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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]

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]

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]

Even though dizziness is a common symptom of heat illness, comprehensive evaluation of the vestibular function has not been available in this potentially life-threatening disorder. Three patients developed vertigo about a week after heat exposure. Evaluation showed bilateral impairments of the vestibulo-ocular reflex during head impulses and the signs of vestibulocerebellar dysfunction that included spontaneous downbeat nystagmus, gaze-evoked nystagmus, and positional downbeat nystagmus. Exposure to excessive heat may give rise to delayed vestibulopathy by damaging the vestibulocerebellum. [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]

Rotational vertebral artery syndrome (RVAS) is characterized by recurrent attacks of paroxysmal vertigo, nystagmus, and ataxia induced by head rotation. We report on a patient who developed atypical RVAS due to compression of the vertebral artery (VA) terminating in the posterior inferior cerebellar artery (PICA). A 59-year-old man suffered from vertigo and nystagmus induced by leftward head rotation and oculography showed right beating horizontal-torsional and downbeat nystagmus. Cerebral angiography showed hypoplastic right VA terminating in PICA without connection to the basilar artery. The basilar artery received its flow from the left VA only and branched out both anterior inferior cerebellar arteries. Cerebral angiography revealed a complete occlusion of the right distal VA at the level of the C1-2 junction when the head was rotated to a leftward position. In contrast, the blood flow through the left vertebral and basilar arteries remained intact while turning the head to either side. The hemodynamic compromise observed in our patient with RVAS indicates that isolated vertigo and nystagmus may occur due to transient ischemia of the inferior cerebellum or lateral medulla. [ABSTRACT FROM AUTHOR]

Objective Perverted downbeat head-shaking nystagmus (pdHSN) has been considered a sign of central pathology. Two hypotheses have been advanced as the mechanisms of pdHSN without proper validation. This study aimed to elucidate the mechanism of pdHSN. Methods Eighteen patients with pdHSN due to central lesions were subjected to analyses of their oculographic characteristics. The peak velocity, temporal features including the duration and time constant (TC), rotational axis of pdHSN were analyzed. To determine the most relevant mechanism of pdHSN, we compared the TCs of pdHSN with those of downbeat nystagmus after vertical head-shaking in four subjects, and with the TCs of horizontal head-shaking nystagmus (HSN) in three subjects who had both horizontal and downbeat nystagmus after head-shaking. Results The duration of pdHSN ranged from 14 to 25 s and the estimated TC was from 3.9 to 7.8 s. When the data were pooled after the intensities of the nystagmus in each patient were normalized, the TC was 5.2 (95% CI = 5.0–5.4) seconds from nonlinear regression test. The rotational vectors of pdHSN ( n = 8) were mostly aligned between the anterior semicircular canals. The estimated TC of downbeat nystagmus after vertical head-shaking (5.8 s) was similar to that of pdHSN (6.0 s) in four subjects tested. In contrast, the TC of horizontal HSN (10.9 s) was significantly larger than that of pdHSN (4.9 s) in three subjects. Conclusions The characteristics of TCs of HSN in our patients with central lesions suggest that pdHSN is due to enhanced activities of the central anterior canal pathway. Significance pdHSN is a sign indicative of central pathology causing cerebellar dysfunction, especially when it associated with other neuro-otological signs. [ABSTRACT FROM AUTHOR]