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.

[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.

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.

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.

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.

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.

Heading Direction Is Significantly Biased by Preceding Whole-Body Roll-Orientation While Lying.

Background: After a prolonged static whole-body roll-tilt, a significant bias of the internal estimates of the direction of gravity has been observed when assessing the subjective visual vertical. Objective: We hypothesized that this post-tilt bias represents a more general phenomenon, broadly affecting spatial orientation and navigation. Specifically, we predicted that after the prolonged roll-tilt to either side perceived straight-ahead would also be biased. Methods: Twenty-five healthy participants were asked to rest in three different lying positions (supine, right-ear-down, and left-ear-down) for 5 min ("adaptation period") prior to walking straight-ahead blindfolded for 2 min. Walking was recorded with the inertial measurement unit sensors attached to different body locations and with sensor shoe insoles. The raw data was segmented with a gait-event detection method. The Heading direction was determined and linear mixed-effects models were used for statistical analyses. Results: A significant bias in heading into the direction of the previous roll-tilt position was observed in the post-adaptation trials. This bias was identified in both measurement systems and decreased again over the 2-min walking period. Conclusions: The bias observed further confirms the influence of prior knowledge on spatial orientation and navigation. Specifically, it underlines the broad impact of a shifting internal estimate of direction of gravity over a range of distinct paradigms, illustrating similar decay time constants. In the broader context, the observed bias in perceived straight-ahead emphasizes that getting up in the morning after a good night's sleep is a vulnerable period, with an increased risk of falls and fall-related injuries due to non-availability of optimally tuned internal estimates of the direction of gravity and the direction of straight-ahead.

Vestibular performance in high-level soccer and ice hockey players: Sport-specific norm values and implications.

OBJECTIVES: Quantitative vestibular testing in athletes after sports-related concussion (SRC) has become more popular due to accompanying injuries of the peripheral-vestibular organs that require targeted treatment. Sports-specific normative values are currently not available. Taking into account potential adaptational mechanisms, we obtained sports-specific, age- and peak-head-velocity-corrected normative values of peripheral-vestibular function and postural-stability in football (soccer, FB) and ice-hockey (IH) players. DESIGN: Retrospective single-center case-control study. METHOD: Pre-seasonal angular vestibulo-ocular reflex (aVOR) gains and cumulative-saccadic-amplitudes were obtained using the video-head-impulse test and performance in the balance-error-scoring-system (BESS) was recorded and compared in high-level FB-players (n = 510, 197 females) and IH-players (n = 210, males only) (age-range = 13-39y) and in healthy normals (n = 49, 22 females). Statistical analysis was performed using a generalized linear model. RESULTS: aVOR-gain values were significantly higher for FB-players than for IH-players (1.07 ±â€¯0.21 vs. 0.98 ±â€¯0.13, p < 0.001) and controls (1.07 ±â€¯0.21 vs. 0.97 ±â€¯0.17, p < 0.001). Significant age-related changes in aVOR-gains were only observed for the anterior and posterior canals in the IH-players. Cumulative-saccadic-amplitudes were clearly below established cut-off values (0.73°/trial). BESS scores were significantly higher in IH-players than in FB-players (15.4 ±â€¯5.1 vs. 11.2 ±â€¯4.9, p < 0.001). CONCLUSIONS: The significantly better performance of the FB players in the vertical aVOR-gains and the BESS compared to the IH-players could be related to sports-specific differences influencing visuo-vestibular and balance performance. Therefore, we recommend using the established normative aVOR-gain values for high-level FB-players, whereas in IH obtaining individual pre-seasonal (baseline) aVOR-gain values is proposed. Further studies should add sports-specific normative aVOR-gain values for IH and other sports.

Negative myoclonus as the leading symptom in acute cefepime neurotoxicity.

An 86-year-old woman was diagnosed with hospital-acquired pneumonia with Pseudomonas aeruginosa and treated with cefepime adjusted to her renal clearance. After 4 days, she developed acute-onset negative myoclonus without signs of altered mental status. After ruling out an acute intracranial haemorrhagic or ischaemic stroke as well as other metabolic and endocrine causes of negative myoclonus, the antibiotic was switched to piperacillin/tazobactam due to a suspicion of cefepime neurotoxicity. The patient improved within 24 hours and her symptoms fully resolved within 4 days. These observations suggest a link of the negative myoclonus to acute cefepime neurotoxicity, which may occur without or with minimal alteration of mental status, thus extending its spectrum of clinical presentation.

Unilateral orolingual angioedema in a patient with sarcoidosis after intravenous thrombolysis due to acute stroke without improvement after treatment with icatibant.

A potential complication after intravenous administration of recombinant tissue plasminogen activators (rtPAs) for thrombolysis in acute ischaemic stroke is orolingual angioedema, with an incidence of 0.4%-7.9%. In the herewith reported case, we discuss potential links between a history of sarcoidosis and the occurrence of orolingual angioedema after rtPA administration. Sarcoidosis is often accompanied by an elevated ACE level. In contrast, low ACE levels appear to play a role in the pathomechanism currently assumed to trigger angioedema, that is, the activation of the bradykinin and complement pathways. Medication with ACE inhibitors is considered a risk factor for angioedema. Based on these considerations, the patient was also treated with icatibant, a bradykinin B2-receptor antagonist, which has been found useful in recent publications on treating orolingual angioedema after intravenous lysis in ischaemic stroke.

Vestibular and Ocular Motor Properties in Lateral Medullary Stroke Critically Depend on the Level of the Medullary Lesion.

Background: Lateral medullary stroke (LMS) results in a characteristic pattern of brainstem signs including ocular motor and vestibular deficits. Thus, an impaired angular vestibulo-ocular reflex (aVOR) may be found if the vestibular nuclei are affected. Objective: We aimed to characterize the frequency and pattern of vestibular and ocular-motor deficits in patients with LMS. Methods: Patients with MR-confirmed acute/subacute unilateral LMS from a stroke registry were included and a bedside neuro-otological examination was performed. Video-oculography and video-based head-impulse testing (vHIT) was obtained and semicircular canal function was determined. The lesion location/extension as seen on MRI was rated and involvement of the vestibular nuclei was judged. Results: Seventeen patients with LMS (age = 59.4 ± 14.3 years) were included. All patients had positive H.I.N.T.S. vHIT showed mild-to-moderate aVOR impairments in three patients (ipsilesional = 1; ipsilesional and contralesional = 1; contralesional = 1). Spontaneous nystagmus (n = 10/15 patients) was more often beating contralesionally than ipsilesionally (6 vs. 3) and was accompanied by upbeat nystagmus in four patients. Head-shaking nystagmus was noted in seven subjects, ipsilesionally beating in six and down-beating in one. On brain MRI, damage of the most caudal parts of the medial and/or inferior vestibular nucleus was noted in 13 patients. Only those two patients with lesions affecting the rostral medulla oblongata demonstrated an ipsilaterally impaired aVOR. Conclusions: While subtle ocular motor signs pointed to damage of the central-vestibular pathways in all 17 patients, aVOR deficits were infrequent, restricted to those patients with rostral medullary lesions and, if present, mild to moderate only. This can be explained by lesions located too far caudally and too far ventrally to substantially affect the vestibular nuclei.

Non-linearity in gaze holding: Experimental results and possible mechanisms.

Cerebellar impairment may cause deficits in horizontal gaze holding, leading to centrifugal gaze-evoked nystagmus during fixation of eccentric targets. Healthy individuals show a weak drift leading to physiological nystagmus only at large gaze angles. These drifts are due to imperfect memory of the neural circuitry generating the eye position signals by integration of velocity signals. The cerebellum plays a crucial role in reducing the "leakiness" of this neural integrator. This neural integrator has been traditionally modeled as a first order low-pass filter, implying a linear relation between drift velocity and eye eccentricity. Evidences of a non-linear behavior, however, can be found in the literature. In a recent series of papers we showed that the eye drift velocity (V) can be descriptively modeled as a tangent function of gaze eccentricity (P) with the following equation: V=k2/k1tan(k1P). Notably, the two parameters have distinct roles: k1 regulates the rate of compression of the tangent, exclusively determining the non-linearity; k2 is a pure scaling factor. This descriptive model robustly fitted the data of healthy individuals both at baseline (n=50) and following transient cerebellar impairment induced by controlled amounts of alcohol [blood alcohol content 0.06% (n=15) and 0.1% (n=15)] and of patients with chronic cerebellar impairment of various origin (n=20). Interestingly, alcohol selectively changed the scaling factor k2, evidencing that an overall, transient cerebellar impairment does not impact the non-linear behavior. Patients with cerebellar degeneration, on the other hand, showed a change in both k1 and k2, implying a role of the cerebellum in limiting the range of eye positions where the non-linearity becomes relevant. Non-linearity has been reported in literature for both the neural integrator and the eye plant. While previous models using a neural network attempted to reproduce the non-linear behavior of the brainstem, we propose a block diagram reproducing the observed PV tangent relation by introducing a position dependency in the parameters of the cerebellar feedback loop.

Vestibular Dysfunction in Wernicke's Encephalopathy: Predominant Impairment of the Horizontal Semicircular Canals.

BACKGROUND: Wernicke's encephalopathy (WE), a metabolic disorder due to thiamine deficiency, manifests with various neurological symptoms and signs. It has been known as a cause of vestibular dysfunction. Preliminary reports have proposed predominant involvement of the horizontal semicircular canals (HSCs). OBJECTIVE: To better characterize the pattern of vestibular impairment in patients with WE using quantitative video head-impulse testing and to review the literature regarding this topic. METHOD: From January 2014 to December 2016, we retrospectively enrolled five cases of WE that received quantitative video-head-impulse testing (vHIT). We retrieved the clinical features from the medical records and reviewed quantitative head-impulse testing (qHIT) and caloric irrigation. Based on the gain and the number of corrective saccades, the function (normal vs. impaired) of each semicircular canal was rated. In addition, we conducted a MEDLINE and EMBASE search to identify other published cases of WE that had received qHIT. Neuro-otologic and neuro-ophthalmologic findings and vestibular testing results were extracted. RESULTS: A total of 17 patients (own series = 5; published cases = 12) aged 54.6 ± 11 years were included. Key neurologic findings were ataxia of stance and gait (13/13, 100%), spontaneous nystagmus (7/14, 50%), gaze-evoked nystagmus (GEN) (17/17, 100%), positive bedside head-impulse testing for the horizontal canals (16/17, 94%), and memory impairment and mental changes (6/11, 54.5%). Regarding vestibular testing, qHIT (either video based or search-coil based) documented selective bilateral horizontal canal dysfunction with normal or minimal vertical canal impairment (14/14, 100%). On caloric irrigation, bilateral horizontal canal paresis was noted in most cases (10/11, 91%). CONCLUSION: In WE, signs of both peripheral and central vestibular dysfunction (i.e., GEN, ataxia of stance and gait, abnormal head-impulse testing) were common. Selective or predominant impairment of the HSCs seems to be the most common finding of WE likely related to enhanced vulnerability of the medial vestibular nuclei neurons to thiamine deficiency. Quantitative vHIT of all six semicircular canals is therefore a useful tool for the diagnosis and should be applied in all patients with suspected WE.

Preferential Impairment of the Contralesional Posterior Semicircular Canal in Internuclear Ophthalmoplegia.

BACKGROUND: The vertical vestibulo-ocular reflex (VOR) may be impaired in internuclear ophthalmoplegia (INO) as the medial longitudinal fasciculus (MLF) conveys VOR-signals from the vertical semicircular canals. It has been proposed that signals from the contralesional posterior semicircular canal (PSC) are exclusively transmitted through the MLF, while for the contralesional anterior canal other pathways exist. OBJECTIVE: Here, we aimed to characterize dysfunction in individual canals in INO-patients using the video-head-impulse test (vHIT) and to test the hypothesis of dissociated vertical canal impairment in INO. METHODS: Video-head-impulse testing and magnetic resonance imaging were obtained in 21 consecutive patients with unilateral (n = 16) or bilateral (n = 5) INO and 42 controls. VOR-gains and compensatory catch-up saccades were analyzed and the overall function (normal vs. impaired) of each semicircular canal was rated. RESULTS: In unilateral INO, largest VOR-gain reductions were noted in the contralesional PSC (0.55 ± 0.11 vs. 0.89 ± 0.08, p < 0.001), while in bilateral INO both posterior (0.43 ± 0.11 vs. 0.89 ± 0.08, p < 0.001) and anterior (0.58 ± 0.19 vs. 0.88 ± 0.09, p < 0.001) canals showed marked drops. Small, but significant VOR-gain reductions were also found in the other canals in unilateral and bilateral INO-patients. Impairment of overall canal function was restricted to the contralesional posterior canal in 60% of unilateral INO-patients, while isolated involvement of the posterior canal was rare in bilateral INO-patients (20%). Reviewers correctly identified the INO-pattern in 15/21 (71%) patients and in all controls (sensitivity = 84.2% [95%-CI = 0.59.5-95.8]; specificity = 95.5% [95%-CI = 83.3-99.2]). CONCLUSION: Using a vHIT based overall rating of canal function, the correct INO-pattern could be identified with high accuracy. The predominant and often selective impairment of the contralesional posterior canal in unilateral INO further supports the role of the MLF in transmitting posterior canal signals. In patients with acute dizziness and abnormal vHIT-results, central pathologies such as INO should be considered as well, especially when the posterior canal is involved.