Best Oculomotor Endpoints for Clinical Trials in Hereditary Ataxias: A Systematic Review and Consensus by the Ataxia Global Initiative Working Group on Digital‑Motor Biomarkers.

Oculomotor deficits are common in hereditary cerebellar ataxias (HCAs) and their quantitative assessment offers a sensitive and reliable manner to capture disease-severity and progression. As a group of experts of the Ataxia Global Initiative to support trial readiness, we previously established harmonized methodology for quantitative oculomotor assessments in HCAs. Here, we aimed to identify to most promising oculomotor/vestibular outcomes as endpoints for future trials.

Vestibular Loss and Cerebellar Ataxia: A Practical Approach.

Cerebellar ataxia has been the remit of neurology but increased awareness of genetic disorders affecting both cerebellar and vestibular function has led to a rise in their reported prevalence. It is not uncommon for such patients to be seen in otolaryngology clinics. This review explores the underlying etiologies of patients presenting with vestibular loss accompanied by signs of cerebellar ataxia and provides a practical approach to diagnosis.

The Challenge of Diagnosing Labyrinthine Stroke-A Critical Review.

Acute vertigo or dizziness that is accompanied by a sudden sensorineural hearing loss (SSNHL) often poses a diagnostic challenge. While a combined audiovestibular deficit makes an inner ear pathology most likely, this does not necessarily exclude a vascular pathology that may be a harbinger of future sinister events. This is especially true for strokes within the territory of the anterior inferior cerebellar artery (AICA), because the labyrinth receives its vascular supply most often by branches of the AICA.

[Acute and episodic dizziness - diagnosis and treatment].

Dizziness is one of the most common, yet diagnostically challenging cardinal symptoms with a wide range of causes. It accounts for 2-8 % of all consultations in both the emergency room and in general practice. The extremely broad differential diagnosis, which covers numerous medical specialties, the often transient nature of the symptoms, and the difficulty for patients to put the experienced symptoms into words, often make the evaluation of dizziness challenging.

Diagnosis and treatment of vertigo and dizziness : Interdisciplinary guidance paper for clinical practice.

Background: Vertigo and dizziness belong to the most common leading symptoms in clinical practice. Their differential diagnosis, however, often imposes a challenge.

Objective: This work aims to provide evidence-based and practice-oriented recommendations for diagnosis and treatment of vertigo and dizziness for primary care providers.

Transient Response to Liberation Maneuvers in Central Positional Nystagmus Due to Cerebral Metastases Mimicking Benign Paroxysmal Positional Vertigo- A Case Report.

Background: Benign paroxysmal positional vertigo (BPPV) is by far the most frequent cause of positional nystagmus (PN). However, PN may also be encountered in central lesions. In this case report we describe a patient with isolated positional vertigo and central PN which mimicked a lateral-canal cupulithiasis, including initial response to liberation maneuvers.

Usefulness of Nystagmus Patterns in Distinguishing Peripheral From Central Acute Vestibular Syndromes at the Bedside: A Critical Review.

Vertigo and dizziness are amongst the most frequent presenting symptoms in the emergency room, accounting for up to 4% of all emergency consultations. The broadness of their differential diagnosis and the often transient nature of these symptoms pose a significant challenge to the treating physician. Combining various subtle oculomotor signs at the bedside has been very successful in distinguishing peripheral from central causes in acutely dizzy patients meeting diagnostic criteria for the acute vestibular syndrome (i.

[Diagnosis and treatment of vertigo and dizziness : Interdisciplinary guidance paper for clinical practice. German version].

Background: Vertigo and dizziness belong to the most common leading symptoms in clinical practice. Their differential diagnosis, however, often poses a challenge.

Objective: This work aims to provide evidence-based and practice-oriented recommendations for diagnosis and treatment of vertigo and dizziness in primary care.

The Spectrum of Peripheral-Vestibular Deficits and Their Change Over Time in CANVAS/RFC1-Related Ataxia Systematic Review and Meta-Analysis of Quantitative Head-Impulse Testing.

Cerebellar ataxia, neuropathy, vestibular-areflexia syndrome (CANVAS) has been linked to biallelic intronic repeat-expansions in RFC1. Video-head-impulse testing (vHIT) offers a quantitative assessment of the angular vestibulo-ocular reflex (aVOR) of all three canals. We evaluated patterns of peripheral-vestibular impairment, its change over time and evaluated correlations with other parameters.

The International Classification of Vestibular Disorders: Achievements, challenges, and future directions.

In 2007, the Bárány Society embarked on a project to establish definitions of vestibular syndromes and disorders based on best available evidence, referred to as the International Classification of Vestibular Disorders (ICVD). Since then, numerous publications providing consensus-driven diagnostic criteria for vestibular symptoms, syndromes, and disorders have been published. Here, we reflect on the rationale for developing the ICVD as well as its subsequent achievements, challenges, and outlook.

First ischemic stroke in young adults: Sex and age-related differences in stroke rates, risk factors, and etiologies.

Introduction: Sex differences in stroke incidence, vascular risk factors (VRFs), and etiologies among young adults remain underexplored, particularly regarding age-related patterns.

Patients And Methods: We retrospectively analyzed young adults (18-55 years) with first-ever ischemic stroke treated at certified acute stroke units/centers between 2014 and 2022, using Swiss Stroke Registry data. Stroke rates (per 100,000 person-years), VRFs, and etiologies were assessed by age and sex.

Diagnostic Errors in the Acutely Dizzy Patient-Lessons Learned.

Acute vertigo or dizziness is a frequent presentation to the emergency department (ED), making up between 2.1% and 4.4% of all consultations.

Intravenous thrombolysis in patients with acute dizziness or imbalance and suspected ischemic stroke-systematic review.

Background: Correct identification of those patients presenting with an acute vestibular syndrome (AVS) or an acute imbalance syndrome (AIS) that have underlying posterior-circulation stroke (PCS) and thus may benefit from revascularization (intravenous thrombolysis (IVT), endovascular therapy (EVT)) is important. Treatment guidelines for AVS/AIS patients are lacking. We reviewed the evidence on acute treatment strategies in AVS/AIS focusing on predictors for IVT/EVT and outcome.

Quantitative Oculomotor and Vestibular Profile in Spinocerebellar Ataxia Type 6 - Systematic Review and Meta-Analysis.

Whereas several studies have reported on quantitative oculomotor and vestibular measurements in spinocerebellar ataxia type 6 (SCA6), selecting the most suitable paradigms remains challenging. We aimed to address this knowledge gap through a systematic literature review and providing disease-specific recommendations for a tailored set of eye-movement recordings in SCA6. A literature search (MEDLINE, Embase) was performed focusing on studies reporting on quantitative oculomotor and/or vestibular measurements in SCA6-patients.

The pioneers of vestibular physiology in the 19th century.

It was known from ancient times that vertigo was a malady and that the inner ears of animals contained an intricate network of structures named the labyrinth, whose function was unknown. The flourishing of human vestibular anatomy in the Renaissance period still adhered to age-old notions of traditional spiritual philosophy. In the post-Renaissance period, when science was being redefined and challenging these traditional thoughts, vestibular physiology was born.

Patient-Related Outcome Measures for Oculomotor Symptoms in the Cerebellar Ataxias: Insights from Non-Cerebellar Disorders.

In patients with cerebellar ataxia (CA), symptoms related to oculomotor dysfunction significantly affect quality of life (QoL). This study aimed to analyze the literature on patient-related outcome measures (PROMs) assessing QoL impacts of vestibular and cerebellar oculomotor abnormalities in patients with CA to identify the strengths and limitations of existing scales and highlight any areas of unmet need. A systematic review was conducted (Medline, Embase) of English-language original articles reporting on QoL measures in patients with vertigo, dizziness or CA.

What's in a Name? Chronic Vestibular Migraine or Persistent Postural Perceptual Dizziness?

Current consensus diagnostic criteria for vestibular migraine (VM) describes this as an episodic disorder. However, a minority of patients report prolonged (>72 h duration) or even persistent VM symptoms, prompting whether a chronic variant of vestibular migraine (CVM) should be introduced to the current classification and how best to define it. Here we summarize current evidence of such a potential chronic variant of VM and critically review proposed definitions for CVM.

Treatment-induced neuropathy of diabetes: challenges in diagnosing neuropathic pain, value of sudomotor function testing.

A patient in his 60s was admitted for an extensive neurological work-up due to progressive asymmetrical, distally pronounced pain in both feet and legs. Conventional pain relievers did not help in pain reduction. A Sudoscan revealed small fibre damage in all extremities indicating an underlying neuropathy.

[Diagnosis and treatment of benign paroxysmal positional vertigo with manual and motorized chairs - Indication and value].

Benign paroxysmal positional vertigo (BPPV) is the most frequent cause of episodic vertigo or dizziness. While this diagnosis can be made reliably in most cases by use of targeted history taking followed by provocation maneuvers on the examination couch and subsequent repositioning maneuver, these maneuvers may not be applicable in a subgroup of patients with pre-existing conditions such as musculoskeletal or neurologic disorders. At the same time, part of patients treated on the examination couch will not respond to the repositioning maneuvers.