Physiological oculo-auricular-facial-mandibular synkinesis elicited in humans by gaze deviations.

Integrated motor behaviors involving ocular motion-associated movements of the head, neck, pinna, and parts of the face are commonly seen in animals orienting to a visual target. A number of coordinated movements have also been observed in humans making rapid gaze shifts to horizontal extremes, which may be vestiges of these. Since such integrated mechanisms point to a nonpathological coactivation of several anatomically separate cranial circuits in humans, it is important to see how the different pairs of integrative motor behaviors with a common trigger (i.

Age-dependent perturbation of the perceptual and postural vertical by visual roll vection and susceptibility to motion sickness in children.

Background: The visual contribution to the perceptual and postural vertical is mediated by a multisensory integration process and may relate to children's susceptibility to motion sickness that is hypothesized to arise from intersensory conflicts.

Objective: To analyze the maturation of visual contribution to the perceptual and postural vertical in conjunction with the motion sickness susceptibility in childhood.

Methods: In 81 healthy children (aged 2-17 years; 57 females), adjustments of the subjective visual vertical and posturographically tested mediolateral displacements of body sway were measured during free upright stance and large-field visual motion stimulation in the roll plane (roll vection).

A cross-sectional, prospective ocular motor study in 72 patients with Niemann-Pick disease type C.

Objective: To characterize ocular motor function in patients with Niemann-Pick disease type C (NPC).

Methods: In a multicontinental, cross-sectional study we characterized ocular-motor function in 72 patients from 12 countries by video-oculography. Interlinking with disease severity, we also searched for ocular motor biomarkers.

Eye movement biomarkers allow for the definition of phenotypes in Gaucher Disease.

Background: Neurological forms of Gaucher disease, the inherited disorder of β-Glucosylceramidase caused by bi-allelic variants in GBA1, is a progressive disorder which lacks a disease-modifying therapy. Systemic manifestations of disease are effectively treated with enzyme replacement therapy, however, molecules which cross the blood-brain barrier are still under investigation. Clinical trials of such therapeutics require robust, reproducible clinical endpoints to demonstrate efficacy and clear phenotypic definitions to identify suitable patients for inclusion in trials.

Spontaneous visual exploration during locomotion in patients with phobic postural vertigo.

Background: Earlier studies on stance and gait with posturographic and EMG-recordings and automatic gait analysis in patients with phobic postural vertigo (PPV) or visual height intolerance (vHI) revealed similar patterns of body stiffening with muscle co-contraction and a slow, cautious gait. Visual exploration in vHI patients was characterized by a freezing of gaze-in-space when standing and reduced horizontal eye and head movements during locomotion.

Objective: Based on the findings in vHI patients, the current study was performed with a focus on visual control of locomotion in patients with PPV while walking along a crowded hospital hallway.

Perception of postural verticality in roll and pitch while sitting and standing in healthy subjects.

Assessment of verticality perception is increasingly used in patients with disorders of upright body orientation, e.g. in pusher behavior after hemispheric stroke or in retropulsion in neurovascular or degenerative diseases.

Health-related quality of life and functional impairment in acute vestibular disorders.

Background And Purpose: Acute vestibular symptoms have a profound impact on patients' well-being. In this study, health-related quality of life (HRQoL) and functional impairment were investigated prospectively in patients with different peripheral and central vestibular disorders during the acute symptomatic stage to decipher the most relevant underlying factors.

Methods: In all, 175 patients with acute vestibular disorders were categorized as central vestibular (CV, n = 40), peripheral vestibular (PV, n = 68) and episodic vestibular disorders (EV, n = 67).

No Evidence of a Contribution of the Vestibular System to Frequent Falls in Progressive Supranuclear Palsy.

Background And Purpose: Conflicting results about vestibular function in progressive supranuclear palsy (PSP) prompted a systematic examination of the semicircular canal function, otolith function, and postural stability.

Methods: Sixteen patients with probable PSP [9 females, age=72±6 years (mean±SD), mean disease duration=3.6 years, and mean PSP Rating Scale score=31] and 17 age-matched controls were examined using the video head impulse test, caloric testing, ocular and cervical vestibular evoked myogenic potentials (o- and cVEMPs), video-oculography, and posturography.

Towards computerized diagnosis of neurological stance disorders: data mining and machine learning of posturography and sway.

We perform classification, ranking and mapping of body sway parameters from static posturography data of patients using recent machine-learning and data-mining techniques. Body sway is measured in 293 individuals with the clinical diagnoses of acute unilateral vestibulopathy (AVS, n = 49), distal sensory polyneuropathy (PNP, n = 12), anterior lobe cerebellar atrophy (CA, n = 48), downbeat nystagmus syndrome (DN, n = 16), primary orthostatic tremor (OT, n = 25), Parkinson's disease (PD, n = 27), phobic postural vertigo (PPV n = 59) and healthy controls (HC, n = 57). We classify disorders and rank sway features using supervised machine learning.

Approach to an experimental model of Mal de Debarquement Syndrome.

Introduction: Mal de Debarquement Syndrome (MdDS) is the rare condition of enduring rocking sensations and subjective unsteadiness following a lengthy exposure to passive motion. The pathogenesis of MdDS is unknown and the available treatment is limited. Here, we developed an experimental model of MdDS that may facilitate systematic inquiry of MdDS pathophysiology and the development of prevention or treatment strategies for this condition.

Oculomotor and Vestibular Findings in Gaucher Disease Type 3 and Their Correlation with Neurological Findings.

Objectives: To evaluate the function of the oculomotor and vestibular systems and to correlate these findings with the clinical status of patients with Gaucher disease type 3 (GD3). The goal of this cross-sectional and longitudinal study was to find oculomotor biomarkers for future clinical trials.

Methods: Twenty-six patients with GD3 were assessed for eligibility and 21 were able to perform at least one task.

Protocol for a prospective interventional trial to develop a diagnostic index test for stroke as a cause of vertigo, dizziness and imbalance in the emergency room (EMVERT study).

Introduction: Identifying stroke as a cause of acute vertigo, dizziness and imbalance in the emergency room is still a clinical challenge. Many patients are admitted to stroke units, but only a minority will have strokes. This imposes a heavy financial burden on the healthcare system.

Vestibular function in patients with Niemann-Pick type C disease.

We investigated whether vestibular dysfunction may cause or contribute to postural imbalance and falls in patients with Niemann-Pick type C disease (NP-C). Eight patients with NP-C disease and 20 healthy controls were examined using the video-based head impulse test (vHIT) and caloric irrigation to investigate horizontal canal function as well as ocular- and cervical vestibular evoked myogenic potentials (o- and cVEMP), and binocular subjective visual vertical estimation (SVV) for otolith function, and static posturography. There were no significant differences in vestibulo-ocular gain, caloric excitability, o-/cVEMP measures or SVV between the two groups.

Subjective body vertical: a promising diagnostic tool in idiopathic normal pressure hydrocephalus?

Postural instability is a frequent symptom of patients with idiopathic normal pressure hydrocephalus (iNPH), and might be due to the misperception of body verticality. The objective of this study was to assess the usefulness of the subjective body vertical (SBV) as a potential tool for diagnosing iNPH. Twenty patients with iNPH underwent tests of SBV in the pitch and roll planes before and after cerebrospinal fluid (CSF) drainage.

Gravity matters: Motion perceptions modified by direction and body position.

Motion coherence thresholds are consistently higher at lower velocities. In this study we analysed the influence of the position and direction of moving objects on their perception and thereby the influence of gravity. This paradigm allows a differentiation to be made between coherent and randomly moving objects in an upright and a reclining position with a horizontal or vertical axis of motion.

Real-time computer-based visual feedback improves visual acuity in downbeat nystagmus - a pilot study.

Background: Patients with downbeat nystagmus syndrome suffer from oscillopsia, which leads to an unstable visual perception and therefore impaired visual acuity. The aim of this study was to use real-time computer-based visual feedback to compensate for the destabilizing slow phase eye movements.

Methods: The patients were sitting in front of a computer screen with the head fixed on a chin rest.

4-aminopyridine improves lower urinary tract symptoms in a patient with benign prostatic hyperplasia and downbeat nystagmus syndrome.

Aminopyridines are potassium channel blockers that increase the excitability of nerve cells and axons; therefore, they are widely used to treat different neurological disorders. Here we present a patient with idiopathic downbeat nystagmus and lower urinary tract symptoms (LUTS) due to benign prostatic hyperplasia who was treated with the sustained-release form of 4-aminopyridine (4-AP). During treatment with 4-AP, the LUTS improved.