Do monosymptomatic stroke patients with dizziness present a vestibular syndrome without nystagmus? An underestimated entity.

BACKGROUND AND PURPOSE: Vestibular symptoms are common in emergency department (ED) patients and have various causes, including stroke. Accurate identification of stroke in patients with vestibular symptoms is crucial for timely management. We conducted a prospective cross-sectional study from 2015 to 2019 to determine stroke prevalence and associated symptoms in ED patients with vestibular symptoms, aiming to improve diagnosis and outcomes. METHODS: As part of the DETECT project, we screened 1647 ED patients with acute vestibular symptoms. Following a retrospective analysis of 961 head and neck magnetic resonance imaging (MRI) scans, we included 122 confirmed stroke cases and assessed them for vestibular signs and symptoms. RESULTS: Stroke prevalence in dizzy patients was 13% (122/961 MRI scans). Most patients (95%) presented with acute vestibular symptoms with or without nystagmus, whereas 5% had episodic vestibular syndrome (EVS). Nystagmus was present in 50% of stroke patients. Eighty percent had a purely posterior circulation stroke, and nystagmus was absent in 46% of these patients. Seven patients (6%) had lesions in both the anterior and posterior circulation. Vertigo was experienced by 52% regardless of territory. CONCLUSIONS: A stroke was identified in 13% of ED patients presenting with acute vestibular symptoms. In 5%, it was EVS. Most strokes were in the posterior circulation territory; vertigo occurred with similar frequency in anterior and posterior circulation stroke, and absence of nystagmus was common in both.

Corrective saccades in acute vestibular neuritis: studying the role of prediction with automated passively induced head impulses.

When the demands for visual stabilization during head rotations overwhelm the ability of the vestibuloocular reflex (VOR) to produce compensatory eye movements, the brain produces corrective saccades that bring gaze toward the fixation target, even without visual cues (covert saccades). What triggers covert saccades and what might be the role of prediction in their generation are unknown. We studied 14 subjects with acute vestibular neuritis. To minimize variability of the stimulus, head impulses were imposed with a motorized torque generator with the subject on a bite bar. Predictable and unpredictable (timing, amplitude, direction) stimuli were compared. Distributions of covert corrective saccade latencies were analyzed with a "LATER" (linear approach to threshold with ergodic rate) approach. On the affected side, VOR gain was higher (0.47 ± 0.28 vs. 0.39 ± 0.22, P ≪ 0.001) with predictable than unpredictable head impulses, and gaze error at the end of the head movement was less (5.4 ± 3.3° vs. 6.9 ± 3.3°, P ≪ 0.001). Analyzing trials with covert saccades, gaze error at saccade end was significantly less with predictable than unpredictable head impulses (4.2 ± 2.8° vs. 5.5 ± 3.2°, P ≪ 0.001). Furthermore, covert corrective saccades occurred earlier with predictable than unpredictable head impulses (140 ± 37 vs. 153 ± 37 ms, P ≪ 0.001). Using a LATER analysis with reciprobit plots, we were able to divide covert corrective saccades into two classes, early and late, with a break point in the range of 88-98 ms. We hypothesized two rise-to-threshold decision mechanisms for triggering early and late covert corrective saccades, with the first being most engaged when stimuli are predictable.NEW & NOTEWORTHY We successfully used a LATER (linear approach to threshold with ergodic rate) analysis of the latencies of corrective saccades in patients with acute vestibular neuritis. We found two types of covert saccades: early (<90 ms) and late (>90 ms) covert saccades. Predictability led to an increase in VOR gain and a decrease in saccade latency.

Automated alternate cover test for 'HINTS' assessment: a validation study.

OBJECTIVE: The alternate cover test (ACT) in patients with acute vestibular syndrome is part of the 'HINTS' battery test. Although quantitative, the ACT is highly dependent on the examiner's experience and could theoretically vary greatly between examiners. In this study, we sought to validate an automated video-oculography (VOG) system based on eye tracking and dedicated glasses. METHODS: We artificially induced a vertical strabismus to simulate a skew deviation on ten healthy subjects, aged from 26 to 66, using different press-on Fresnel prisms on one eye while recording eye position with VOG of the contralateral eye. We then compared the system's performance to that of a blinded trained orthoptist using conventional, semi-quantitative method of skew measurement known as the alternate prism cover test (APCT) as a gold standard. RESULTS: We found a significant correlation between the reference APCT and the Skew VOG (Pearson's R2 = 0.606, p < 0.05). There was a good agreement between the two tests (intraclass correlation coefficient 0.852, 95 CI 0.728-0.917, p < 0.001). The overall accuracy of the VOG was estimated at 80.53% with an error rate of 19.46%. There was no significant difference in VOG skew estimations compared with the gold standard except for very small skews. CONCLUSIONS: VOG offers an objective and quantitative skew measurement and proved to be accurate in measuring vertical eye misalignment compared to the ACT with prisms. Precision was moderate, which mandates a sufficient number of tests per subject.

Minimal Out-Toeing and Good Hip Scores of Severe SCFE Patients Treated With Modified Dunn Procedure and Contralateral Prophylactic Pinning at Minimal 5-year Follow up.

BACKGROUND: Slipped capital femoral epiphyses (SCFE) is associated with out-toeing of the foot and external rotation gait. But it is unknown if SCFE patients treated with the modified Dunn procedure have out-toeing at follow up.Therefore, we used instrumented gait analysis and questioned (1) do severe SCFE patients treated with a modified Dunn procedure have symmetrical foot progression angle (FPA) compared with contralateral side and compared with asymptomatic volunteers (2) what is the prevalence of out-toeing gait and what are the outcome socres at follow up. METHODS: Gait analysis of 22 patients (22 hips) treated with an unilateral modified Dunn procedure for severe SCFE (slip angle >60 degrees, 2002 to 2011) was retrospectively evaluated. Of 38 patients with minimal 5-year follow up, 2 hips (4%) had avascular necrosis of the femoral head and were excluded for gait analysis. Twenty-two patients were available for gait analysis at follow up (mean follow up of 9±2 y). Mean age at follow up was 22±3 years. Mean preoperative slip angle was 64±8 degrees (33% unstable slips) and decreased postoperatively (slip angle of 8±4 degrees). Gait analysis was performed with computer-based instrumented walkway system (GAITRite) to measure FPA with embedded pressure sensors. Patients were compared with control group of 18 healthy asymptomatic volunteers (36 feet, mean age 29±6 y). RESULTS: (1) Mean FPA of SCFE patients (3.6±6.4 degrees) at follow up was not significantly different compared with their contralateral side (5.6±5.5 degrees) and compared with FPA of controls (4.0±4.5 degrees). (2) Of the 22 SCFE patients, most of them (19 hips, 86%) had normal FPA (-5 to 15 degrees), 2 patients had in-toeing (FPA<-5 degrees) and 1 had out-toeing (FPA >15 degrees) and was not significantly different compared with control group. (3) Mean modified Harris hip score (mHHS) was 93±11 points, mean Hip Disability and Osteoarthritis Outcome Score (HOOS) score was 91±10 points. Three patients (14%) had mHHS <80 points and walked with normal FPA. The 2 patients with in-toeing and one patient with out-toeing had mHHS >95 points. CONCLUSIONS: Patients with severe SCFE treated with modified Dunn procedure had mostly symmetrical FPA and good hip scores at long term follow up. This is in contrast to previous studies. Although 1 patient had out-toeing and 2 patients had in-toeing at follow up, they had good hip scores. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Incidence and Outcome of Aneurysmal Subarachnoid Hemorrhage: The Swiss Study on Subarachnoid Hemorrhage (Swiss SOS).

BACKGROUND AND PURPOSE: The purpose of this study was to assess nationwide incidence and outcomes of aneurysmal subarachnoid hemorrhage (aSAH). The Swiss SOS (Swiss Study on Subarachnoid Hemorrhage) was established in 2008 and offers the unique opportunity to provide this data from the point of care on a nationwide level. METHODS: All patients with confirmed aneurysmal subarachnoid hemorrhage admitted between January 1, 2009 and December 31, 2014, within Switzerland were recorded in a prospective registry. Incidence rates were calculated based on time-matched population data. Admission parameters and outcomes at discharge and at 1 year were recorded. RESULTS: We recorded data of 1787 consecutive patients. The incidence of aneurysmal subarachnoid hemorrhage in Switzerland was 3.7 per 100 000 persons/y. The number of female patients was 1170 (65.5%). With a follow-up rate of 91.3% at 1 year, 1042 patients (58.8%) led an independent life according to the modified Rankin Scale (0-2). About 1 in 10 patients survived in a dependent state (modified Rankin Scale, 3-5; n=185; 10.4%). Case fatality was 20.1% (n=356) at discharge and 22.1% (n=391) after 1 year. CONCLUSIONS: The current incidence of aneurysmal subarachnoid hemorrhage in Switzerland is lower than expected and an indication of a global trend toward decreasing admissions for ruptured intracranial aneurysms. Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT03245866.

Proximal arterial diameters on CT angiography and digital subtraction angiography correlate both at admission and in the vasospasm period after aneurysmal subarachnoid hemorrhage.

BACKGROUND: Comparison of artery diameters between CT angiography (CTA) and subtraction arteriography (DSA) has the limitation that measurements on DSA are provided as relative units, making a quantitative comparison difficult. On CTA, artery diameters may depend on windowing settings and may lead to false measurements. This study assesses the correlation between CTA and DSA based on measurements in a basic imaging viewer using normalized DSA values, and assesses whether the validity is time dependent. METHODS: Patients with aneurysmal subarachnoid hemorrhage (aSAH) were included if they underwent both CTA and DSA within 24 h. The analysis was performed using the basic imaging application Centricity Enterprise PACS viewer (GE Healthcare). A total of 15 arterial locations were assessed on CTA and DSA and a specific measurement protocol with normalization of all artery diameters to the cavernous segment of the internal carotid artery was used. Pearson correlation analysis was calculated to access the correlation of normalized arterial diameters measured with both methods at admission and at clinical onset of CVS. RESULTS: A total of 627 arteries in 38 patients were analyzed in both CTA and DSA. There was a significant correlation coefficient (R = 0.706) of artery diameters between CTA and DSA measures (p < 0.0001). This correlation remained high when comparing CTA and DSA at admission (correlation coefficient: 0.641; p < 0.0001) vs. in the vasospasm period (0.835; p < 0.0001). The correlation was good in all proximal artery segments and lost significance only when distal vessel segments were considered. CONCLUSION: Using basic imaging viewers, mostly accessible for clinicians, CTA is a noninvasive and reliable method to assess proximal arterial diameters of the brain in the management of cerebral vasospasm in the acute phase after aSAH. Significance is reached, independent of whether CTA is obtained in the acute phase or during the period of vasospasm, by normalization of basal cerebral artery diameters to a non-variable anatomic landmark, i.e., the petrous or cavernous internal carotid artery diameter.

Acute angiographic vasospasm and the incidence of delayed cerebral vasospasm: preliminary results.

BACKGROUND: More than half of subarachnoid hemorrhage (SAH) patients develop angiographically detectable delayed cerebral vasospasm (dCVS). It mostly occurs between days 4 and 15 after ictus and can be associated with neurological deficits that contribute to increased morbidity and mortality after SAH. Although dCVS is well studied, there are only a handful of reports on the acute phase of vasospasm (APV) occurring after treatment of intracranial aneurysms, whether ruptured or not. The aim of the current study is to elucidate the association of intraoperative cerebral vasospasm (iCVS) with the incidence of dCVS. METHOD: We retrospectively reviewed consecutive patients who were treated for aneurysmal SAH or incidental aneurysms during the study period. Angiograms of patients undergoing aneurysm treatment were reviewed. Spasm severity was classified with respect to reduction in the transverse diameter. Mild vasospasm was defined as a reduction in vessel diameter of 10-30 %; moderate, 30-50 %; and severe vasospasm, >50 %. Statistical significance was tested using the Χ² test with p < 0.05. Correlations between iCVS and other factors were investigated. RESULTS: Of 109 patients, 77 patients (33 men and 44 women) presented with acute SAH and 32 patients (9 men and 23 women) were treated for incidental aneurysms. Seventeen (22 %) of 77 patients presenting with acute SAH had evidence of acute (within 72 h after SAH ictus) CVS. In 16 of 17 (94.1 %) patients, this vasospasm was observed immediately after treatment and was therefore termed iCVS. Eleven (30 %) of 36 patients undergoing clipping and 5 (14 %) of 36 patients with endovascular aneurysm occlusion had iCVS (p = 0.07). Patients presenting with acute SAH had a higher incidence of iCVS than patients undergoing elective aneurysm treatment (p = 0.02). Only one patient (3 %) had iCVS in the elective treatment group whereas 16 (20 %) had iCVS after SAH. The incidence of dCVS, delayed ischemic neurological deficits (DNDs), and poor outcome in patients presenting with iCVS during surgical treatment of ruptured aneurysms was 56 % (p = 0.001), 63 % (p = 0.02), and 38 % (p = 0.14), respectively. CONCLUSION: APV exists and is a common finding in patients with SAH. Further studies are warranted to correlate the presence of APV with postoperative ischemia, dCVS, and outcome.

Unilateral corneal arcus and conjunctival vessel alterations in cranial autonomic dysregulation: A case report.

BACKGROUND: Cranial autonomic dysregulation is a common symptom of patients suffering from cluster headache or migraine. The peripheral vascular dysfunction may increase the risk for ischemic or hemorrhagic strokes, myocardial infarction, retinal vasculopathy, cardiovascular mortality, and peripheral artery diseases. Furthermore, it may also manifest with ocular symptoms, e.g., increased lacrimation, conjunctival injection, and facial swelling. CASE PRESENTATION: We here report a case of a patient with migraine and ocular signs of a vascular dysregulation that have led to persisting changes of conjunctival vessels and to a corneal arcus. CONCLUSIONS: Autonomic vascular dysregulation may not only cause headaches but also persisting changes of ocular tissues, e.g., conjunctival vessel alterations and a corneal arcus.

The Behavioral Mapping of Psychomotor Slowing in Psychosis Demonstrates Heterogeneity Among Patients Suggesting Distinct Pathobiology.

OBJECTIVES: Psychomotor slowing (PS) occurs in up to half of schizophrenia patients and is linked to poorer outcomes. As standard treatment fails to improve PS, novel approaches are needed. Here, we applied the RDoC framework using 3 units of analysis, ie, behavior, self-report, and physiology to test, whether patients with PS are different from patients without PS and controls. METHODS: Motor behavior was compared between 71 schizophrenia patients with PS, 25 without PS, and 42 healthy controls (HC) using 5 different measures: (1) for behavior, an expert rating scale: Motor score of the Salpêtrière Retardation Rating Scale, (2) for self-report, the International Physical Activity Questionnaire; and for physiology, (3) Actigraphy, which accounts for gross motor behavior, (4) Gait velocity, and (5) coin rotation task to assess manual dexterity. RESULTS: The ANCOVAs comparing the 3 groups revealed differences between patients with PS and HC in expert ratings, self-report, and instrumental measures (all P ≤ .001). Patients with PS also scored higher in expert ratings and had lower instrumental activity levels compared to patients without PS (all P ≤ .045). Instrumental activity levels correlated with an expert rating of PS (rho = -0.51, P-fdr corrected <.001) and classified similarly at 72% accuracy. CONCLUSIONS: PS is characterized by slower gait, lower activity levels, and slower finger movements compared to HC. However, only actigraphy and observer ratings enable to clearly disentangle PS from non-PS patients. Actigraphy may become the standard assessment of PS in neuroimaging studies and clinical trials.

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.

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.

What neuro-otology specialists need for better care of dizzy patients: a national survey.

Background: A substantial fraction of dizzy patients are assessed by neurologists and ear-nose-throat (ENT) physicians. With the differential diagnosis being broad and often different specialties involved, we aimed to assess the interaction with generalists from the specialists' perspective to identify limitations and needs and to define strategies for improvement in patient care and education by the specialist. Methods: One hundred eleven board-certified neurologists (n = 62) and ENT physicians (n = 49) working in Switzerland participated in an online survey. Here, we focused on limitations faced in the diagnostic workup and treatment of the dizzy patient and potential strategies to improve the standard of care and the interaction between generalists and specialists. Descriptive statistical analyses were performed. We hypothesized that those specialists applying modern concepts in history-taking and bedside examination techniques reach a specific diagnosis more often and request fewer referrals. Results: Specialists indicated higher confidence in reaching a specific diagnosis for patients presenting with acute dizziness than episodic/chronic dizziness (80% vs. 60%) at the first consultation. Knowledge of the timing-and-trigger concept [odds ratio (OR) = 0.81 (0.67-0.98), p = 0.034], as well as of subtle oculomotor/vestibular signs [OR = 0.80 (0.68-0.94), p = 0.007] was predictive of the self-reported probability of reaching a specific diagnosis in patients with episodic/chronic dizziness, while no such differences were observed in the care of acutely dizzy patients. Further referrals of acutely dizzy patients were significantly higher in neurologists than in ENT physicians (17% vs. 10%, p < 0.001) and in specialists located in the Latin part of Switzerland [OR = 2.84 (1.63-4.93), p < 0.001], while this was not the case for patients with episodic/chronic dizziness. Identified unmet needs included regular communication between physicians (27%/53%; always/often true) and sufficiently detailed information on the previous workup from the referrals (27%/53%). Specialists expressed most interest in hands-on courses/workshops, webinars, and practical guidelines for education. Conclusion: In our survey, bedside state-of-the-art assessments were key in reducing the fraction of unclear dizzy cases. Several gaps were identified that should be addressed. Specifically, referring physicians should provide more comprehensive details regarding urgency, prior diagnostics, and treatment. Specifically, when promoting the knowledge of neurologists and ENT physicians, this should be preferentially done by offering a combination of hands-on courses and webinars.

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.

Pharmacological and Behavioral Strategies to Improve Vision in Acquired Pendular Nystagmus.

BACKGROUND Acquired pendular nystagmus (APN) is a back and forth, oscillatory eye movement in which the 2 oppositely directed slow phases have similar waveforms. APN occurs commonly in multiple sclerosis and causes a disabling oscillopsia that impairs vision. Previous studies have proven that symptomatic therapy with gabapentin or memantine can reduce the nystagmus amplitude or frequency. However, the effect of these medications on visual acuity (VA) is less known and to our knowledge the impact of non-pharmacological strategies such as blinking on VA has not been reported. This is a single observational study without controls (Class IV) and is meant to suggest a future strategy for study of vision in patients with disabling nystagmus and impaired vision. CASE REPORT A 49-year-old woman with primary progressive multiple sclerosis with spastic paraparesis and a history of optic atrophy presented with asymmetrical binocular APN and bothersome oscillopsia. We found that in the eye with greater APN her visual acuity improved by 1 line (from 0.063 to 0.08 decimals) immediately after blinking. During treatment with memantine, her VA without blinking increased by 2 lines, from 0.063 to 0.12, but improved even more (from 0.12 to 0.16) after blinking. In the contralateral eye with a barely visible nystagmus, VA was reduced by 1 line briefly (~500 ms) after blinking. CONCLUSIONS In a patient with APN, blinking transiently improved vision. The combination of pharmacological treatment with memantine and the blinking strategy may induce better VA and less oscillopsia than either alone.

Effects of Acetyl-DL-Leucine on Ataxia and Downbeat-Nystagmus in Six Patients With Ataxia Telangiectasia.

BACKGROUND: There is no authorized treatment for ataxia telangiectasia (AT). As cerebellar symptoms of storage diseases were improved by acetyl-DL-leucine (ADLL), the authors hypothesized a symptomatic and disease-modifying effect in AT upon supplementation with ADLL. METHODS: Six patients were treated with ADLL 3 g/day for 1 week followed by 5g/day for 3 weeks to 1 year. Cerebellar ataxia was evaluated by validated scales. Gaze-holding, saccades and smooth pursuit were examined by video-oculography. Measurements took place at baseline, at 1 month of therapy in 5 patients, and after 6 and 12 months in 1 patient. RESULTS: The Scale for Assessment and Rating of Ataxia changed from the baseline, mean, (SD, min-max) of 22.1 (5.88, 11-28.5) to 18 points (5.39, 8.5-23.5) after 1 month on medication (P = .0028). All patients demonstrated gaze-holding deficits; 3 patients had central-position downbeat-nystagmus. Mean slow-phase velocity of this nystagmus with the gaze straight-ahead changed from 5.57°/s (1.8, 3.53-6.99) to 4.7°/s (0.79, 3.97-5.56) after 1 month on treatment (1.35, -2.56-4.17) (P = .046). INTERPRETATION: ADLL may improve ataxia and ocular stability in AT patients, while the molecular basis still remains to be elucidated. A multicentric, rater-blinded, phase II trial currently investigates the effects of acetyl-L-leucine in AT (NCT03759678).

Psychomotor slowing alters gait velocity, cadence, and stride length and indicates negative symptom severity in psychosis.

Schizophrenia is a severe mental disorder, in which 50% of the patients present with motor abnormalities such as psychomotor slowing. Slow spontaneous gait has been reported in schizophrenia. However, comprehensive objective instrumental assessments of multiple gait conditions are missing. Finally, the specific gait patterns of subjects with psychomotor slowing are still unknown. Therefore, this study aimed to objectively assess multiple gait parameters at different walking conditions in patients with schizophrenia with and without psychomotor slowing. Also, we hypothesised gait impairments to correlate with expert ratings of hypokinetic movement disorders and negative symptoms. We collected gait data (GAITRite®) in 70 patients with psychomotor slowing (SRRS (Salpetriere retardation rating scale) ≥15), 22 non-psychomotor slowed patients (SRRS < 15), and 42 healthy controls. Participants performed four walking conditions (self-selected speed, maximum speed, head reclined, and eyes closed) and six gait parameters were extracted (velocity, cadence, stride length, functional ambulation profile (FAP), and variance of stride length and time). Patients with psychomotor slowing presented slower velocity, lower cadence, and shorter stride length in all walking conditions compared to healthy controls, with the non-slowed patients in an intermediate position (all F > 16.18, all p < 0.001). Secondly, slower velocity was associated with more severe hypokinetic movement disorders and negative symptoms. In conclusion, gait impairments exist in a spectrum with healthy controls on one end and patients with psychomotor slowing on the other end. Patients with psychomotor slowing are specifically impaired when an adaptation of gait patterns is required, contributing to the deleterious effects of sedentary behaviours.

Less in-toeing after femoral derotation osteotomy in adult patients with increased femoral version and posterior hip impingement compared to patients with femoral retroversion.

In-toeing of the foot was associated with high femoral version (FV), while Out-toeing was associated with femoral-retroversion. Therefore, we report on (i) foot-progression-angle (FPA), (ii) prevalence of In-toeing and Out-toeing, and (iii) clinical outcome of patients treated with femoral-derotation-osteotomy (FDO). We performed a retrospective analysis involving 20 patients (20 hips) treated with unilateral FDO (2017-18). Of them, 14 patients had increased FV, 6 patients had femoral-retroversion. Follow-up time was mean 1 ± 1 years. All patients had minimal 1-year follow-up and the mean age was 29 ± 8 years. Patients with increased FV (FV > 35°) presented with positive posterior-impingement-test and mean FV was 49 ± 11° (Murphy method). Six patients with femoral-retroversion (FV < 10°) had positive anterior impingement test and mean FV of 5 ± 4°. Instrumented gait analysis was performed preoperatively and at follow-up using the Gaitrite system to measure FPA and was compared to a control group of 18 healthy asymptomatic volunteers (36 feet, mean age 29 ± 6 years). (i) Mean FPA increased significantly (P = 0.006) from preoperative 1.3 ± 7° to 4.5 ± 6° at follow-up for patients with increased FV and was not significantly different compared to the control group (4.0 ± 4.5°). (ii) In-toeing decreased from preoperatively (five patients) to follow-up (two patients) for patients with increased FV. Out-toeing decreased from preoperatively (two patients) to follow-up (no patient) for patients with femoral-retroversion. (iii) Subjective-hip-value of all patients increased significantly (P < 0.001) from preoperative 21 to 78 points at follow-up. WOMAC was 12 ± 8 points at follow-up. Patients with increased FV that underwent FDO walked with less In-toeing. FDO has the potential to reduce In-toeing and Out-toeing and to improve subjective satisfaction at follow-up.

Stroke Prediction Based on the Spontaneous Nystagmus Suppression Test in Dizzy Patients: A Diagnostic Accuracy Study.

OBJECTIVE: Failure of fixation suppression of spontaneous nystagmus is sometimes seen in patients with vestibular strokes involving the cerebellum or brainstem; however, the accuracy of this test for the discrimination between peripheral and central causes in patients with an acute vestibular syndrome (AVS) is unknown. METHODS: Patients with AVS were screened and recruited (convenience sample) as part of a prospective cross-sectional study in the emergency department between 2015 and 2020. All patients received neuroimaging, which served as a reference standard. We recorded fixation suppression with video-oculography (VOG) for forward, right, and left gaze. The ocular fixation index (OFI) and the spontaneous nystagmus slow velocity reduction was calculated. RESULTS: We screened 1,646 patients reporting dizziness in the emergency department and tested for spontaneous nystagmus in 148 patients with AVS. We analyzed 56 patients with a diagnosed acute unilateral vestibulopathy (vestibular neuritis) and 28 patients with a confirmed stroke. There was a complete nystagmus fixation suppression in 49.5% of patients with AVS, in 40% of patients with vestibular neuritis, and in 62.5% of patients with vestibular strokes. OFI scores had no predictive value for detecting strokes; however, a nystagmus reduction of less than 2 °/s showed a high accuracy of 76.9% (confidence interval 0.59-0.89) with a sensitivity of 62.2% and specificity of 84.8% in detecting strokes. CONCLUSIONS: The presence of fixation suppression does not rule out a central lesion. The magnitude of suppression was lower compared to patients with vestibular neuritis. The nystagmus suppression test predicts vestibular strokes accurately provided that eye movements are recorded with VOG. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that in patients with an AVS, decreased fixation suppression recorded with VOG occurred more often in stroke (76.9%) than in vestibular neuritis (37.8%).

Vestibular rehabilitation in Europe: a survey of clinical and research practice.

Vestibular rehabilitation (VR) is practiced across Europe but little in this area has been quantified. The aim of this study was to investigate current VR assessment, treatment, education, and research practices. This was an online, cross-sectional survey with 39 VR specific questions and four sections: demographics, current practice, education, and research. The survey was disseminated through the Dizzynet network to individual therapists through country-specific VR special interest groups. Results were analysed descriptively. A thematic approach was taken to analyse open questions. A total of 471 individuals (median age 41, range 23 - 68 years, 73.4% women), predominately physiotherapists (89.4%) from 20 European countries responded to the survey. They had worked for a median of 4 years (range < 1 - 35) in VR. The majority (58.7%) worked in hospital in-patient or out-patient settings and 21.4% in dedicated VR services. Most respondents specialized in neurology, care of the elderly (geriatrics), or otorhinolaryngology. VR was reported as hard/very hard to access by 48%, with the main barriers to access identified as lack of knowledge of health care professionals (particularly family physicians), lack of trained therapists, and lack of local services. Most respondents reported to know and treat benign paroxysmal positional vertigo (BPPV 87.5%), unilateral vestibular hypofunction (75.6%), and cervicogenic dizziness (63%). The use of vestibular assessment equipment varied widely. Over 70% used high-density foam and objective gait speed testing. Over 50% used dynamic visual acuity equipment. Infrared systems, Frenzel lenses, and dynamic posturography were not commonly employed (< 20%). The most frequently used physical outcome measures were the Clinical Test of the Sensory Interaction of Balance, Functional Gait Assessment/Dynamic Gait Index, and Romberg/Tandem Romberg. The Dizziness Handicap Inventory, Visual Analogue Scale, Falls Efficacy Scale, and the Vertigo Symptom Scale were the most commonly used patient reported outcome measures. Adaptation, balance, and habituation exercises were most frequently used (> 80%), with virtual reality used by 15.6%. Over 70% reported knowledge/use of Semont, Epley and Barbeque-Roll manoeuvres for the treatment of BPPV. Most education regarding VR was obtained at post-registration level (89.5%) with only 19% reporting pre-registration education. There was strong (78%) agreement that therapists should have professionally accredited postgraduate certification in VR, with blended learning the most popular mode. Three major research questions were identified for priority: management of specific conditions, effectiveness of VR, and mechanisms/factors influencing vestibular compensation and VR. In summary, the survey quantified current clinical practice in VR across Europe. Knowledge and treatment of common vestibular diseases was high, but use of published subjective and objective outcome measures as well as vestibular assessment varied widely. The results stress the need of improving both training of therapists and standards of care. A European approach, taking advantage of best practices in some countries, seems a reasonable approach.