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Cleveland Clinic Journal of Medicine Nov 2022Benign paroxysmal positional vertigo (BPPV), caused by wayward crystals ("rocks") in the semicircular canals of the inner ear, is the most common cause of brief symptoms... (Review)
Review
Benign paroxysmal positional vertigo (BPPV), caused by wayward crystals ("rocks") in the semicircular canals of the inner ear, is the most common cause of brief symptoms of vertigo secondary to head and body movements. Diagnosing and treating it are simple to do in the medical office. This article reviews the differential diagnosis for patients presenting with dizziness and vertigo, the pathophysiology of BPPV, how to diagnose it using maneuvers to elicit symptoms and nystagmus, how to interpret the nystagmus pattern to determine where the rocks are, and how to treat it using different maneuvers to reposition ("roll") the rocks back where they belong.
Topics: Humans; Benign Paroxysmal Positional Vertigo; Semicircular Canals; Dizziness; Nystagmus, Pathologic; Patient Positioning
PubMed: 36319052
DOI: 10.3949/ccjm.89a.21057 -
Current Opinion in Neurology Feb 2018The clinical and laboratory assessment of nystagmus in patients with neurologic disorders can provide crucial elements for a state-of-the-art differential diagnosis. An... (Review)
Review
PURPOSE OF REVIEW
The clinical and laboratory assessment of nystagmus in patients with neurologic disorders can provide crucial elements for a state-of-the-art differential diagnosis. An increasing number of publications in the fields of neuro-otology and neuro-ophthalmology have nystagmus in the center of interest, which makes frequent updates on the diagnostic and therapeutic relevance of these contributions indispensable. This review covers important clinical studies and studies in basic research relevant for the neurologist published from January 2016 to August 2017.
RECENT FINDINGS
Current themes include vestibular nystagmus, positional nystagmus, optokinetic nystagmus and after-nystagmus, vibration-induced nystagmus, head-shaking nystagmus, postrotatory nystagmus, caloric nystagmus, nystagmus in cerebellar disorders, differential diagnosis of nystagmus and treatment approaches (whereas infantile nystagmus syndrome is not addressed in this review). These studies address mechanisms/pathomechanisms, differential diagnoses and treatment of different forms of nystagmus.
SUMMARY
In clinical practice, a structured description of nystagmus including its three-dimensional beating direction, trigger factors and duration is of major importance. The differential diagnosis of downbeat nystagmus is broad and includes acute intoxications, neurodegenerative disorders and cerebrovascular causes amongst others. In patients with positional nystagmus, the distinction between frequent benign peripheral and rare but dangerous central causes is imperative.
Topics: Humans; Nystagmus, Pathologic
PubMed: 29120919
DOI: 10.1097/WCO.0000000000000517 -
Pediatrics and Neonatology Oct 2014Nystagmus is an involuntary rhythmic oscillation of the eyes, which leads to reduced visual acuity due to the excessive motion of images on the retina. Nystagmus can be... (Review)
Review
Nystagmus is an involuntary rhythmic oscillation of the eyes, which leads to reduced visual acuity due to the excessive motion of images on the retina. Nystagmus can be grouped into infantile nystagmus (IN), which usually appears in the first 3-6 months of life, and acquired nystagmus (AN), which appears later. IN can be idiopathic or associated to albinism, retinal disease, low vision, or visual deprivation in early life, for example due to congenital cataracts, optic nerve hypoplasia, and retinal dystrophies, or it can be part of neurological syndromes and neurologic diseases. It is important to differentiate between infantile and acquired nystagmus. This can be achieved by considering not only the time of onset of the nystagmus, but also the waveform characteristics of the nystagmus. Neurological disease should be suspected when the nystagmus is asymmetrical or unilateral. Electrophysiology, laboratory tests, neurological, and imaging work-up may be necessary, in order to exclude any underlying ocular or systemic pathology in a child with nystagmus. Furthermore, the recent introduction of hand-held spectral domain optical coherence tomography (HH SD-OCT) provides detailed assessment of foveal structure in several pediatric eye conditions associated with nystagmus and it can been used to determine the underlying cause of infantile nystagmus. Additionally, the development of novel methods to record eye movements can help to obtain more detailed information and assist the diagnosis. Recent advances in the field of genetics have identified the FRMD7 gene as the major cause of hereditary X-linked nystagmus, which will possibly guide research towards gene therapy in the future. Treatment options for nystagmus involve pharmacological and surgical interventions. Clinically proven pharmacological treatments for nystagmus, such as gabapentin and memantine, are now beginning to emerge. In cases of obvious head posture, eye muscle surgery can be performed to shift the null zone of the nystagmus into the primary position, and also to alleviate neck problems that can arise due to an abnormal head posture.
Topics: Cataract; Child; Eye Movements; Head; Humans; Nystagmus, Pathologic; Posture
PubMed: 25086850
DOI: 10.1016/j.pedneo.2014.02.007 -
Academic Emergency Medicine : Official... May 2023This third Guideline for Reasonable and Appropriate Care in the Emergency Department (GRACE-3) from the Society for Academic Emergency Medicine is on the topic adult...
This third Guideline for Reasonable and Appropriate Care in the Emergency Department (GRACE-3) from the Society for Academic Emergency Medicine is on the topic adult patients with acute dizziness and vertigo in the emergency department (ED). A multidisciplinary guideline panel applied the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach to assess the certainty of evidence and strength of recommendations regarding five questions for adult ED patients with acute dizziness of less than 2 weeks' duration. The intended population is adults presenting to the ED with acute dizziness or vertigo. The panel derived 15 evidence-based recommendations based on the timing and triggers of the dizziness but recognizes that alternative diagnostic approaches exist, such as the STANDING protocol and nystagmus examination in combination with gait unsteadiness or the presence of vascular risk factors. As an overarching recommendation, (1) emergency clinicians should receive training in bedside physical examination techniques for patients with the acute vestibular syndrome (AVS; HINTS) and the diagnostic and therapeutic maneuvers for benign paroxysmal positional vertigo (BPPV; Dix-Hallpike test and Epley maneuver). To help distinguish central from peripheral causes in patients with the AVS, we recommend: (2) use HINTS (for clinicians trained in its use) in patients with nystagmus, (3) use finger rub to further aid in excluding stroke in patients with nystagmus, (4) use severity of gait unsteadiness in patients without nystagmus, (5) do not use brain computed tomography (CT), (6) do not use routine magnetic resonance imaging (MRI) as a first-line test if a clinician trained in HINTS is available, and (7) use MRI as a confirmatory test in patients with central or equivocal HINTS examinations. In patients with the spontaneous episodic vestibular syndrome: (8) search for symptoms or signs of cerebral ischemia, (9) do not use CT, and (10) use CT angiography or MRI angiography if there is concern for transient ischemic attack. In patients with the triggered (positional) episodic vestibular syndrome, (11) use the Dix-Hallpike test to diagnose posterior canal BPPV (pc-BPPV), (12) do not use CT, and (13) do not use MRI routinely, unless atypical clinical features are present. In patients diagnosed with vestibular neuritis, (14) consider short-term steroids as a treatment option. In patients diagnosed with pc-BPPV, (15) treat with the Epley maneuver. It is clear that as of 2023, when applied in routine practice by emergency clinicians without special training, HINTS testing is inaccurate, partly due to use in the wrong patients and partly due to issues with its interpretation. Most emergency physicians have not received training in use of HINTS. As such, it is not standard of care, either in the legal sense of that term ("what the average physician would do in similar circumstances") or in the common parlance sense ("the standard action typically used by physicians in routine practice").
Topics: Adult; Humans; Dizziness; Benign Paroxysmal Positional Vertigo; Nystagmus, Pathologic; Risk Factors; Emergency Service, Hospital
PubMed: 37166022
DOI: 10.1111/acem.14728 -
Neurologic Clinics Aug 2015Diagnosing dizziness can be challenging, and the consequences of missing dangerous causes, such as stroke, can be substantial. Most physicians use a diagnostic paradigm... (Review)
Review
Diagnosing dizziness can be challenging, and the consequences of missing dangerous causes, such as stroke, can be substantial. Most physicians use a diagnostic paradigm developed more than 40 years ago that focuses on the type of dizziness, but this approach is flawed. This article proposes a new paradigm based on symptom timing, triggers, and targeted bedside eye examinations (TiTrATE). Patients fall into 1 of 4 major syndrome categories, each with its own differential diagnosis and set of targeted examination techniques that help make a specific diagnosis. Following an evidence-based approach could help reduce the frequency of misdiagnosis of serious causes of dizziness. In the spirit of the flipped classroom, the editors of this Neurologic Clinics issue on emergency neuro-otology have assembled a collection of unknown cases to be accessed electronically in multimedia format. By design, cases are not linked with specific articles, to avoid untoward cueing effects for the learner. The cases are real and are meant to demonstrate and reinforce lessons provided in this and subsequent articles. In addition to pertinent elements of medical history, cases include videos of key examination findings.
Topics: Acute Disease; Critical Care; Dizziness; Emergency Service, Hospital; Humans; Nystagmus, Pathologic; Syndrome; Vertigo
PubMed: 26231273
DOI: 10.1016/j.ncl.2015.04.011 -
Stroke Nov 2009Acute vestibular syndrome (AVS) is often due to vestibular neuritis but can result from vertebrobasilar strokes. Misdiagnosis of posterior fossa infarcts in emergency... (Comparative Study)
Comparative Study
BACKGROUND AND PURPOSE
Acute vestibular syndrome (AVS) is often due to vestibular neuritis but can result from vertebrobasilar strokes. Misdiagnosis of posterior fossa infarcts in emergency care settings is frequent. Bedside oculomotor findings may reliably identify stroke in AVS, but prospective studies have been lacking.
METHODS
The authors conducted a prospective, cross-sectional study at an academic hospital. Consecutive patients with AVS (vertigo, nystagmus, nausea/vomiting, head-motion intolerance, unsteady gait) with >or=1 stroke risk factor underwent structured examination, including horizontal head impulse test of vestibulo-ocular reflex function, observation of nystagmus in different gaze positions, and prism cross-cover test of ocular alignment. All underwent neuroimaging and admission (generally <72 hours after symptom onset). Strokes were diagnosed by MRI or CT. Peripheral lesions were diagnosed by normal MRI and clinical follow-up.
RESULTS
One hundred one high-risk patients with AVS included 25 peripheral and 76 central lesions (69 ischemic strokes, 4 hemorrhages, 3 other). The presence of normal horizontal head impulse test, direction-changing nystagmus in eccentric gaze, or skew deviation (vertical ocular misalignment) was 100% sensitive and 96% specific for stroke. Skew was present in 17% and associated with brainstem lesions (4% peripheral, 4% pure cerebellar, 30% brainstem involvement; chi(2), P=0.003). Skew correctly predicted lateral pontine stroke in 2 of 3 cases in which an abnormal horizontal head impulse test erroneously suggested peripheral localization. Initial MRI diffusion-weighted imaging was falsely negative in 12% (all <48 hours after symptom onset).
CONCLUSIONS
Skew predicts brainstem involvement in AVS and can identify stroke when an abnormal horizontal head impulse test falsely suggests a peripheral lesion. A 3-step bedside oculomotor examination (HINTS: Head-Impulse-Nystagmus-Test-of-Skew) appears more sensitive for stroke than early MRI in AVS.
Topics: Acute Disease; Adult; Aged; Aged, 80 and over; Cross-Sectional Studies; Diffusion Magnetic Resonance Imaging; Female; Humans; Male; Middle Aged; Nausea; Nystagmus, Pathologic; Point-of-Care Systems; Prospective Studies; Reflex, Vestibulo-Ocular; Stroke; Syndrome; Time Factors; Vertigo; Vomiting
PubMed: 19762709
DOI: 10.1161/STROKEAHA.109.551234 -
Acta Otorhinolaryngologica Italica :... Dec 2017Differential diagnosis between peripheral and central spontaneous nystagmus can be difficult to classify (as peripheral or central) even on the basis of criteria...
Differential diagnosis between peripheral and central spontaneous nystagmus can be difficult to classify (as peripheral or central) even on the basis of criteria recommended in the recent literature. The aim of this paper is to use the combination of spontaneous nystagmus and ocular tilt reaction to determine the site of origin of the disease that causes nystagmus. We propose to classify the nystagmus in: 1) "Uphill" nystagmus in which the nystagmus takes on an inclined plane and the direction of the fast phase is towards the hypertropic eye (this type of nystagmus is likely peripheral); 2) "Downhill" nystagmus when the nystagmus beats toward the hypotropic eye (this type of nystagmus is likely central); 3) "Flat" nystagmus when the plane on which nystagmus beats is perfectly horizontal: in this case, we cannot say anything about the site of lesion (it was only detected in 15% of cases). The spatial position of nystagmus vector has to be considered as an intrinsic characteristic of the nystagmus itself (as direction, frequency, angular velocity etc.) and must be reported in the description, possibly giving an indication of the site of damage (peripheral or central). In particular, similar results are obtained by comparing the inclination of the nystagmus with the head impulse test (HIT, considered the best bedside test now available). It seems that this sign may confirm HIT for safer diagnosis or replace it in case of doubt. In contrast, in case of "Flat" nystagmus (probably attributable to the fact that the utricular maculae are spared), HIT can replace observation of the plane of the nystagmus. Thus, the two signs confirm and integrate each other. The test does not require additional time and is not tedious for the patient. It is proposed that it be included in the evaluation of spontaneous nystagmus in everyday clinical practice.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Nystagmus, Pathologic; Young Adult
PubMed: 29327737
DOI: 10.14639/0392-100X-1403 -
Journal of the Neurological Sciences Apr 2017Acquired pendular nystagmus is comprised of quasi-sinusoidal oscillations of the eyes significantly affecting gaze holding and clarity of vision. The most common causes... (Review)
Review
Acquired pendular nystagmus is comprised of quasi-sinusoidal oscillations of the eyes significantly affecting gaze holding and clarity of vision. The most common causes of acquired pendular nystagmus include demyelinating disorders such as multiple sclerosis and the syndrome of ocular palatal tremor. However, several other deficits, such as pharmacological intoxication, metabolic and genetic disorders, and granulomatous disorders can lead to syndromes mimicking acquired pendular nystagmus. Study of the kinematic features of acquired pendular nystagmus has suggested a putative pathophysiology of an otherwise mysterious neurological disorder. Here we review clinical features of neurological deficits that co-occur with acquired pendular nystagmus. Subsequent discussion of the pathophysiology of individual forms of pendular nystagmus speculates on mechanisms of the underlying disease while providing insights into pharmacotherapy of nystagmus.
Topics: Demyelinating Diseases; Diagnosis, Differential; Humans; Nervous System Diseases; Nystagmus, Pathologic; Tremor
PubMed: 28320194
DOI: 10.1016/j.jns.2017.01.033 -
Journal of Vestibular Research :... 2019This paper presents a classification and definitions for types of nystagmus and other oscillatory eye movements relevant to evaluation of patients with vestibular and... (Review)
Review
This paper presents a classification and definitions for types of nystagmus and other oscillatory eye movements relevant to evaluation of patients with vestibular and neurological disorders, formulated by the Classification Committee of the Bárány Society, to facilitate identification and communication for research and clinical care. Terminology surrounding the numerous attributes and influencing factors necessary to characterize nystagmus are outlined and defined. The classification first organizes the complex nomenclature of nystagmus around phenomenology, while also considering knowledge of anatomy, pathophysiology, and etiology. Nystagmus is distinguished from various other nystagmus-like movements including saccadic intrusions and oscillations.View accompanying videos at http://www.jvr-web.org/ICVD.html.
Topics: Diagnosis, Differential; Eye Movements; Humans; Nystagmus, Pathologic; Ocular Motility Disorders; Saccades; Terminology as Topic; Vestibular Diseases; Vestibular Function Tests; Vestibule, Labyrinth
PubMed: 31256095
DOI: 10.3233/VES-190658 -
Investigative Ophthalmology & Visual... Feb 2022Children with Down's syndrome (DS) are known to have poorer visual acuity than neurotypical children. One report has shown that children with DS and nystagmus also have... (Comparative Study)
Comparative Study
PURPOSE
Children with Down's syndrome (DS) are known to have poorer visual acuity than neurotypical children. One report has shown that children with DS and nystagmus also have poor acuity when compared to typical children with nystagmus. What has not been established is the extent of any acuity deficit due to nystagmus and whether nystagmus affects refractive error within a population with DS.
METHODS
Clinical records from the Cardiff University Down's Syndrome Vision Research Unit were examined retrospectively. Binocular visual acuity and refraction data were available for 50 children who had DS and nystagmus and 176 children who had DS but no nystagmus. Data were compared between the two groups and with published data for neurotypical children with nystagmus.
RESULTS
The study confirms the deficit in acuity in DS, compared to neurotypical children, of approximately 0.2 logMAR and shows a deficit attributable to nystagmus of a further 0.2 logMAR beyond the first year of life. Children with both DS and nystagmus clearly have a significant additional impairment. Children with DS have a wide range of refractive errors, but nystagmus increases the likelihood of myopia. Prevalence and axis direction of astigmatism, on the other hand, appear unaffected by nystagmus.
CONCLUSIONS
Nystagmus confers an additional visual impairment on children with DS and must be recognized as such by families and educators. Children with both DS and nystagmus clearly need targeted support.
Topics: Astigmatism; Child; Child, Preschool; Down Syndrome; Female; Humans; Infant; Infant, Newborn; Male; Nystagmus, Pathologic; Refraction, Ocular; Refractive Errors; Retrospective Studies; Vision Disorders; Vision Tests; Vision, Binocular; Visual Acuity
PubMed: 35195683
DOI: 10.1167/iovs.63.2.28