History of Chronic Traumatic Encephalopathy
Semin Neurol DOI: 10.1055/s-0040-1713622The long-term effects of repetitive head impacts have been recognized for close to a century. What is now referred to as chronic traumatic encephalopathy (CTE) was first described by Martland in 1928 in a series of boxers. Over the years, several important articles were published, including Critchley's 1957 report where he introduced the term “chronic traumatic encephalopathy,” Robert's study in 1969 which provided evidence of the prevalence of neurological impairment in retired fighters, and Corsellis' initial description of the pathology of CTE. However, what brought pu...
Source: Seminars in Neurology - August 10, 2020 Category: Neurology Authors: Cantu, Robert C. Bernick, Charles Tags: Review Article Source Type: research

Fluid Biomarkers for Chronic Traumatic Encephalopathy
Semin Neurol DOI: 10.1055/s-0040-1715095Chronic traumatic encephalopathy (CTE) is a neuropathological condition that has been described in individuals who have been exposed to repetitive head impacts, including concussions and subconcussive trauma. Currently, there is no fluid or imaging biomarker for diagnosing CTE during life. Based on retrospective clinical data, symptoms of CTE include changes in behavior, cognition, and mood, and may develop after a latency phase following the injuries. However, these symptoms are often nonspecific, making differential diagnosis based solely on clinical symptoms unreliable. Thus, obje...
Source: Seminars in Neurology - August 2, 2020 Category: Neurology Authors: Shahim, Pashtun Gill, Jessica M. Blennow, Kaj Zetterberg, Henrik Tags: Review Article Source Type: research

Clinical Presentation of Chronic Traumatic Encephalopathy
This article reviews what is currently known about the clinical presentation of CTE and describes the evolution of this knowledge from early case reports of “punch drunk” boxers through larger case series of neuropathologically confirmed CTE. This article concludes with a discussion of gaps in research and future directions to address these areas. [...] Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals: Table of contents  |  Abstract  |  Full text (Source: Seminars in Neurology)
Source: Seminars in Neurology - August 2, 2020 Category: Neurology Authors: Mariani, Megan Alosco, Michael L. Mez, Jesse Stern, Robert A. Tags: Review Article Source Type: research

Chronic Traumatic Encephalopathy: Advocacy and Communicating with the Public
Semin Neurol DOI: 10.1055/s-0040-1713621Over the past 40 years, advocacy groups have been instrumental in raising awareness for neurodegenerative diseases such as Alzheimer's disease. More recently, advocates have emerged to educate about sports concussions and chronic traumatic encephalopathy (CTE), including the Concussion Legacy Foundation (CLF). CTE is a neurodegenerative disease caused in part by repetitive head impacts (RHI). While the majority of CTE research has focused on studying former American football players, CTE has also been found in military personnel, victims of domestic violence, and contact sport athlet...
Source: Seminars in Neurology - July 26, 2020 Category: Neurology Authors: Uretsky, Madeline Nowinski, Christopher J. Tags: Review Article Source Type: research

The Neuropathology of Chronic Traumatic Encephalopathy: The Status of the Literature
Semin Neurol DOI: 10.1055/s-0040-1713632Chronic traumatic encephalopathy (CTE) is a tauopathy associated with repetitive mild head trauma, including concussion and asymptomatic subconcussive impacts. CTE was first recognized in boxers almost a century ago and has been identified more recently in contact sports athletes, military veterans exposed to blast, and victims of domestic violence. Like most neurodegenerative diseases, CTE is diagnosed conclusively by a neuropathological examination of brain tissue. CTE is characterized by the buildup of hyperphosphorylated tau (p-tau) in neurofibrillary tangles (NFTs), neurites, an...
Source: Seminars in Neurology - July 26, 2020 Category: Neurology Authors: McKee, Ann C. Tags: Review Article Source Type: research

Genetics of Chronic Traumatic Encephalopathy
Semin Neurol DOI: 10.1055/s-0040-1713631Although chronic traumatic encephalopathy (CTE) garners substantial attention in the media and there have been marked scientific advances in the last few years, much remains unclear about the role of genetic risk in CTE. Two athletes with comparable contact-sport exposure may have varying amounts of CTE neuropathology, suggesting that other factors, including genetics, may contribute to CTE risk and severity. In this review, we explore reasons why genetics may be important for CTE, concepts in genetic study design for CTE (including choosing controls, endophenotypes, gene by environm...
Source: Seminars in Neurology - July 26, 2020 Category: Neurology Authors: Abdolmohammadi, Bobak Dupre, Alicia Evers, Laney Mez, Jesse Tags: Review Article Source Type: research

Risk Factors for Chronic Traumatic Encephalopathy: A Proposed Framework
Semin Neurol DOI: 10.1055/s-0040-1713633Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disease that has been neuropathologically diagnosed in contact and collision sport athletes, military veterans, and others with a history of exposure to repetitive head impacts (RHI). Identifying methods to diagnose and prevent CTE during life is a high priority. Timely diagnosis and implementation of treatment and preventative strategies for neurodegenerative diseases, including CTE, partially hinge upon early and accurate risk characterization. Here, we propose a framework of risk factors that influence the n...
Source: Seminars in Neurology - July 16, 2020 Category: Neurology Authors: Phelps, Alyssa Mez, Jesse Stern, Robert A. Alosco, Michael L. Tags: Review Article Source Type: research

Repetitive Head Trauma Induces Chronic Traumatic Encephalopathy by Multiple Mechanisms
Semin Neurol DOI: 10.1055/s-0040-1713620Exposure to repetitive neurotrauma increases lifetime risk for developing progressive cognitive deficits, neurobehavioral abnormalities, and chronic traumatic encephalopathy (CTE). CTE is a tau protein neurodegenerative disease first identified in boxers and recently described in athletes participating in other contact sports (notably American football, ice hockey, rugby, and wrestling) and in military veterans with blast exposure. Currently, CTE can only be diagnosed by neuropathological examination of the brain after death. The defining diagnostic lesion of CTE consists of patchy p...
Source: Seminars in Neurology - July 16, 2020 Category: Neurology Authors: Cherry, Jonathan D. Babcock, Katharine J. Goldstein, Lee E. Tags: Review Article Source Type: research

Chronic Traumatic Encephalopathy and Neuropathological Comorbidities
Semin Neurol DOI: 10.1055/s-0040-1713628With age, the presence of multiple neuropathologies in a single individual becomes increasingly common. Given that traumatic brain injury and the repetitive head impacts (RHIs) that occur in contact sports have been associated with the development of many neurodegenerative diseases, including chronic traumatic encephalopathy (CTE), Alzheimer's disease, Lewy body disease, and amyotrophic lateral sclerosis, it is becoming critical to understand the relationship and interactions between these pathologies. In fact, comorbid pathology is common in CTE and likely influenced by both age and...
Source: Seminars in Neurology - June 30, 2020 Category: Neurology Authors: Stein, Thor D. Crary, John F. Tags: Review Article Source Type: research

Pediatric Neurology
Semin Neurol 2020; 40: 275-276 DOI: 10.1055/s-0040-1713451 Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Neurology)
Source: Seminars in Neurology - May 28, 2020 Category: Neurology Authors: Singhal, Nilika Shah Nash, Kendall B. Tags: Preface Source Type: research

Nilika Singhal, MD, and Kendal Nash, MD
Semin Neurol 2020; 40: 273-274 DOI: 10.1055/s-0040-1713450 Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Neurology)
Source: Seminars in Neurology - May 28, 2020 Category: Neurology Authors: Greer, David M. Tags: Introduction to the Guest Editor Source Type: research

Update in Pediatric Pseudotumor Cerebri Syndrome
Semin Neurol DOI: 10.1055/s-0040-1708847Pseudotumor cerebri syndrome (PTCS) is a rare condition in children presenting with headache and papilledema from increased intracranial pressure that can cause significant morbidity. This can be idiopathic, also known as idiopathic intracranial hypertension or primary intracranial hypertension, or can be secondary to medications and associated medical conditions. Given the threat to vision, early detection and treatment is needed in all age groups. However, identifying papilledema or pseudopapilledema in children presents unique challenges sometimes as a result of differences betwee...
Source: Seminars in Neurology - May 18, 2020 Category: Neurology Authors: Beres, Shannon J. Tags: Review Article Source Type: research

Neurocognitive Outcomes in Children with Brain Tumors
Semin Neurol DOI: 10.1055/s-0040-1708867Brain tumors are the most common solid tumor in childhood, and although survival outcomes continue to improve, survival is frequently offset by devastating late effects from tumor and treatment. The long-term effects of brain tumors and the therapy necessary to treat them range from sensory and neuroendocrine abnormalities to neurocognitive deficits leading to inferior quality of life. The multifactorial neurocognitive injury is one of the most broadly impacting and challenging late effects to predict and subsequently treat. Certain treatment modalities, such as intrathecal methotrex...
Source: Seminars in Neurology - May 11, 2020 Category: Neurology Authors: Kline, Cassie N. Mueller, Sabine Tags: Review Article Source Type: research

Lifestyle Advice for Pediatric Migraine: Blaming the Patient, or Evidence Based?
This article reviews the current state of the evidence for each of these behavioral interventions for migraine prevention, and provides the clinician with practical advice for counseling patients. [...] Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals: Table of contents  |  Abstract  |  Full text (Source: Seminars in Neurology)
Source: Seminars in Neurology - April 15, 2020 Category: Neurology Authors: Gelfand, Amy A. Irwin, Samantha L. Tags: Review Article Source Type: research

Targeted Treatments for Inherited Neuromuscular Diseases of Childhood
Semin Neurol DOI: 10.1055/s-0040-1702940In the past decade, the number of genes linked to neuromuscular diseases of childhood has expanded dramatically, and this genetic information is forming the basis for gene-specific and even mutation-specific therapies. At the forefront of these advances are the two recently approved treatments for spinal muscular atrophy: one, an antisense oligonucleotide that modifies splicing of the SMN2 gene, and, the other, a gene therapy vector that delivers the SMN1 gene to motor neurons, both of which are allowing patients to acquire developmental milestones previously unseen in this fatal dis...
Source: Seminars in Neurology - April 15, 2020 Category: Neurology Authors: Fay, Alex J. Knox, Renatta Neil, Erin E. Strober, Jonathan Tags: Review Article Source Type: research

Multiple Sclerosis in Children: Current and Emerging Concepts
Semin Neurol 2020; 40: 192-200 DOI: 10.1055/s-0040-1703000Multiple sclerosis is being increasingly recognized and diagnosed in children. In the past several years, advances have been made in diagnosing multiple sclerosis in children, identifying new genetic and environmental risk factors, delineating underlying immunobiology, characterizing imaging findings, and implementing new treatment strategies. In this review, we discuss these advances. Future research into the determinants of multiple sclerosis in children and into new treatment options will be aided by continued international collaboration. [...] Thieme Medical Pub...
Source: Seminars in Neurology - April 15, 2020 Category: Neurology Authors: Brenton, J. Nicholas Kammeyer, Ryan Gluck, Lauren Schreiner, Teri Makhani, Naila Tags: Review Article Source Type: research

Pediatric Neurology
Semin Neurol 2020; 40: 175-176 DOI: 10.1055/s-0040-1709146 Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Neurology)
Source: Seminars in Neurology - April 8, 2020 Category: Neurology Authors: Singhal, Nilika Shah Nash, Kendall B. Tags: Preface Source Type: research

Nilika Shah Singhal, MD, and Kendall B. Nash, MD
Semin Neurol 2020; 40: 173-174 DOI: 10.1055/s-0040-1709147 Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Neurology)
Source: Seminars in Neurology - April 8, 2020 Category: Neurology Authors: Greer, David M. Tags: Introduction to the Guest Editor Source Type: research

Juvenile Dermatomyositis and the Inflammatory Myopathies
Semin Neurol DOI: 10.1055/s-0040-1705120The inflammatory myopathies comprise disorders of immune-mediated muscle injury. The histopathology and clinical features help distinguish them. Juvenile dermatomyositis (JDM) is the most common form of myositis in children and adolescents. Children with JDM present with proximal muscle weakness and characteristic rashes. The presentation is similar in children and adults, but JDM is a primary disorder and the adult form often is concerning for a paraneoplastic syndrome. Proximal muscle weakness occurs with dermatomyositis, polymyositis, and immune-mediated necrotizing myopathy, but ...
Source: Seminars in Neurology - April 6, 2020 Category: Neurology Authors: Swafford, Collin Roach, E Steve Tags: Review Article Source Type: research

High-Flow Vascular Malformations in Children
Semin Neurol DOI: 10.1055/s-0040-1708869Children can have a variety of intracranial vascular anomalies ranging from small and incidental with no clinical consequences to complex lesions that can cause substantial neurologic deficits, heart failure, or profoundly affect development. In contrast to high-flow lesions with direct arterial-to-venous shunts, low-flow lesions such as cavernous malformations are associated with a lower likelihood of substantial hemorrhage, and a more benign course. Management of vascular anomalies in children has to incorporate an understanding of how treatment strategies may affect the normal dev...
Source: Seminars in Neurology - April 6, 2020 Category: Neurology Authors: Morshed, Ramin A. Winkler, Ethan A. Kim, Helen Braunstein, Steve Cooke, Daniel L. Hetts, Steven W. Abla, Adib A. Fullerton, Heather J. Gupta, Nalin Tags: Review Article Source Type: research

Extinguishing Febrile Infection-Related Epilepsy Syndrome: Pipe Dream or Reality?
Semin Neurol DOI: 10.1055/s-0040-1708503Febrile infection-related epilepsy syndrome (FIRES) is a rare and devastating epileptic encephalopathy with historically abysmal neurocognitive outcomes, including a high incidence of mortality. It tends to affect children and young adults and is characterized by superrefractory status epilepticus following a recent febrile illness. Growing evidence suggests a heterogeneous etiology resulting in fulminant nonantibody-mediated neuroinflammation. For some children with FIRES, this aberrant neuroinflammation appears secondary to a functional deficiency in the endogenous interleukin-1 re...
Source: Seminars in Neurology - March 17, 2020 Category: Neurology Authors: Payne, Eric T. Koh, Sookyong Wirrell, Elaine C. Tags: Review Article Source Type: research

Advances in the Treatment of Drug-Resistant Pediatric Epilepsy
Semin Neurol DOI: 10.1055/s-0040-1702941Epilepsy is a common disorder in children and adults that causes significant morbidity and affects many aspects of a patient's lives. Two-thirds of patients with epilepsy are controlled with established antiseizure medications, leaving a significant number of patients searching for other options. The purpose of this review is to provide an overview of recent advancements in the management of treatment-resistant epilepsy in pediatric patients. Recent publications have shown the efficacy of new pharmaceutical options such as fenfluramine and cannabidiol, some of which have been tested ...
Source: Seminars in Neurology - March 17, 2020 Category: Neurology Authors: Gonzalez-Giraldo, Ernesto Sullivan, Joseph E. Tags: Review Article Source Type: research

Rasmussen Encephalitis: An Update
Semin Neurol DOI: 10.1055/s-0040-1708504Rasmussen encephalitis (RE) is a rare, devastating, progressive pediatric epilepsy. First described 60 years ago, RE continues to present challenges in diagnosis and management. RE causes a unilateral focal epilepsy in children that typically becomes medically refractory, results in significant hemiparesis, and causes progressive cognitive decline. The etiology is a cell-mediated immune attack on one cerebral hemisphere, though the inciting antigen remains unknown. While the underlying histopathology is unilateral and RE is described as “unihemispheric,” studies have demo...
Source: Seminars in Neurology - March 17, 2020 Category: Neurology Authors: Cay-Martinez, Karla C. Hickman, Richard A. McKhann II, Guy M. Provenzano, Frank A. Sands, Tristan T. Tags: Review Article Source Type: research

Metabolic Disorders Presenting with Seizures in the Neonatal Period
Semin Neurol DOI: 10.1055/s-0040-1705119Metabolic disorders represent rare but often treatable causes of seizures and epilepsy of neonatal onset. As seizures are relatively common in the neonatal period, systemic clues to a specific diagnosis may be lacking or shrouded by acute illness. An important role of the consulting pediatric neurologist is to identify neonates with a possible metabolic or otherwise genetic diagnosis. In this review, the authors describe presenting signs and symptoms, a diagnostic framework, and disorder-specific treatment options for inborn errors of metabolism that may present in the neonatal perio...
Source: Seminars in Neurology - March 17, 2020 Category: Neurology Authors: Brimble, Elise Ruzhnikov, Maura R.Z. Tags: Review Article Source Type: research

Neonatal Seizures: Diagnosis, Etiologies, and Management
Semin Neurol DOI: 10.1055/s-0040-1702943Neonates are exquisitely susceptible to seizures due to several physiologic factors and combination of risks that are uniquely associated with gestation, delivery, and the immediate postnatal period. Neonatal seizures can be challenging to identify; therefore, it is imperative that clinicians have a high degree of suspicion for seizures based on the clinical history or the presence of encephalopathy with or without paroxysmal abnormal movements. Acute symptomatic neonatal seizures are due to an acute brain injury, whereas neonatal-onset epilepsy may be related to underlying structura...
Source: Seminars in Neurology - March 6, 2020 Category: Neurology Authors: Ziobro, Julie Shellhaas, Ren ée A. Tags: Review Article Source Type: research

Acute Flaccid Myelitis: A Clinical Review
Semin Neurol DOI: 10.1055/s-0040-1705123Acute flaccid myelitis (AFM) is an emerging disorder primarily affecting children that is characterized by acute flaccid paralysis accompanied by abnormalities of the spinal cord gray matter on magnetic resonance imaging. In most cases, prodromal fever or respiratory symptoms occur, followed by acute-onset flaccid limb weakness. Respiratory, axial, bulbar, facial, and extraocular muscles may also be affected. The clinical manifestations have been described as “polio-like,” due to striking similarities to cases of poliomyelitis. The primary site of injury in AFM is the ant...
Source: Seminars in Neurology - March 6, 2020 Category: Neurology Authors: Murphy, Olwen C. Pardo, Carlos A. Tags: Review Article Source Type: research

Infantile Spasms: Opportunities to Improve Care
Semin Neurol DOI: 10.1055/s-0040-1705121Infantile spasm (IS) is a distinct epilepsy syndrome characterized by epileptic spasms (the clinical seizure type) and hypsarrhythmia (the electrographic abnormality). IS is frequently accompanied by impaired neurodevelopment and is often associated with structural, genetic, or metabolic etiologies. Prompt treatment of this severe epileptic encephalopathy improves long-term outcomes but remains elusive in many situations. Despite common misconceptions, even patients with identified etiologies or preexisting developmental delay benefit from proven standard therapies, including adrenoc...
Source: Seminars in Neurology - March 6, 2020 Category: Neurology Authors: Messer, Ricka Knupp, Kelly G. Tags: Review Article Source Type: research

Neuro-Otology
Semin Neurol 2020; 40: 003-004 DOI: 10.1055/s-0040-1701514 Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Neurology)
Source: Seminars in Neurology - March 4, 2020 Category: Neurology Authors: Fife, Terry D. Tags: Preface Source Type: research

Terry D. Fife, MD, FAAN, FANS
Semin Neurol 2020; 40: 001-002 DOI: 10.1055/s-0040-1701517 Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Neurology)
Source: Seminars in Neurology - March 4, 2020 Category: Neurology Authors: Greer, David M. Tags: Introduction to the Guest Editor Source Type: research

Review on the Diagnosis and Treatment of Reversible Cerebral Vasoconstriction Syndrome in Children and Adolescents
Semin Neurol DOI: 10.1055/s-0040-1702942Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical–radiologic diagnosis that affects children and adolescents, but it is much more frequently reported in adults. Clinically, patients present with severe and commonly recurrent thunderclap headaches. Typical precipitating triggers include vasoactive substances, serotonergic agents, and the postpartum period. There may be associated neurologic complications at presentation or in the weeks following, such as convexity subarachnoid hemorrhage, stroke, cerebral edema, cervical artery dissection (CeAD), and seizures. A...
Source: Seminars in Neurology - February 20, 2020 Category: Neurology Authors: Qubty, William Irwin, Samantha Lee Fox, Christine K. Tags: Review Article Source Type: research

Predictors of Long-Term Neurodevelopmental Outcome of Hypoxic-Ischemic Encephalopathy Treated with Therapeutic Hypothermia
Semin Neurol DOI: 10.1055/s-0040-1702939Hypoxic-ischemic encephalopathy (HIE) is a manifestation of perinatal asphyxial insult that continues to evolve over days to weeks following the initial injury. Therapeutic hypothermia has demonstrated that a proportion of this secondary brain injury may indeed be preventable. However, therapeutic hypothermia has also altered the prognostic utility of many bedside tools that are commonly used as predictors of long-term neurodevelopmental outcome in HIE. Clinicians are often confronted with uncertainty when assessing the prognosis of infants with HIE. Improved understanding of the imp...
Source: Seminars in Neurology - February 20, 2020 Category: Neurology Authors: Goswami, Ipsita Guillot, Mireille Tam, Emily W. Y. Tags: Review Article Source Type: research

Treatable Movement Disorders of Infancy and Early Childhood
Semin Neurol DOI: 10.1055/s-0040-1702938Movement disorders in childhood can be difficult to diagnose early. Disease processes present variably and can mimic each other. It is particularly important to remain vigilant for the subset of these movement disorders that are treatable. These disorders can be managed with (1) treatments specific to the disease that substantially reduce symptoms; (2) treatments that can prevent progression; (3) treatments that can hasten recovery; or (4) surveillance and management of the associated, sometimes life-threatening, comorbidities. Here, we present a practical and phenomenology-oriented ...
Source: Seminars in Neurology - February 20, 2020 Category: Neurology Authors: Aravamuthan, Bhooma R. Pearson, Toni S. Tags: Review Article Source Type: research

Visual Vertigo, Motion Sickness, and Disorientation in Vehicles
This article discusses the putative mechanisms, consequences and approach to managing patients with visual vertigo, motion sickness, and motorist's disorientation syndrome in the context of chronic dizziness and motion sensitivity. [...] Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals: Table of contents  |  Abstract  |  Full text (Source: Seminars in Neurology)
Source: Seminars in Neurology - February 11, 2020 Category: Neurology Authors: Bronstein, A.M. Golding, J.F. Gresty, M.A. Tags: Review Article Source Type: research

Acute Vestibular Syndrome
Semin Neurol DOI: 10.1055/s-0039-3402739The acute vestibular syndrome (AVS) is a label for presentations of new-onset severe dizziness, vertigo, or imbalance, with examination findings of nystagmus or gait unsteadiness. The prototypical AVS presentation is the acute unilateral vestibulopathy due to vestibular neuritis. Stroke is also a serious concern in patients with AVS. Most other peripheral vestibular disorders present as episodic or chronic syndromes. In this article, the diagnostic considerations, exam findings, and management of AVS are reviewed. [...] Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001...
Source: Seminars in Neurology - January 28, 2020 Category: Neurology Authors: Kerber, Kevin A. Tags: Review Article Source Type: research

Superior Semicircular Canal Dehiscence Syndrome
Semin Neurol DOI: 10.1055/s-0039-3402738Superior canal dehiscence syndrome (SCDS) is a vestibular disorder caused by a pathologic third window into the labyrinth that can present with autophony, sound- or pressure-induced vertigo, and chronic disequilibrium among other vestibulocochlear symptoms. Careful history taking and examination in conjunction with appropriate diagnostic testing can accurately diagnose the syndrome. Key examination techniques include fixation-suppressed ocular motor examination investigating for sound- or pressure-induced eye movements in the plane of the semicircular canal. Audiometry, vestibular ev...
Source: Seminars in Neurology - January 27, 2020 Category: Neurology Authors: Steenerson, Kristen K. Crane, Benjamin T. Minor, Lloyd B. Tags: Review Article Source Type: research

Mal de Debarquement Syndrome
Semin Neurol DOI: 10.1055/s-0039-3402740Mal de debarquement syndrome (MdDS) is a disorder of persistent vertigo characterized by a feeling of oscillation such as rocking, bobbing, or swaying. It is triggered by passive motion, typically by exposure to water, air, or land transportation. This syndrome affects middle-aged individuals who are predominantly women. MdDS presents as a balance disorder that carries significant risk of morbidity due to both the direct effects of balance impairment and associated symptoms of fatigue, cognitive slowing, and visual motion intolerance. The Barany Society will be publishing criteria fo...
Source: Seminars in Neurology - January 27, 2020 Category: Neurology Authors: Saha, Kamala Cha, Yoon-Hee Tags: Review Article Source Type: research

Autoimmune Vestibulocerebellar Syndromes
This article describes the epidemiology, clinical presentation, diagnostic considerations, imaging findings, treatment, and prognosis of autoimmune disorders, in which the vestibulocerebellar syndrome is the main or presenting clinical presentation. Antibodies specific for intracellular antigenic targets described in the article are PCA-1 (Purkinje cell cytoplasmic antibody type 1, also known as anti-Yo), ANNA-1 (antinuclear neuronal antibody type 1, also known as anti-Hu), ANNA-2 (antinuclear neuronal antibody type 2, also known as anti-Ri), Ma1/2 (anti-Kelch-like 11/12 antibody), Kelch-like 11, amphiphysin, CV2 (collapsi...
Source: Seminars in Neurology - January 20, 2020 Category: Neurology Authors: Narayan, Ram N. McKeon, Andrew Fife, Terry D. Tags: Review Article Source Type: research

Vestibular-Evoked Myogenic Potential Testing in Vestibular Localization and Diagnosis
Semin Neurol DOI: 10.1055/s-0039-3402068Vestibular-evoked myogenic potentials (VEMPs) are short-latency, otolith-dependent reflexes recorded from the neck and eye muscles. They are widely used in neuro-otology clinics as tests of otolith function. Cervical VEMPs are recorded from the neck muscles and reflect predominantly saccular function, while ocular VEMPs are reflexes of the extraocular muscles and reflect utricular function. They have an important role in the diagnosis of superior canal dehiscence syndrome and provide complementary information about otolith function that is useful in the diagnosis of other vestibular ...
Source: Seminars in Neurology - January 14, 2020 Category: Neurology Authors: Taylor, Rachael L. Welgampola, Miriam S. Nham, Benjamin Rosengren, Sally M. Tags: Review Article Source Type: research

Persistent Postural-Perceptual Dizziness
Semin Neurol DOI: 10.1055/s-0039-3402736Persistent postural-perceptual dizziness (PPPD) was defined for the International Classification of Vestibular Disorders in 2017. It is a chronic vestibular disorder that manifests with waxing and waning symptoms of dizziness, unsteadiness, or nonspinning vertigo that last for 3 months or more and are exacerbated by upright posture, active or passive motion of self, and exposure to environments with complex or moving visual stimuli. Triggers of PPPD include a wide variety of conditions that may cause vestibular symptoms or disrupt balance functioning, including neuro-otologic and oth...
Source: Seminars in Neurology - January 14, 2020 Category: Neurology Authors: Staab, Jeffrey P. Tags: Review Article Source Type: research

Benign Paroxysmal Positional Vertigo: What We Do and Do Not Know
Semin Neurol DOI: 10.1055/s-0039-3402733Benign paroxysmal positional vertigo (BPPV) is common, sometimes terrifying, but rarely portends serious disease. It is usually easily diagnosed and treated, and both the patient and the physician are immediately gratified. While much has been learned about the pathogenesis of BPPV in the past decades, many of its features remain mysterious, and one must still be wary of the rare times it mimics a dangerous brain disorder. Here we review common, relatively well understood clinical features of BPPV but also emphasize what we do not know and when the physician must look deeper for a mo...
Source: Seminars in Neurology - January 14, 2020 Category: Neurology Authors: Nuti, Daniele Zee, David S. Mandal à, Marco Tags: Review Article Source Type: research

Bilateral Vestibular Dysfunction
Semin Neurol DOI: 10.1055/s-0039-3402066Bilateral vestibular dysfunction (BVD) refers to hypofunction of the vestibular nerves or labyrinths on both sides. Patients with BVD present with dizziness, oscillopsia, and unsteadiness, mostly during locomotion, which worsen in darkness or on uneven ground. Although aminoglycoside ototoxicity, Meniere's disease, infection, and genetic disorders frequently cause BVD, the etiology remains undetermined in up to 50% of the patients. The diagnosis of BVD requires both symptoms and documentation of deficient vestibulo-ocular reflex function using head-impulse, bithermal caloric, and rot...
Source: Seminars in Neurology - January 14, 2020 Category: Neurology Authors: Lee, Sun-Uk Kim, Hyo-Jung Kim, Ji-Soo Tags: Review Article Source Type: research

Vertigo Due to Vascular Mechanisms
Semin Neurol DOI: 10.1055/s-0039-3402737Isolated dizziness and vertigo due to vascular mechanisms are frequently misdiagnosed as peripheral vestibulopathy or vestibular migraine. For diagnosis of strokes presenting with an acute prolonged (≥ 24 hours) vestibular syndrome, findings on clinical examination, such as HINTS (negative head impulse tests, detection of direction-changing gaze-evoked nystagmus, and presence of skew deviation), are more sensitive than findings on neuroimaging. Since HINTS alone cannot securely detect anterior inferior cerebellar artery strokes, additional attention should be paid to the pa...
Source: Seminars in Neurology - January 14, 2020 Category: Neurology Authors: Kim, Hyun Ah Lee, Hyung Kim, Ji-Soo Tags: Review Article Source Type: research

Video Head Impulse Testing: From Bench to Bedside
Semin Neurol DOI: 10.1055/s-0039-3402063Over 30 years ago, the head impulse test (HIT) was measured with search coil recordings and it provided robust evidence for a new test of vestibular function that could detect impairment of a single semicircular canal, that is, the lateral canal. Over the next two decades, the diagnostic spectrum of HIT was expanded to the testing of vertical canals, differentiation of central from peripheral vestibulopathy, and incorporation of visual interaction - the suppressed head impulse. However, HIT measurement was limited to very few specialized laboratories that were able to maintain the ti...
Source: Seminars in Neurology - January 14, 2020 Category: Neurology Authors: Chen, Luke Halmagyi, G. Michael Tags: Review Article Source Type: research

Vestibular Migraine I: Mechanisms, Diagnosis, and Clinical Features
Semin Neurol DOI: 10.1055/s-0039-3402735Vestibular migraine (VM), also known as migrainous vertigo or migraine-associated vertigo, is characterized by recurrent vestibular attacks often accompanied by migraine headaches and other migraine symptoms. It is one of the most common presenting complaints to physicians in primary care, otolaryngology, and neurology. Epidemiologic data suggest that VM may affect 1 to 3% of the general population and 10 to 30% of patients seeking treatment for dizziness. Attacks typically last minutes to hours and range from spontaneous and positional vertigo to extreme sensitivity to self and surr...
Source: Seminars in Neurology - January 14, 2020 Category: Neurology Authors: Baloh, Robert W. Tags: Review Article Source Type: research

Cerebellar Dizziness and Vertigo: Etiologies, Diagnostic Assessment, and Treatment
Semin Neurol DOI: 10.1055/s-0039-3400315Cerebellar dizziness and vertigo account for approximately 10% of diagnoses in a tertiary dizziness center. This term summarizes a large group of disorders with chronic (degenerative, hereditary, acquired cerebellar ataxias), recurrent (episodic ataxias), or acute (stroke, inflammation) presentations. Key to the diagnosis is a comprehensive examination of central ocular motor and vestibular function. Patients with cerebellar dizziness and vertigo usually show a pattern of deficits in smooth pursuit, gaze-holding, saccade accuracy, or fixation-suppression of the vestibulo-ocular refle...
Source: Seminars in Neurology - December 30, 2019 Category: Neurology Authors: Zwergal, Andreas Feil, Katharina Schniepp, Roman Strupp, Michael Tags: Review Article Source Type: research

Vestibular Rehabilitation and Factors That Can Affect Outcome
Semin Neurol DOI: 10.1055/s-0039-3402062This review will discuss the developmental, environmental, medical, psychological, visual, and other sensory-related factors that affect recovery after vestibular dysfunction. A general overview of the evidence for vestibular rehabilitation for patients with peripheral and central vestibular disorders is provided. Recent findings suggest that age, physical activity, certain congenital disorders, length of symptoms, musculoskeletal, visual and neuromuscular comorbidities, cognition, sleep, and medications are all factors that influence the effectiveness and outcome of vestibular rehab...
Source: Seminars in Neurology - December 30, 2019 Category: Neurology Authors: Whitney, Susan L. Sparto, Patrick J. Furman, Joseph M. Tags: Review Article Source Type: research

Vestibular Migraine: Treatment and Prognosis
Semin Neurol DOI: 10.1055/s-0039-3402067Treatment of vestibular migraine currently lacks a firm scientific basis, as high quality randomized controlled trials are not available. Therefore, recommendations are largely borrowed from the migraine sphere. The first therapeutic step is explanation and reassurance. Many patients do not need pharmacological treatment, as attacks may be infrequent and tolerable. Acute attacks can be ameliorated in some patients with antiemetic drugs such as diphenhydramine, meclizine, and metoclopramide. Frequent attacks may warrant pharmacological prophylaxis with metoprolol, amitriptyline, topir...
Source: Seminars in Neurology - December 30, 2019 Category: Neurology Authors: von Brevern, Michael Lempert, Thomas Tags: Review Article Source Type: research

Current Understanding and Clinical Management of Meniere's Disease: A Systematic Review
Semin Neurol DOI: 10.1055/s-0039-3402065Meniere's disease (MD) is a set of uncommon disorders with core phenotype of tinnitus, episodic vertigo, and sensorineural hearing loss. MD shows a genetic predisposition and a family history is found in 10% cases, with an autosomal dominant inheritance pattern. It is a multifactorial condition whose onset and development are triggered by the combined effect of genetic and environmental factors. Histopathological studies have associated MD with the accumulation of endolymph in the cochlea and the vestibular organs. However, endolymphatic hydrops does not fully explain the persistence...
Source: Seminars in Neurology - December 30, 2019 Category: Neurology Authors: Perez-Carpena, Patricia Lopez-Escamez, Jose A. Tags: Review Article Source Type: research

The Evidence for Selective Loss of Otolithic Function
Semin Neurol DOI: 10.1055/s-0039-3402064Recent advances in vestibular testing now permit functional testing of all peripheral vestibular sense organs (all three semicircular canals, utricle, and saccule). This makes it possible to identify patients with isolated dysfunction of the utricle or saccule, even though parallel pathways for vestibular information are ultimately integrated centrally. Selective, isolated unilateral loss of utricular function as measured by ocular vestibular-evoked myogenic potentials (VEMPs) has been observed in patients with normal semicircular canal function as measured by the video head impulse ...
Source: Seminars in Neurology - December 30, 2019 Category: Neurology Authors: Curthoys, Ian S. Burgess, Ann M. Manzari, Leonardo Tags: Review Article Source Type: research

Eye Movement Recordings: Practical Applications in Neurology
Semin Neurol 2019; 39: 775-784 DOI: 10.1055/s-0039-1698742Accurate detection and interpretation of eye movement abnormalities often guides differential diagnosis, discussions on prognosis and disease mechanisms, and directed treatment of disabling visual symptoms and signs. A comprehensive clinical eye movement examination is high yield from a diagnostic standpoint; however, skillful recording and quantification of eye movements can increase detection of subclinical deficits, confirm clinical suspicions, guide therapeutics, and generate expansive research opportunities. This review encompasses an overview of the clinical e...
Source: Seminars in Neurology - December 17, 2019 Category: Neurology Authors: Rizzo, John-Ross Beheshti, Mahya Dai, Weiwei Rucker, Janet C. Tags: Review Article Source Type: research