Systematic Review and Meta-Analysis of the Diagnostic Accuracy of a Graded Gait and Truncal Instability Rating in Acutely Dizzy and Ataxic Patients.

BACKGROUND: In patients presenting with acute prolonged vertigo and/or gait imbalance, the HINTS [Head-Impulse, Nystagmus, Test-of-Skew] are very valuable. However, their application may be limited by lack of training and absence of vertigo/nystagmus. Alternatively, a graded gait/truncal-instability (GTI, grade 0-3) rating may be applied. METHODS: We performed a systematic search (MEDLINE/Embase) to identify studies reporting on the diagnostic accuracy of bedside examinations in adults with acute vestibular syndrome. Diagnostic test properties were calculated for findings using a random-effects model. Results were stratified by GTI-rating used. RESULTS: We identified 6515 articles and included 18 studies (n = 1025 patients). Ischemic strokes (n = 665) and acute unilateral vestibulopathy (n = 306) were most frequent. Grade 2/3 GTI had moderate sensitivity (70.8% [95% confidence-interval (CI) = 59.3-82.3%]) and specificity (82.7 [71.6-93.8%]) for predicting a central cause, whereas grade 3 GTI had a lower sensitivity (44.0% [34.3-53.7%] and higher specificity (99.1% [98.0-100.0%]). In comparison, diagnostic accuracy of HINTS (sensitivity = 96.8% [94.8-98.8%]; specificity = 97.6% [95.3-99.9%]) was higher. When combining central nystagmus-patterns and grade 2/3 GTI, sensitivity was increased to 76.4% [71.3-81.6%] and specificity to 90.3% [84.3-96.3%], however, no random effects model could be used. Sensitivity was higher in studies using the GTI rating (grade 2/3) by Lee (2006) compared to the approach by Moon (2009) (73.8% [69.0-78.0%] vs. 57.4% [49.5-64.9%], p = 0.001). CONCLUSIONS: In comparison to HINTS, the diagnostic accuracy of GTI is inferior. When combined with central nystagmus-patterns, diagnostic accuracy could be improved based on preliminary findings. GTI can be readily applied in the ED-setting and also in patients with acute imbalance syndrome.

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. Pre-specified parameters were retrieved, including diseases studied, scales applied and conclusions drawn. Our search yielded 3671 articles of which 467 studies (n = 111,606 participants) were deemed relevant. The most frequently studied disease entities were (a) non-specific dizziness/gait imbalance (114 studies; 54,581 participants), (b) vestibular schwannomas (66; 15,360), and (c) vestibular disorders not further specified (66; 10,259). The Dizziness Handicap Inventory (DHI) was the most frequently used PROM to assess QoL (n = 91,851), followed by the Penn Acoustic Neuroma Quality-of-Life Scale (n = 12,027) and the Activities-Specific Balance Confidence Scale (n = 2'471). QoL-scores capturing symptoms related to oculomotor abnormalities in CA were rare, focused on visual impairments (e.g., National-Eye-Institute Visual Function Questionnaire, Oscillopsia Functional Impact, oscillopsia severity score) and were unvalidated. The DHI remains the most widely used and versatile scale for evaluating dizziness. A lack of well-established PROMs for assessing the impact of oculomotor-related symptoms on QoL in CA was noted, emphasizing the need for developing and validating a new QoL-score dedicated to the oculomotor domain for individuals with CA.

Quantitative Oculomotor Assessment in Hereditary Ataxia: Discriminatory Power, Correlation with Severity Measures, and Recommended Parameters for Specific Genotypes.

Characterizing bedside oculomotor deficits is a critical factor in defining the clinical presentation of hereditary ataxias. Quantitative assessments are increasingly available and have significant advantages, including comparability over time, reduced examiner dependency, and sensitivity to subtle changes. To delineate the potential of quantitative oculomotor assessments as digital-motor outcome measures for clinical trials in ataxia, we searched MEDLINE for articles reporting on quantitative eye movement recordings in genetically confirmed or suspected hereditary ataxias, asking which paradigms are most promising for capturing disease progression and treatment response. Eighty-nine manuscripts identified reported on 1541 patients, including spinocerebellar ataxias (SCA2, n = 421), SCA3 (n = 268), SCA6 (n = 117), other SCAs (n = 97), Friedreich ataxia (FRDA, n = 178), Niemann-Pick disease type C (NPC, n = 57), and ataxia-telangiectasia (n = 85) as largest cohorts. Whereas most studies reported discriminatory power of oculomotor assessments in diagnostics, few explored their value for monitoring genotype-specific disease progression (n = 2; SCA2) or treatment response (n = 8; SCA2, FRDA, NPC, ataxia-telangiectasia, episodic-ataxia 4). Oculomotor parameters correlated with disease severity measures including clinical scores (n = 18 studies (SARA: n = 9)), chronological measures (e.g., age, disease duration, time-to-symptom onset; n = 17), genetic stratification (n = 9), and imaging measures of atrophy (n = 5). Recurrent correlations across many ataxias (SCA2/3/17, FRDA, NPC) suggest saccadic eye movements as potentially generic quantitative oculomotor outcome. Recommendation of other paradigms was limited by the scarcity of cross-validating correlations, except saccadic intrusions (FRDA), pursuit eye movements (SCA17), and quantitative head-impulse testing (SCA3/6). This work aids in understanding the current knowledge of quantitative oculomotor parameters in hereditary ataxias, and identifies gaps for validation as potential trial outcome measures in specific ataxia genotypes.

What is the current status of care by neuro-otology specialists in Switzerland-A national survey.

Background: Vertigo and dizziness are frequent presenting symptoms in the emergency department and in outpatient centers. While the majority of dizzy patients are evaluated by primary care physicians, specialists are often involved in the diagnostic workup. We aimed to gain more insights into the role of specialists in the care of dizzy patients. Materials and methods: Board-certified neurologists and ear-nose-throat (ENT) physicians working in Switzerland were invited to participate in an online survey. Descriptive statistical analyses were performed, and prospectively defined hypotheses were assessed using correlation analyses. Results: All 111 participating specialists (neurologists = 62; ENT specialists = 49) were familiar with testing for posterior canal benign paroxysmal positional vertigo (BPPV), and 66% regularly applied provocation maneuvers for suspected lateral canal BPPV. Reposition maneuvers for posterior (99%) and lateral (68%) canals were frequently performed. ENT physicians were familiar with lateral canal BPPV repositioning maneuvers significantly more often than neurologists (84 vs. 56%, p ≤ 0.012). Specialists strongly agreed that performing the head impulse test (86%) and looking for deficient eccentric gaze holding (82%) are important. Compared to neurologists, significantly fewer ENT physicians indicated ordering brain MRI in acutely dizzy patients (OR = 0.33 [0.16-0.067], p = 0.002) and physical therapy in patients with acute (50 vs. 20%, p = 0.005) or episodic/chronic dizziness (78 vs. 50%, p = 0.003). Conclusion: We found substantial differences in the care of dizzy patients by neurologists and ENT physicians. This underlines the need for a standardized, guideline-oriented diagnostic workup and treatment across specialties. Dedicated training for performing lateral canal BPPV repositioning maneuvers should be prioritized for neurologists. Similarly, physical therapy should be considered more often by ENT physicians.

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. Potential approaches to establish a diagnostic framework for CVM include (a) following the distinction between episodic and chronic migraine headaches, namely, frequent and/or prolonged episodes of VM (but not persistent vertigo or dizziness) in the context of chronic migraine headaches or (b) daily dizzy spells over more than 6 months that responded well to prophylactic anti-migraine therapy. A key challenge when defining diagnostic criteria for CVM is how to distinguish it from other chronic vestibular syndromes such as motion sickness, persistent postural-perceptual dizziness (PPPD), and mal de débarquement syndrome. Indeed, more than 50% of patients with PPPD and up to 46% with mal de débarquement syndrome fulfil diagnostic criteria for episodic migraine headaches, suggesting these disorders may all lie along a spectrum. We propose that when VM becomes persistent, it is best classified as PPPD but that VM and PPPD are not mutually exclusive, such that patients with PPPD need not have features of VM, and the triggering event for persistent dizziness may be independent of migraine. However, further research is needed to better characterize the spectrum of clinical phenotypes in patients with chronic dizziness, migraine headaches and anxiety, to define whether a chronic variant of VM sufficiently differs from current persistent dizziness definitions.

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. The patient had started insulin treatment around 6 months prior to hospitalisation because of a newly diagnosed late-onset diabetes. Due to a rapid drop in glycated haemoglobin (from over 14% to 6% in 4 months), treatment-induced neuropathy of diabetes (TIND) was hypothesised. On increasing the dose of pregabalin and adding duloxetine, the patient reported improvement of symptoms, which further underlined the suspected diagnosis. Hence, in patients with severe hyperglycaemia, changes in glycaemic control should be stepwise and not rapid; however, to date, no guidelines exist how to avoid TIND.

Needs and supporting tools for primary care physicians to improve care of patients with vertigo and dizziness: a national survey.

Background: The diagnostic workup and treatment decisions for vertigo or dizziness in primary care can be challenging due to the broad range of possible causes and limited time and expertise of physicians. This can lead to delays in treatment and unnecessary tests. We aimed to identify the unmet needs of primary care physicians (PCPs) and strategies to improve care for dizzy patients. Materials and methods: An online survey was conducted among board-certified PCPs in Switzerland to explore needs in caring for dizzy patients and potential educational approaches. Results: Based on responses from 152 participating PCPs, satisfaction and confidence were higher in diagnosing (82%) and treating (76%) acute dizziness compared to episodic/chronic cases (63 and 59%, respectively). Younger PCPs had lower diagnostic yield and confidence. Areas for improvement in specialist interactions included communication between physicians (23%/36%; always/often true), shorter waiting times for consultations (19%/40%), more detailed feedback (36%/35%), and consistent patient back referrals (31%/30%). PCPs expressed interest in hands-on courses, workshops, practical guidelines, web-based algorithms, and digital tools such as printed dizzy diaries and apps for follow-up. Conclusion: Enhanced dialog between PCPs and specialists is crucial to address the most common unmet needs. Reducing waiting times for referrals and providing clear instructions to specialists for triage are essential. The findings from this survey will guide the development of tools to improve the diagnosis and treatment of dizzy patients. Younger PCPs, who face higher diagnostic uncertainty, should be prioritized for educational approaches such as hands-on courses, workshops, and practical recommendations.

Bedside Testing in Acute Vestibular Syndrome-Evaluating HINTS Plus and Beyond-A Critical Review.

Acute vertigo and dizziness are frequent presenting symptoms in patients in the emergency department. These symptoms, which can be subtle and transient, present diagnostic challenges because they can be caused by a broad range of conditions that cut across many specialties and organ systems. Previous work has emphasized the value of combining structured history taking and a targeted examination focusing on subtle oculomotor signs. In this review, we discuss various diagnostic bedside algorithms proposed for the acutely dizzy patient. We analyzed these different approaches by calculating their area-under-the-curve (ROC) characteristics and sensitivity/specificity. We found that the algorithms that incorporated structured history taking and the use of subtle oculomotor signs had the highest diagnostic accuracy. In fact, both the HINTS+ bedside exam and the STANDING algorithm can more accurately diagnose acute strokes than early (<24 to 48 h after symptom onset) MRI with diffusion-weighted imaging (DWI). An important caveat is that HINTS and STANDING require moderate training to achieve this accuracy. Therefore, for physicians who have not undergone adequate training, other approaches are needed. These other approaches (e.g., ABCD2 score, PCI score, and TriAGe+ score) rely on vascular risk factors, clinical symptoms, and focal neurologic findings. While these other scores are easier for frontline providers to use, their diagnostic accuracy is far lower than HINTS+ or STANDING. Therefore, a focus on providing dedicated training in HINTS+ or STANDING techniques to frontline clinicians will be key to improving diagnostic accuracy and avoiding unnecessary brain imaging.

What is the current status of primary care in the diagnosis and treatment of patients with vertigo and dizziness in Switzerland? A national survey.

Background: Vertigo and dizziness are among the most frequent presenting symptoms in the primary care physicians' (PCPs) office. With patients facing difficulties in describing their complaints and clinical findings often being subtle and transient, the diagnostic workup of the dizzy patient remains challenging. We aimed to gain more insights into the current state of practice in order to identify the limitations and needs of the PCPs and define strategies to continuously improve their knowledge in the care of the dizzy patient. Materials and methods: Board-certified PCPs working in Switzerland were invited to participate in an online survey. A descriptive statistical analysis was performed, and prospectively defined hypotheses were assessed using regression analyses. Results: A vast majority of participating PCPs (n = 152) were familiar with the key questions when taking the dizzy patient's history and with performing provocation/repositioning maneuvers when posterior-canal benign paroxysmal positional vertigo (BPPV) was suspected (91%). In contrast, strong agreement that performing the alternating cover test (21%), looking for a spontaneous nystagmus with fixation removed (42%), and performing the head-impulse test (47%) were important was considerably lower, and only 19% of PCPs were familiar with lateral-canal BPPV treatment. No specific diagnosis could be reached in substantial fractions of patients with acute (35% [25; 50%], median [inter-quartile range]) and episodic/chronic (50% [40; 65.8%]) dizziness/vertigo. Referral to specialists was higher in patients with episodic/chronic dizziness than in acutely dizzy patients (50% [20.3; 75] vs. 30% [20; 50]), with younger PCPs (aged 30-40 years) demonstrating significantly increased odds of referral to specialists (odds ratio = 2.20 [1.01-4.81], p = 0.048). Conclusion: The assessment of dizzy patients takes longer than that of average patients in most primary care practices. Many dizzy patients remain undiagnosed even after a thorough examination, highlighting the challenges faced by PCPs and potentially leading to frequent referrals to specialists. To address this, it is crucial to promote state-of-the-art neuro-otological examination and treatment techniques that are currently neglected by most PCPs, such as "HINTS" and lateral-canal BPPV treatment. This can help reduce referral rates allowing more targeted treatment and referrals.

[Diagnosis and treatment of benign paroxysmal positional vertigo with manual and motorized chairs - Indication and value]. / Abklärung und Behandlung des benignen paroxysmalen Lagerungsschwindels mittels manueller und motorisierter Drehstühle.

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. The use of a manual or motorized turntable thus extends both the diagnostic and therapeutic options in BPPV. Thus, it enables patients with disabilities that do not tolerate classic maneuvers on the examination couch access to the required diagnostic procedures and - if needed - provides targeted treatment as well. Furthermore, turntables extend both the diagnostic and the therapeutic spectrum, offering a broader range of repositioning maneuvers in unclear or treatment-refractory cases. In this narrative review potential indications are described and evidence for using turntables in the diagnosis and treatment of BPPV on the turntable is discussed.

Ischaemic stroke despite antiplatelet therapy: Causes and outcomes.

BACKGROUND: Ischaemic stroke may occur despite antiplatelet therapy (APT). We aimed to investigate frequency, potential causes and outcomes in patients with ischaemic stroke despite APT. METHODS: In this cohort study, we enrolled patients with imaging-confirmed ischaemic stroke from the Swiss Stroke Registry (01/2014-07/2022). We determined the frequency of prior APT, assessed stroke aetiology (modified TOAST classification) and determined the association of prior APT with unfavourable functional outcome (modified Rankin Scale score 3-6) and recurrent ischaemic stroke at 3 months using regression models. RESULTS: Among 53,352 patients, 27,484 (51.5%) had no prior antithrombotic treatment, 17,760 (33.3%) were on APT, 7039 (13.2%) on anticoagulation and 1069 (2.0%) were on APT + anticoagulation. In patients with a history of ischaemic stroke/TIA (n = 11,948; 22.4%), 2401 (20.1%) had no prior antithrombotic therapy, 6594 (55.2%) were on APT, 2489 (20.8%) on anticoagulation and 464 (3.9%) on APT + anticoagulation. Amongst patients with ischaemic stroke despite APT, aetiology was large artery atherosclerosis in 19.8% (n = 3416), cardiac embolism in 23.6% (n = 4059), small vessel disease in 11.7% (n = 2011), other causes in 7.4% (n = 1267), more than one cause in 6.3% (n = 1078) and unknown cause in 31.3% (n = 5388). Prior APT was not independently associated with unfavourable outcome (aOR = 1.06; 95% CI: 0.98-1.14; p = 0.135) or death (aOR = 1.10; 95% CI: 0.99-1.21; p = 0.059) at 3-months but with increased odds of recurrent stroke (6.0% vs 4.3%; aOR 1.26; 95% CI: 1.11-1.44; p < 0.001). CONCLUSIONS: One-third of ischaemic strokes occurred despite APT and 20% of patients with a history of ischaemic stroke had no antithrombotic therapy when having stroke recurrence. Aetiology of breakthrough strokes despite APT is heterogeneous and these patients are at increased risk of recurrent stroke.

Systematic review and meta-analysis of the diagnostic accuracy of spontaneous nystagmus patterns in acute vestibular syndrome.

Objectives: For the assessment of patients presenting with acute prolonged vertigo meeting diagnostic criteria for acute vestibular syndrome (AVS), bedside oculomotor examinations are essential to distinguish peripheral from central causes. Here we assessed patterns of spontaneous nystagmus (SN) observed in AVS and its diagnostic accuracy at the bedside. Methods: MEDLINE and Embase were searched for studies (1980-2022) reporting on the bedside diagnostic accuracy of SN-patterns in AVS patients. Two independent reviewers determined inclusion. We identified 4,186 unique citations, examined 219 full manuscripts, and analyzed 39 studies. Studies were rated on risk of bias (QUADAS-2). Diagnostic data were extracted and SN beating-direction patterns were correlated with lesion locations and lateralization. Results: Included studies reported on 1,599 patients, with ischemic strokes (n = 747) and acute unilateral vestibulopathy (n = 743) being most frequent. While a horizontal or horizontal-torsional SN was significantly more often found in peripheral AVS (pAVS) than in central AVS (cAVS) patients (672/709 [94.8%] vs. 294/677 [43.4%], p < 0.001), torsional and/or vertical SN-patterns were more prevalent in cAVS than in pAVS (15.1 vs. 2.6%, p < 0.001). For an (isolated) vertical/vertical-torsional SN or an isolated torsional SN specificity (97.7% [95% CI = 95.1-100.0%]) for a central origin etiology was high, whereas sensitivity (19.1% [10.5-27.7%]) was low. Absence of any horizontal SN was more frequently observed in cAVS than in pAVS (55.2 vs. 7.0%, p < 0.001). Ipsilesional and contralesional beating directions of horizontal SN in cAVS were found at similar frequency (28.0 vs. 21.7%, p = 0.052), whereas for pAVS a contralesional SN was significantly more frequent (95.2 vs. 2.5%, p < 0.001). For PICA strokes presenting with horizontal SN, beating direction was ipsilesional more often than contralesional (23.9 vs. 6.4%, p = 0.006), while the opposite was observed for AICA strokes (2.2 vs. 63.0%, p < 0.001). Conclusions: (Isolated) vertical and/or torsional SN is found in a minority (15.1%) of cAVS patients only. When present, it is highly predictive for a central cause. A combined torsional-downbeating SN-pattern may be observed in pAVS also in cases with isolated lesions of the inferior branch of the vestibular nerve. Furthermore, in cAVS patients the SN beating direction itself does not allow a prediction on the lesion side.

Triggered episodic vestibular syndrome and transient loss of consciousness due to a retrostyloidal vagal schwannoma: a case report.

Background: Various conditions may trigger episodic vertigo or dizziness, with positional changes being the most frequently identified condition. In this study, we describe a rare case of triggered episodic vestibular syndrome (EVS) accompanied by transient loss of consciousness (TLOC) linked to retrostyloidal vagal schwannoma. Case description: A 27-year woman with known vestibular migraine presented with a 19-month history of nausea, dysphagia, and odynophagia triggered by swallowing food and followed by recurrent TLOC. These symptoms occurred independently of her body position, resulting in a weight loss of 10 kg within 1 year and in an inability to work. An extensive cardiologic diagnostic work-up undertaken before she presented to the neurologic department was normal. On the fiberoptic endoscopic evaluation of swallowing, she showed a decreased sensitivity, a slight bulging of the right lateral pharyngeal wall, and a pathological pharyngeal squeeze maneuver without any further functional deficits. Quantitative vestibular testing revealed an intact peripheral-vestibular function, and electroencephalography was read as normal. On the brain MRI, a 16 x 15 x 12 mm lesion in the right retrostyloidal space suspicious of a vagal schwannoma was detected. Radiosurgery was preferred over surgical resection, as resection of tumors in the retrostyloid space bears the risk of intraoperative complications and may result in significant morbidity. A single radiosurgical procedure (stereotactic CyberKnife radiosurgery, 1 x 13Gy) accompanied by oral steroids was performed. On follow-up, a cessation of (pre)syncopes was noted 6 months after treatment. Only residual infrequent episodes of mild nausea were triggered by swallowing solid food remained. Brain MRI after 6 months demonstrated no progression of the lesion. In contrast, migraine headaches associated with dizziness remained frequent. Discussion: Distinguishing triggered and spontaneous EVS is important, and identifying specific triggers by structured history-taking is essential. Episodes being elicited by swallowing solid foods and accompanied by (near) TLOC should initiate a thorough search for vagal schwannoma, as symptoms are often disabling, and targeted treatment is available. In the case presented here, cessation of (pre)syncopes and significant reduction of nausea triggered by swallowing was noted with a 6-month delay, illustrating the advantages (no surgical complications) and disadvantages (delayed treatment response) of first-line radiotherapy in vagal schwannoma treatment.

Test-re-test reliability and dynamics of the Fukuda-Unterberger stepping test.

Background: The Fukuda-stepping-test (FST), i.e., repetitive walking on the spot while blindfolded, has been proposed as a means to assess the integrity of the vestibular pathways. While its sensitivity to detect abnormalities in patients is limited, it may be useful in studying the physiology of the subjective-straight-ahead (SSA). Considering reported systematic shifts in SSA in humans, we hypothesize that such asymmetries arise from individual differences in the orientation/configuration of the macular organs and in central processing of vestibular input. We hypothesize that such asymmetries are stable over time in individual subjects. Alternatively, such asymmetries may arise from random noise in the sensory/motor systems involved, demonstrating low reproducibility over time. Materials and methods: Twenty-four subjects walked on the spot over 60 s while blindfolded (n = 6 trials per subject). Using an inertial measurement unit (IMU) placed at the chest, angular deviations were recorded and compared to manually-measured final positions. Both static (direction, magnitude) and dynamic (time-to-onset of deviation, pattern of deviations) parameters were retrieved from the yaw slopes. Results: Significant deviations were found in 15/24 participants for the manual measurements (leftwards = 8; rightwards = 7), whereas when using the IMU-sensor 13/24 participants showed significant shifts (leftwards = 9; rightwards = 4). There was a high correlation (0.98) between manually measured rotation angles (average absolute deviations = 58.0 deg ± 48.6 deg; intra-individual variability = 39 deg ± 24 deg) and sensor-based yaw slopes (1.00 deg/s ± 0.88 deg/s; 0.67 deg/s ± 0.41 deg/s). Relevant yaw deviation was detected 22.1 s ± 12.3 s (range = 5.6 s-59.2 s) after the onset of marching (no relevant yaw-deviation in 15/139 measurements), showing a mostly linear behavior over time. Conclusion: We observed significant inter-individual variability in task performance in the FST, reproducing findings from previous studies. With test-re-test reliability being moderate only, but at the same time observing a preference in the side of shifts in most trials and subjects, we conclude that likely both individually varying estimates of straight-ahead and random noise contribute to the pattern of angular deviations observed. Using an IMU-sensory based approach, additional dynamic parameters could be retrieved, emphasizing the value of such a quantitative approach over manual measurements. Such an approach may provide useful additional information to distinguish patients from healthy controls.

Impact of Clinician Training Background and Stroke Location on Bedside Diagnostic Test Accuracy in the Acute Vestibular Syndrome - A Meta-Analysis.

OBJECTIVE: Acute dizziness/vertigo is usually due to benign inner-ear causes but is occasionally due to dangerous neurologic ones, particularly stroke. Because symptoms and signs overlap, misdiagnosis is frequent and overuse of neuroimaging is common. We assessed the accuracy of bedside findings to differentiate peripheral vestibular from central neurologic causes. METHODS: We performed a systematic search (MEDLINE and Embase) to identify studies reporting on diagnostic accuracy of physical examination in adults with acute, prolonged dizziness/vertigo ("acute vestibular syndrome" [AVS]). Diagnostic test properties were calculated for findings. Results were stratified by examiner type and stroke location. RESULTS: We identified 6,089 citations and included 14 articles representing 10 study cohorts (n = 800). The Head Impulse, Nystagmus, Test of Skew (HINTS) eye movement battery had high sensitivity 95.3% (95% confidence interval [CI] = 92.5-98.1) and specificity 92.6% (95% CI = 88.6-96.5). Sensitivity was similar by examiner type (subspecialists 94.3% [95% CI = 88.2-100.0] vs non-subspecialists 95.0% [95% CI = 91.2-98.9], p = 0.55), but specificity was higher among subspecialists (97.6% [95% CI = 94.9-100.0] vs 89.1% [95% CI = 83.0-95.2], p = 0.007). HINTS sensitivity was lower in anterior cerebellar artery (AICA) than posterior inferior cerebellar artery (PICA) strokes (84.0% [95% CI = 65.3-93.6] vs 97.7% [95% CI = 93.3-99.2], p = 0.014) but was "rescued" by the addition of bedside hearing tests (HINTS+). Severe (grade 3) gait/truncal instability had high specificity 99.2% (95% CI = 97.8-100.0) but low sensitivity 35.8% (95% CI = 5.2-66.5). Early magnetic resonance imaging (MRI)-diffusion-weighted imaging (DWI; within 24-48 hours) was falsely negative in 15% of strokes (sensitivity 85.1% [95% CI = 79.2-91.0]). INTERPRETATION: In AVS, HINTS examination by appropriately trained clinicians can differentiate peripheral from central causes and has higher diagnostic accuracy for stroke than MRI-DWI in the first 24-48 hours. These techniques should be disseminated to all clinicians evaluating dizziness/vertigo. ANN NEUROL 2023;94:295-308.

Quantitative Oculomotor Assessment in Hereditary Ataxia: Systematic Review and Consensus by the Ataxia Global Initiative Working Group on Digital-motor Biomarkers.

Oculomotor deficits are common in hereditary ataxia, but disproportionally neglected in clinical ataxia scales and as outcome measures for interventional trials. Quantitative assessment of oculomotor function has become increasingly available and thus applicable in multicenter trials and offers the opportunity to capture severity and progression of oculomotor impairment in a sensitive and reliable manner. In this consensus paper of the Ataxia Global Initiative Working Group On Digital Oculomotor Biomarkers, based on a systematic literature review, we propose harmonized methodology and measurement parameters for the quantitative assessment of oculomotor function in natural-history studies and clinical trials in hereditary ataxia. MEDLINE was searched for articles reporting on oculomotor/vestibular properties in ataxia patients and a study-tailored quality-assessment was performed. One-hundred-and-seventeen articles reporting on subjects with genetically confirmed (n=1134) or suspected hereditary ataxia (n=198), and degenerative ataxias with sporadic presentation (n=480) were included and subject to data extraction. Based on robust discrimination from controls, correlation with disease-severity, sensitivity to change, and feasibility in international multicenter settings as prerequisite for clinical trials, we prioritize a core-set of five eye-movement types: (i) pursuit eye movements, (ii) saccadic eye movements, (iii) fixation, (iv) eccentric gaze holding, and (v) rotational vestibulo-ocular reflex. We provide detailed guidelines for their acquisition, and recommendations on the quantitative parameters to extract. Limitations include low study quality, heterogeneity in patient populations, and lack of longitudinal studies. Standardization of quantitative oculomotor assessments will facilitate their implementation, interpretation, and validation in clinical trials, and ultimately advance our understanding of the evolution of oculomotor network dysfunction in hereditary ataxias.

Video head impulse testing in patients with isolated (hemi)nodular infarction.

Background: Isolated (hemi)nodular strokes as underlying cause of acute dizziness are rare, thus there are still gaps of knowledge in the clinical presentation of affected patients. Clinical and experimental evidence has suggested that lesions involving the nodulus lead to various vestibulo-ocular deficits including prolonged velocity-storage, periodic-alternating nystagmus, positional nystagmus, abolished suppression of post-rotatory nystagmus by head-tilt and impaired verticality perception. At the bedside, the angular vestibulo-ocular reflex (aVOR), as assessed by the horizontal head-impulse test (HIT), has been reported to be normal, however quantitative assessments of all six semicircular canals are lacking. Objective: The primary aim of this case series was to characterize the spectrum of clinical presentations in isolated (hemi)nodular strokes. Furthermore, based on preliminary observations, we hypothesized that the aVOR is within normal limits in isolated nodular strokes. Methods: We retrospectively included patients with isolated (hemi)nodular stroke on diffusion-weighted MR-imaging from a prospective stroke-registry. All patients received a standardized bedside neuro-otological assessment and quantitative, video-based HIT (vHIT) of all six semicircular canals. Overall ratings of vHIT (normal vs. abnormal function) were performed independently by two reviewers and disagreements were resolved. Results: Between January 2015 and December 2021 six patients with isolated nodular (n = 1) or heminodular (n = 5) ischemic stroke were included. Clinical presentation met diagnostic criteria for acute vestibular syndrome (AVS) in 5/6 patients and for episodic vestibular syndrome (EVS) in 1/6 patients. Ocular motor abnormalities observed included the presence of spontaneous horizontal nystagmus (n = 2), positional nystagmus (5/6), head-shaking nystagmus (3/6), skew deviation (n = 1), and moderate or severe truncal ataxia (5/6). Bedside HIT was normal in all patients and no gaze-evoked or periodic alternating nystagmus was observed. aVOR-gains were within normal range in all patients and overall aVOR-function as assessed by vHIT was rated as normal in all six patients. Conclusions: Using quantitative, video-based testing of the horizontal and vertical aVOR, preserved integrity of the aVOR in (hemi)nodular strokes was confirmed, extending preliminary findings at the bedside. Furthermore, widespread deficits of both ocular stability, postural control and volitional eye movements were observed in our study cohort, being consistent with findings reported in previous studies.