Vestibular Migraine.

PURPOSE OF REVIEW: To explore recent developments in vestibular migraine (VM). RECENT FINDINGS: This review discusses the current diagnostic criteria for VM in the adult and pediatric populations, as proposed by the International Headache Society and Bárány Society. Recent VM studies confirm the prior findings and reveal new insights, including the wide range of vestibular symptoms, symptoms in the attack-free period, and triggers. Many patients experience persistent vestibular symptoms, even in the absence of acute attacks, which often significantly impact patients' quality of life. The syndrome of benign recurrent vertigo and its relationship to migraine, VM, and Meniere's disease is also discussed. There is a dearth of randomized controlled trials in VM treatment. Prospective and retrospective studies support the benefit of many migraine treatments are effective in VM, including neuromodulation, and calcitonin gene-related peptide monoclonal antibodies. VM affects almost 3% of the population, but remains under-diagnosed. Recent diagnostic criteria can help clinicians diagnose VM in adults and children.

The Neuropsychology of Dizziness and Related Disorders.

There is a reciprocal relationship between vestibular and neuropsychological disorders. People with vertigo and dizziness are at higher risk of various psychiatric disorders, particularly anxiety, depression, and panic disorder. On the other hand, people with mood disorders are at higher risk of experiencing vertigo and dizziness. Vestibular information plays a crucial role in cognitive processes, especially visuo-spatial abilities. Consequently, vestibular disorders (both peripheral and central) often result in visuo-spatial deficits. In addition, lesions of the cortical and subcortical components of the vestibular system result in disorders of higher vestibular function, such as hemispatial neglect, pusher syndrome, and topographagnosia.

Care Gaps and Recommendations in Vestibular Migraine: An Expert Panel Summit.

Vestibular migraine (VM) is an increasingly recognized pathology yet remains as an underdiagnosed cause of vestibular disorders. While current diagnostic criteria are codified in the 2012 Barany Society document and included in the third edition of the international classification of headache disorders, the pathophysiology of this disorder is still elusive. The Association for Migraine Disorders hosted a multidisciplinary, international expert workshop in October 2020 and identified seven current care gaps that the scientific community needs to resolve, including a better understanding of the range of symptoms and phenotypes of VM, the lack of a diagnostic marker, a better understanding of pathophysiologic mechanisms, as well as the lack of clear recommendations for interventions (nonpharmacologic and pharmacologic) and finally, the need for specific outcome measures that will guide clinicians as well as research into the efficacy of interventions. The expert group issued several recommendations to address those areas including establishing a global VM registry, creating an improved diagnostic algorithm using available vestibular tests as well as others that are in development, conducting appropriate trials of high quality to validate current clinically available treatment and fostering collaborative efforts to elucidate the pathophysiologic mechanisms underlying VM, specifically the role of the trigemino-vascular pathways.

The Interconnections of Mal de Débarquement Syndrome and Vestibular Migraine.

OBJECTIVES/HYPOTHESIS: Mal de débarquement syndrome (MDDS) is characterized by a persistent rocking sensation, as though on a boat. It may occur following exposure to passive motion (motion-triggered MDDS [MT-MDDS]), or spontaneously (spontaneous-onset MDDS [SO-MDDS]). This study investigated the characteristics of MDDS patients with vestibular migraine (MDDS-VM) to those without (MDDS-O). STUDY DESIGN: Retrospective review. METHODS: Retrospective, single-center study of 62 patients with MDDS. Clinical characteristics, Dizziness Handicap Inventory (DHI), Migraine Disability Assessment Score (MIDAS), job impact, and optimal treatment(s) were studied. RESULTS: There were 23 MDDS-O (19 women), and 39 MDDS-VM (35 women) patients. Comparisons between MDDS-VM and MDDS-O showed significant differences in age of onset (41 vs. 52 years, P = .005), interictal visually induced dizziness (89.7% vs. 30.4%, P < .001), interictal head motion-induced dizziness (87.2% vs. 47.8%, P = .001), other vestibular sensations (59% vs. 13%, P < .001), interictal aural symptoms (25.6% vs. 0%, P = .008), number of interictal symptoms (4.3 vs. 2.3, P < .001), total DHI score (54.9 vs. 38.1, P = .005), DHI-P (physical domain) score (16.1 vs. 10, P = .004), DHI-F (functional domain) score (20.9 vs. 15.7, P = .016 MIDAS (4.6 vs. 32, P = .002), and job resignations (23.2% vs. 5%, P = .016). On the other hand, between-group comparisons for MT-MDDS and SO-MDDS did not reveal any significant differences whatsoever. For optimal treatment, venlafaxine was the most used (27.3%) in all groups. For MDDS-VM, antiepileptic drugs and migraine preventive vitamins were also useful in relieving symptoms. CONCLUSIONS: MDDS-VM patients appear to be more disabled than MDDS-O, in terms of severity of dizziness, job impact, and number of symptoms, but have good potential for improvement, particularly with migraine prophylactic treatment. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:E1653-E1661, 2021.

Nystagmus and Vertigo in Acute Vestibular Migraine Attacks: Response to Non-Invasive Vagus Nerve Stimulation.

OBJECTIVE: Vestibular migraine (VM) is the most common neurologic cause of vertigo in adults, but there are no currently-approved rescue therapies. This study describes the benefits of non-invasive vagus nerve stimulation (nVNS) on vertigo, headache, and nystagmus during VM attacks. METHODS: Case series of four VM patients who were evaluated during acute VM episodes in a tertiary referral neurology clinic between February 2019 and January 2020. They underwent bedside neuro-otologic examination, and graded the severity of vertigo and headache using a 10-point visual-analog scale (VAS; 0-no symptoms, 10-worst ever symptoms), before and 15 minutes after nVNS. RESULTS: Average vertigo severity was 5 (median 4.5) before, and 1.5 (median 0.5) after nVNS. Mean headache severity (three patients) before treatment was 4 (median 4), and 0.7 (median 0) after. Spontaneous right-beating nystagmus (Patient 1) nystagmus, upbeat nystagmus (Patient 2), and positional nystagmus (Patient 3) resolved with nVNS. Baseline left-beating nystagmus in Patient 4 from previous vestibular neuritis damped during acute VM but returned to baseline following nVNS. In all four patients, ictal nystagmus resolved, and examination findings returned to baseline. CONCLUSIONS: This study suggests nVNS may ameliorate vertigo and headache, as well as nystagmus associated with VM attacks. Larger, sham device-controlled studies are needed to elucidate the benefits of nVNS in VM.

External trigeminal nerve stimulation: Potential rescue treatment for acute vestibular migraine.

OBJECTIVE: Vestibular migraine (VM) is the most common neurologic cause of vertigo among adults. However, there are no specifically studied or approved rescue therapies for acute VM attacks. This study describes how external trigeminal nerve stimulation (eTNS) using the Cefaly® (CEFALY Technology, Seraing, Belgium) device relieves acute VM episodes. METHODS: Single-center, retrospective review of 19 patients with acute VM attacks (seen between May 2018 and June 2019) treated with 20-min eTNS. Prior to treatment, patients graded the severity of their vertigo/headache using a 10-point visual analog scale (VAS) with 0 representing no vertigo/headache, and 10 representing the worst imaginable vertigo/headache. After eTNS, patients graded their vertigo/headache using the same VAS 15 min. In addition, bedside neuro-otologic examination was performed before and after treatment. RESULTS: 19/19 patients reported improvement in vertigo severity. Mean vertigo severity was 6.6 (±2.1; median 7) before eTNS, and 2.7 (±2.6; median 3) following treatment; mean improvement in vertigo was 61.3% (±32.6; median 50.0%). During VM episodes, 14/19 experienced headache. Mean headache severity was 4.8 (±2.4; median 4.5) before eTNS, and was 1.4 (±2.4; median 0) following treatment; mean improvement in headache was 77.2% (±32.7; median 100.0%). Neuro-otologic examination was normal during VM attacks in all except Patient 7 who had spontaneous upbeat nystagmus which resolved after eTNS. Other improvements include improvement of eye pressure, head pressure, and chronic facial pain. No intolerable side effects were reported. CONCLUSION: This study provides preliminary evidence that eTNS is a novel, non-invasive, safe and effective treatment for acute VM attacks.

Acute vestibular migraine treatment with noninvasive vagus nerve stimulation.

OBJECTIVE: To report on the benefits of noninvasive vagus nerve stimulation (nVNS) on acute vestibular migraine (VM) treatment. METHODS: This was a retrospective chart review of patients with VM treated with nVNS in a single tertiary referral center between November 2017 and January 2019. Eighteen patients (16 women) were identified (mean age 45.7 [±14.8] years); 14 were treated for a VM attack and 4 for bothersome interictal dizziness consistent with persistent perceptual postural dizziness (PPPD). Patients graded the severity of vestibular symptoms and headache using an 11-point visual analog scale (VAS; 0 = no symptoms, 10 = worst ever symptoms) before and 15 minutes after nVNS. RESULTS: In those with acute VM, vertigo improved in 13/14 (complete resolution in 2, at least 50% improvement in 5). The mean vertigo intensity before nVNS was 5.2 (±1.6; median 6), and 3.1 (±2.2; median 3) following stimulation; mean reduction in vertigo intensity was 46.9% (±31.5; median 45%). Five experienced headache with the VM attack; all reported improvement following nVNS. Mean headache severity was 6 (±1.4; median 6) prior to treatment and 2.4 (±1.5; median 3) following nVNS; mean reduction in headache intensity was 63.3% (±21.7; median 50). All 4 treated with nVNS for interictal PPPD reported no benefit. CONCLUSION: Our study provides preliminary evidence that nVNS may provide rapid relief of vertigo and headache in acute VM, and supports further randomized, sham-controlled studies into nVNS in VM. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for patients with acute VM, nVNS rapidly relieves vertigo and headache.

Vestibular Migraine: How to Sort it Out and What to Do About it.

BACKGROUND: Vestibular migraine (VM) is the most common neurologic cause of vertigo in adults and results in significant utilization of health care resources, but remains under-recognized and underdiagnosed. EVIDENCE ACQUISITION: Review of literature in PubMed using the following terms: vestibular migraine, migraine-associated vertigo, vertiginous migraine, benign recurrent vertigo, migraine-associated dizziness, migraine, migraine treatment, Meniere disease (MD), vertebrobasilar ischemia (VBI), posterior circulation stroke, benign paroxysmal positional vertigo, and episodic-ataxia Type 2 (EA2). RESULTS: VM can manifest with a variety of vestibular symptoms, including spontaneous vertigo, triggered vertigo, positional vertigo, and head-motion dizziness. Patients may report more than 1 vestibular symptom. Episodes of vertigo are often, but not always, accompanied by headache. Auditory symptoms are frequently associated with VM attacks and may mimic the manifestations of MD. Other migrainous features that accompany VM attacks include photophobia, phonophobia, osmophobia, and visual aura. Interictally, patients may suffer from persistent dizziness or isolated paroxysmal vestibular symptoms. Mood disorders (particularly anxiety) are often found in VM. Abnormal neuro-otologic findings are not uncommon in patients with VM. Differential diagnoses for VM include MD, VBI, EA2, and migraine with brainstem aura. For rescue treatment, triptans, vestibular suppressants, and/or antiemetic agents may be considered. Pharmacologic migraine preventives (antiepileptics, beta-blockers, and antidepressants) are often useful. CONCLUSIONS: The keys to correctly diagnosing VM is identifying a relationship between vestibular symptoms and migrainous features and being aware of the heterogeneity of manifestations of this enigmatic, but treatable, condition. The principles of treatment of VM include rescue therapy, lifestyle modification, nonpharmacologic migraine preventives, pharmacologic migraine prophylaxis, and treatment of comorbidities.

Seeing function in structure: "incidental" eye findings on OCT in a patient with multiple sclerosis.

A 17-year-old girl with relapsing-remitting multiple sclerosis (MS) was referred for a 2-year history of visual blurriness. Her bedside examination was remarkable for gait unsteadiness only. Optical coherence tomography was performed as part of her workup. Unexpectedly, Spectralis© video imaging revealed left torsional nystagmus that was not apparent on bedside examination. Review of previous brain magnetic resonance images (MRI) revealed left ocular deviation, as well as a left dorsolateral medullary MS plaque, which was the cause of her torsional nystagmus. We highlight how Spectralis© scanning confocal laser ophthalmoscopy allows video imaging that can capture torsional ocular movements.

The Spectrum of Vestibular Migraine: Clinical Features, Triggers, and Examination Findings.

OBJECTIVE: To assess the ictal symptoms, interictal symptoms, psychiatric comorbidities, and interictal neuro-otologic examination findings in vestibular migraine (VM). METHODS: Retrospective chart review of 491 patients seen from August 2014 until March 2018 at a tertiary neurology referral center for vestibular disorders to identify patients fulfilling the 2012 VM criteria. RESULTS: One hundred and thirty-one patients (105 women) were identified. Mean age of VM onset was 44.3 (±13.7) years. Preceding the onset of vestibular symptoms, most had migraine (57.3%) and motion sickness (61.1%). It was common to have a family history of migraine (50.8%) and episodic vestibular symptoms (28.1%). Common ictal symptoms were triggered (visually induced and head-motion) and spontaneous vertigo, accompanied by photophobia and phonophobia (118/131 [90.1%] patients), nausea (105/131 [80.2%] patients), aural symptoms (79/131 [60.3%] patients), and headache (65/131 [49.6%] patients). Interictally, many experienced visually induced (116/131 [88.6%] patients), head-motion (86/131 [65.6%] patients), and persistent (67/131 [51.1%] patients) dizziness. Psychiatric comorbidities include anxiety (92/131 [70.2%] patients), depression (53/131 [40.5%] patients), insomnia (38/131 [29.0%] patients), phobic disorders (15/131 [11.5%] patients), and psychogenic disorders (11/131 [8.4%] patients). Common triggers were stress (52/131 [39.7%] patients), bright lights (35/131 [26.7%] patients), weather changes (34/131 [26.0%] patients), and sleep deprivation (34/131 [26.0%] patients). Interictal neuro-otologic examination was abnormal in 56/131 (42.7%), usually hyperventilation-induced, head-shaking-induced, vibration-induced, and positional nystagmus. The most common balance-test finding was impaired sharpened Romberg's test (22/130 [16.9%] patients). CONCLUSIONS: In this single center study, we found that VM typically affects women in their 40s, with a personal and family history of migraine. Typical ictal symptoms were triggered and spontaneous vertigo, associated with photophobia and phonophobia, nausea, aural symptoms, and headache. Interictal vestibular symptoms, comorbid psychiatric disorders, and non-specific interictal neuro-otologic findings were common.

Characteristics of morphologic macular abnormalities in neuroimmunology practice.

BACKGROUND: Morphologic macular abnormalities (MMAs) are frequently seen on macular optical coherence tomography (OCT) imaging in neuroimmunology practice, yet studies pragmatically assessing prevalence and risk factors of MMAs to date are limited. OBJECTIVE: To describe the characteristics of MMAs in a neuroimmunology-based academic practice. METHODS: Cross-sectional study of 1450 patients (2900 eyes) who underwent spectral-domain macular OCT between June 2010 and June 2012. The association between MMAs and demographic variables was analyzed using mixed-effects logistic regression. Odds ratios (ORs) were calculated per 5-year age increments. RESULTS: MMAs were observed in 338/2872 eyes (11.7%) of 232/1445 participants (16.1%). The most common abnormalities identified, included drusen (6.0%), epiretinal membrane (ERM; 5.5%), and microcystoid macular pathology (MMP; 1.9%). Overall, patients with MMAs were older (OR: 1.79, p = 5 × 10-5) and more likely to be males (OR: 2.45, p = 0.014). In particular, advancing age was associated with higher risk of drusen and ERM (OR: 1.80 and 4.26, p = 2 × 10-5 and 7 × 10-3, respectively). MMP prevalence declined with age (OR: 0.73, p = 0.015) and was associated with African-American ethnicity (OR: 15.0, p = 5 × 10-5). CONCLUSION: Unexpected or incidental MMAs are common in patients assessed with OCT in neuroimmunology practice, emphasizing the importance of comprehensive OCT image review for risk stratification and appropriate ophthalmology referral.

Clinical characteristics of Alice in Wonderland syndrome in a cohort with vestibular migraine.

BACKGROUND: Alice in Wonderland syndrome (AIWS) is a rare sensory perception disorder, most often caused by migraine in adults. We aimed to characterize the clinical characteristics of AIWS in a cohort of vestibular migraine (VM) patients. METHODS: Retrospective chart review of patients diagnosed with VM seen between August 2014 and January 2018. RESULTS: Seventeen patients were identified (10 women) with a median age at onset of 45 years (range 15-61 years), and median age at presentation of 49 years (range 17-63 years). Eighty-two percent reported 1 AIWS symptom, 12% reported 3 symptoms, and 6% described 2 symptoms. The most common symptom was visual distortions (47%), followed by extrapersonal misperceptions (41%) and somesthetic distortions (29%). Most AIWS occurred during VM episodes (77%). Eleven patients were seen in follow-up; 10 described complete or partial resolution of both AIWS and VM with migraine preventive therapy, while 1 experienced complete resolution of VM but continued to have AIWS. Neuro-otologic abnormalities improved in 2 patients. CONCLUSIONS: This study characterizes the clinical features of AIWS in patients with VM. We observed several rare and highly unusual AIWS misperceptions (frosted-glass vision, underwater vision, dolly zoom effect, sensation of the brain coming out of the head, closed-eye visual hallucinations, and headlight glare-induced marco/microsomatognosia), and resolution or improvement in AIWS and VM with migraine preventive treatment.

Horizontal Direction-Changing Positional Nystagmus and Vertigo: A Case of Vestibular Migraine Masquerading as Horizontal Canal BPPV.

Episodic positional vertigo is typically due to benign paroxysmal positional vertigo (BPPV) but may also be a manifestation of vestibular migraine. Distinguishing vestibular migraine from BPPV is essential since the treatment of each disorder is markedly different. The 31-month clinical course of a 41-year-old woman with vestibular migraine causing recurrent positional vertigo is described. During vestibular migraine attacks, she developed left-beating nystagmus in the upright position with removal of fixation, and geotropic horizontal nystagmus during the supine roll test. Interictally, her exam demonstrated positional apogeotropic horizontal nystagmus with the supine roll test, more intense in the supine head left position. Her vestibular migraine was successfully controlled with topiramate and eletriptan.

Temporal evolution of the trident and piglet signs of osmotic demyelination syndrome.

Central pontine myelinolysis (CPM) is a potentially-devastating complication of rapid osmolar shifts, classically attributed to overlyaggressive correction of chronic hyponatremia. Magnetic resonance imaging (MRI) allowed earlier diagnosis of CPM, but most importantly, it has revealed that the odds of good functional recovery are surprisingly high. A trident shaped pontine lesion is a typical finding in CPM (the trident sign). The "piglet sign" is a much less well-known radiologic finding in CPM. Due to the rarity of CPM, very little has been published on the evolution of these MRI findings. We present a case of CPM in an alcoholic young man, and describe the temporal evolution of both the trident and piglet signs on MRI in CPM.

Cerebellar Control of Eye Movements.

BACKGROUND: The cerebellum plays a central role in the online, real-time control, and long-term modulation of eye movements. EVIDENCE ACQUISITION: We reviewed the latest (fifth) edition of Leigh and Zee's textbook, The Neurology of Eye Movements, and literature in PUBMED using the following terms: cerebellum, flocculus, paraflocculus, vermis, oculomotor vermis, dorsal vermis, caudal fastigial nucleus, fastigial oculomotor region, uvula, nodulus, ansiform lobule, eye movements, saccades, ipsipulsion, contrapulsion, smooth pursuit, vergence, convergence, divergence, gaze-holding, down beat nystagmus, vestibulo-ocular reflex (VOR), angular VOR, translational VOR, skew deviation, velocity storage. RESULTS: The cerebellum is vital in optimizing the performance of all classes of gaze-shifting and gaze-stabilizing reflexes. The flocculus-paraflocculus are crucial to VOR gain and direction, pulse-step matching for saccades, pursuit gain, and gaze-holding. The ocular motor vermis and caudal fastigial nuclei are essential in saccadic adaptation and accuracy, and pursuit gain. The nodulus and ventral uvula are involved in processing otolothic signals and VOR responses, including velocity storage. CONCLUSIONS: The cerebellum guarantees the precision of ocular movements to optimize visual performance and occupies a central role in all classes of eye movements both in real-time control and in long-term calibration and learning (i.e., adaptation).