Is There an “Acquired Idiopathic Head-Shaking Nystagmus”?—A Discussion of Mechanisms and Clinical Implications Based on a Case Report

Background: Head-shaking nystagmus (HSN) occurs in both peripheral and central vestibular disorders. In most cases, HSN can be attributed to an asymmetric peripheral vestibular input or a structural lesion mostly in the cerebellum affecting the central velocity storage mechanism. An isolated HSN is very rare. Case Presentation: We report on a young female patient with the clinical picture of recurrent episodes of vertigo, which were induced by fast head movements and were accompanied by a severe right-beating HSN with a long time constant of 60 s. There was no other clinical and instrument-based evidence of peripheral vestibular dysfunction (including video head impulse test, caloric test, vestibular-evoked myogenic potentials) or indication of a structural lesion in the nodulus, uvula or pontomedullary brainstem on fine-slice magnetic resonance imaging. She had no previous history of migraine, hearing deficits, or other focal neurological symptoms. Diagnostic criteria for vestibular paroxysmia, vestibular migraine, benign peripheral paroxysmal vertigo, or any other known vestibular disorders were not fulfilled. Chart review in the database of the German Center for Vertigo and Balance Disorders indicated eight additional patients with a similar clinical phenotype between 2018 and 2022. Conclusion: We propose a clinical entity called acquired idiopathic head shaking nystagmus (aiHSN) as a rare cause of episodic vertigo induced by fast head movements. Nystagmus characteristics suggest a subtle functional pathology of the central velocity storage mechanism in the nodulus and uvula, which is exacerbated during symptomatic episodes

Episodic ataxias in children and adolescents: Clinical findings and suggested diagnostic criteria

Background: The main clinical presentation of episodic ataxias (EAs) consists of vertigo and dizziness attacks lasting for minutes to hours with widely varying accompanying symptoms. The differentiation of EA and episodic vertigo/dizziness syndromes in childhood and adolescence such as vestibular migraine (VM) and recurrent vertigo of childhood (RVC) can be challenging. Furthermore, only few prospective studies of children/adolescents with EA are available. Objective: This study aims to characterize clinical and instrument-based findings in EA patients under 18 years of age, to delineate the clinical and therapeutic course in EA, and to present potentially new genetic mutations. Furthermore, the study aims to differentiate distinct characteristics between EA, VM, and RVC patients. Methods: We prospectively collected clinical and instrument-based data of patients younger than 18 years, who presented at the German Center for Vertigo and Balance Disorders (DSGZ) at the LMU University Hospital in Munich with EA, VM, or RVC between January 2016 and December 2021. All patients underwent a comprehensive evaluation of neurological, ocular-motor, vestibular and cochlear function, including video-oculography with caloric testing, video head impulse test, vestibular evoked myogenic potentials, posturography, and gait analysis. Results: Ten patients with EA, 15 with VM, and 15 with RVC were included. In EA the main symptoms were vertigo/dizziness attacks lasting between 5 min and 12 h. Common accompanying symptoms included walking difficulties, paleness, and speech difficulties. Six EA patients had a previously unknown gene mutation. In the interictal interval all EA patients showed distinct ocular-motor deficits. Significant differences between EA, VM, and RVC were found for accompanying symptoms such as speech disturbances and paleness, and for the trigger factor “physical activity”. Furthermore, in the interictal interval significant group differences were observed for different pathological nystagmus types, a saccadic smooth pursuit, and disturbed fixation suppression. Conclusion: By combining clinical and ocular-motor characteristics we propose diagnostic criteria that can help to diagnose EA among children/adolescents and identify patients with EA even without distinct genetic findings. Nevertheless, broad genetic testing (e.g., next generation sequencing) in patients fulfilling the diagnostic criteria should be conducted to identify even rare or unknown genetic mutations for EA

Recurrent Vertigo of Childhood: Clinical features and prognosis

INTRODUCTION: “Recurrent Vertigo of Childhood” (RVC) has recently replaced the term “Benign Paroxysmal Vertigo of Childhood” and was defined as recurrent spells of vertigo without evidence of a vestibular migraine of childhood (VMC). RVC and VMC are considered the most frequent causes of vertigo and dizziness in children below 18 years of age. Diagnosis might be challenging since clinical features of RVC and VMC may overlap. OBJECTIVE: This study aims to characterize clinical and instrument-based findings in patients with RVC and to evaluate the course of the disorder. METHODS: We prospectively collected clinical and instrument-based data of children/adolescents younger than 18 years, who presented at the German Center for Vertigo and Balance Disorders (DSGZ) at the LMU University Hospital in Munich. All patients underwent a comprehensive neurological, ocular motor, vestibular and cochlear examination. Furthermore, findings from follow-up examinations were analyzed. RESULTS: Overall 42 children (24 male and 18 female) with RVC were included in the study. The mean age at diagnosis was 7 ± 3.6 years with a mean onset of symptoms at the age of 5.6 ± 3.4 years. Attack duration ranged between 1 min and 4 h. The most common accompanying symptoms included nausea, vomiting, expression of fear, and falls. Non-migrainous headaches were reported by 11 patients during initial presentation, 7 of whom were later diagnosed with migraine. Female patients showed a higher age at symptom onset, a higher attack frequency, and attack duration. Eleven of the 24 patients seen at a 3.5 year follow-up reported a complete cessation of attacks. Patients still experiencing vertigo attacks had a significantly reduced attack frequency, especially those who implemented at least one prophylactic measure. CONCLUSION: A precise characterization of symptoms is essential for diagnosing children with RVC. Age at symptom onset does not exceed the age of 12. Gender-specific differences should be considered and may further support the evidence of an association with migraine. The disease course of RVC is benign, nevertheless implementing prophylactic measures such as regular exercise, increased fluid intake, sleep hygiene, and relaxation exercises, can improve attack frequency

Reflexive and Intentional Saccadic Eye Movements in Migraineurs

Background: Migraine has been postulated to lead to structural and functional changes of different cortical and subcortical areas, including the frontal lobe, the brainstem, and cerebellum. The (sub-)clinical impact of these changes is a matter of debate. The spectrum of possible clinical differences include domains such as cognition but also coordination. The present study investigated the oculomotor performance of patients with migraine with and without aura compared to control subjects without migraine in reflexive saccades, but also in intentional saccades, which involve cerebellar as well as cortical networks. Methods: In 18 patients with migraine with aura and 21 patients with migraine without aura saccadic eye movements were recorded in two reflexive (gap, overlap) and two intentional (anti, memory) paradigms and compared to 25 controls without migraine. Results: The main finding of the study was an increase of saccade latency in patients with and without aura compared to the control group solely in the anti-task. No deficits were found in the execution of reflexive saccades. Conclusions: Our results suggest a specific deficit in the generation of correct anti-saccades, such as vector inversion. Such processes are considered to need cortical networks to be executed correctly. The parietal cortex has been suggested to be involved in vector inversion processes but is not commonly described to be altered in migraine patients. It could be discussed that the cerebellum, which is recently thought to be involved in the pathophysiology of migraine, might be involved in distinct processes such as spatial re-mapping through known interconnections with parietal and frontal cortical areas

Migraine and tension type headache in adolescents at grammar school in Germany - burden of disease and health care utilization

Background Tension-type headache and migraine are among the most prevalent chronic disorders in children/adolescents. Data on health care utilization for headache in this age group, however, are sparse. Methods In 1399 grammar school students (aged 12–19 years) with headache in the last six months in Germany a) the burden of disease for headache (mean intensity, mean frequency in the last three months and PedMIDAS means), b) medical care utilization defined by proportion of students consulting a physician in the last 12 months and/or taking analgetic drugs in the last three months by headache types (migraine and tension-type headache) and by burden of disease were assessed. Results Primary headache substantially impaired daily living activities in adolescents which was mainly related to migraine. Medical care utilization and drug use, however, was low (consulting a physician: 12.0 %, 95 %-CI = [10.3-13.8]; taking analgetic drugs: 29.9 %, 95 %-CI = [27.5-32.4]) - even among students with severe headache (physician consultation: <35 %; taking analgetic drugs: <63 %). Two thirds of students with any headache and 40 % of those with migraine had neither seen a physician nor used analgetic drugs because of their headache in the preceding 12 months. Conclusions Adolescents with headache might too rarely seek professional help for treatment of headache. Health promotion in adolescents should increase awareness for evidence-based treatment options for headache

Computerized clinical decision system and mobile application with expert support to optimize management of vertigo in primary care: study protocol for a pragmatic cluster-randomized controlled trial

Vertigo and dizziness are amongst the most common symptoms in medicine and often have a major impact on activities of daily life. Although many causes of vertigo and dizziness can easily be recognized, patients often receive inappropriate and ineffective treatment. The reasons for this are various. Because vertigo/dizziness is an interdisciplinary symptom and there is a lack of standardised diagnostic tools, it is easy to lose the overview of the possible differential diagnoses. There is evidence though, that the management of patients with vertigo/dizziness can be optimized using standardized care pathways with digital support. The present study (within the framework of \textquotedblPoiSe-prevention, online feedback, and interdisciplinary therapy of acute vestibular syndromes by e-health\textquotedbl) aims to evaluate the implementation of a program with several interlocking components. The three main components are a computerized clinical decision system, a mobile application, a counselling and interdisciplinary educational program developed by the German Center for Vertigo and Balance Disorders (DSGZ). The study is a cluster-randomized controlled trial with a parallel-group design, as well as a detailed process evaluation. Clusters comprise of primary care physician practices in Bavaria, Germany. In the scope of the study the effectiveness, acceptability and efficiency of the intervention will be evaluated. It is anticipated that the intervention will improve the quality and efficiency of the management of dizzy patients. A higher diagnostic accuracy, optimized treatment, and disease progression monitoring is expected to improve patient-relevant outcomes and reduce health-care costs

Coronary artery calcification score in migraine patients

Epidemiological studies have shown an increased risk of cardiovascular events in migraineurs. The pathophysiological mechanisms of this observation remain largely unknown. Recent genetic and epidemiologic studies suggest, that atherosclerosis might be the overlapping pathophysiological mechanism in migraine and coronary heart disease. The aim of the present study was to evaluate if the increased cardiovascular risk in migraineurs is attributed to an increased coronary artery calcification. For this the coronary artery calcium score was assessed by computed tomography of the heart in 1.437 patients of which 337 were migraineurs. All patients had a similar cardiovascular risk profile, so that the risk for coronary calcifications could be considered similar between migraineurs and non-migraineurs. The results showed no significant differences in the amount of coronary calcifications in patients with or without migraine. This suggests that a more pronounced coronary artery calcification, as a surrogate marker of coronary atherosclerosis, does not underlie the increased cardiovascular risk in migraineurs. A distinct common pathophysiological mechanism in migraine and coronary heart disease such as endothelial dysfunction or vasospasm should be discussed instead. However, it has to be considered, that the coronary artery calcification score does not indicate the total risk of atherosclerotic changes in the coronary arteries

Period Prevalence of Dizziness and Vertigo in Adolescents

Objectives To assess the period prevalence and severity of dizziness and vertigo in adolescents. Methods In 1661 students in 8th-10th grade in twelve grammar schools in Munich, Germany information on vertigo/dizziness was assessed by a questionnaire in the class room setting. Three month prevalence of dizziness/vertigo was estimated;symptoms were categorized as orthostatic dizziness, spinning vertigo, swaying vertigo or unspecified dizziness. Duration of symptoms and impact on daily life activities were assessed. Results 72.0% (95%-CI = [69.8-74.2];N = 1196) of the students (mean age 14.5 +/- 1.1) reported to suffer fromat least one episode of dizziness or vertigo in the last three months. Most adolescents ticked to have symptoms of orthostatic dizziness (52.0%,95%-CI = [49.5-54.4],N = 863). The period prevalence for the other types of vertigo were spinning vertigo: 11.6%,95%-CI = [10.1-13.3],N = 193;swaying vertigo: 12.2%,95%-CI = [10.6-13.8],N = 202;and unspecified dizziness: 15.2%,95%-CI = [13.5-17.1],N = 253. About 50% of students with spinning vertigo and swaying vertigo also report to have orthostatic dizziness. Most vertigo/dizziness types were confined to less than one minute on average. The proportion of students with any dizziness/vertigo accounting for failure attending school, leisure activities or obliging them to stay in bed were more pronounced for spinning or swaying vertigo. Conclusion Dizziness and vertigo in grammar school students appear to be as common as in adults. In face of the high period prevalence and clinical relevance of dizziness/vertigo in adolescents there is a need for prevention strategies. Risk factors for dizziness/vertigo need to be assessed to allow for conception of an intervention programme