30 %), SCA ( n = 2, 7 %), and anterior inferior cerebellar artery ( n = 1, 4 %) from 2010 to 2012. The patients had evaluation of the ocular tilt reaction [head tilt, ocular torsion (OT), and skew deviation], tilt of the subjective visual vertical (SVV), cervical vestibular evoked myogenic potentials (VEMPs) in response to air conducted tone bursts, and ocular VEMPs induced by tapping the head at AFz. The evaluation was completed within 2 weeks after symptom onset. Patients often showed OT or SVV tilt (15/27, 55.6 %) that was either ipsi- ( n = 6) or contraversive ( n = 9). Overall, there were no differences in the amplitudes and latencies of cervical and ocular VEMPs between the ipsi- and contralesional sides. However, individual analyses revealed frequent abnormalities of cervical (11/27, 41 %) and/or ocular (9/27, 33 %) VEMPs. While 11 (73 %) of the 15 patients with the OTR/SVV tilt showed abnormalities of cervical ( n = 9) and/or ocular ( n = 7) VEMP responses, only three (25 %) of the 12 patients without the OTR/SVV tilt had abnormal cervical ( n = 2) and/or ocular ( n = 2) VEMPs (73 % vs. 25 %, Fisher's exact test, p = 0.021). The concordance rate in the results of cervical and ocular VEMPs was marginally significant (19/27, 70 %, p = 0.052, binominal). Unilateral cerebellar lesions may generate otolithic imbalances, as evidenced by the OTR/SVV tilt and asymmetric ocular or cervical VEMP responses, but without directionality according to the lesion side. Patients with the OTR/SVV tilt had abnormal VEMPs more often than those without. [ABSTRACT FROM AUTHOR]

Patients with cerebellar lesions may show horizontal (positive)- or downward (perverted)-corrective saccades during horizontal head impulse test (HIT). However, corrective saccades in the direction of head rotation (reversed corrective saccades) have not been reported during HIT. We present two patients who showed reversed corrective saccades during horizontal HIT as an initial sign of acute cerebellitis. In contrast to the corrective saccades mostly observed in peripheral vestibular paresis, this paradoxical response indicates abnormally increased vestibulo-ocular responses due to cerebellar disinhibition over the vestibulo-ocular reflex. This paradoxical response should be considered an additional bedside cerebellar sign. [ABSTRACT FROM AUTHOR]

Rebound nystagmus is a transient nystagmus that occurs on resuming the straight-ahead position after prolonged eccentric gaze. Even though rebound nystagmus is commonly associated with gaze-evoked nystagmus (GEN), development of rebound nystagmus in a different plane of gaze has not been described. We report a patient with episodic ataxia type 2 who showed transient upbeat nystagmus on resuming the straight-ahead position after sustained lateral gaze that had induced GEN and downbeat nystagmus. The rebound upbeat nystagmus may be ascribed to a shifting null in the vertical plane as a result of an adaptation to the downbeat nystagmus that developed during lateral gaze. [ABSTRACT FROM AUTHOR]

The article discusses a case study of a 57-year-old man with multiple system atrophy (MSA) presenting with ocular flutter triggered specifically by vestibular stimulation, alongside various neurological symptoms including gait difficulty, oscillopsia, and dysarthria. It highlights the diagnostic challenges and neurological manifestations associated with MSA, emphasizing the role of vestibular and ocular motor deficits in differentiating this condition from other neurodegenerative disorders.

Two vestibular signals, rotational and inertial cues, converge for the perception of complex motion. However, how vestibular perception is built on neuronal behaviors and decision-making processes, especially during the simultaneous presentation of rotational and inertial cues, has yet to be elucidated in humans. In this study, we analyzed the perceptual responses of 20 participants after pairwise rotational experiments, comprised of four control and four test sessions. In both control and test sessions, participants underwent clockwise and counterclockwise rotations in head-down and head-up positions. The difference between the control and test sessions was the head re-orientation relative to gravity after rotations, thereby providing only rotational cues in the control sessions and both rotational and inertial cues in the test sessions. The accuracy of perceptual responses was calculated by comparing the direction of rotational and inertial cues acquired from participants with that predicted by the velocity-storage model. The results showed that the accuracy of rotational perception ranged from 80 to 95% in the four control sessions but significantly decreased to 35 to 75% in the four test sessions. The accuracy of inertial perception in the test sessions ranged from 50 to 70%. The accuracy of rotational perception improved with repetitive exposure to the simultaneous presentation of both rotational and inertial cues, while the accuracy of inertial perception remained steady. The results suggested a significant interaction between rotational and inertial perception and implied that vestibular perception acquired in patients with vestibular disorders are potentially inaccurate. [ABSTRACT FROM AUTHOR]

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]