american neurology academy

OBJECTIVE: This article reviews the clinical spectrum of amyotrophic lateral sclerosis (ALS), its variant presentations, and the approach to diagnosis and management. This review includes a detailed discussion of current and emerging disease-modifying therapies and the management of respiratory and bulbar manifestations of disease. An updated review of ALS genetics and pathophysiology is also provided. This article also touches on several other important motor neuron diseases. LATEST DEVELOPMENTS: A new set of simplified diagnostic criteria may help identify patients at earlier stages of the disease...

The American Academy of Pediatrics coauthored and endorsed the following publication: Greer DM, Kirschen MP, Lewis A, et al. Pediatric and Adult Brain Death/Death by Neurologic Criteria Consensus Guideline. Report of the AAN Guidelines Subcommittee, AAP, CNS, and SCCM. Neurology. Published online October 2023. DOI: https://doi.org/10.1212/WNL.0000000000207740 All statements of endorsement automatically expire 5 years after publication unless reaffirmed, revised, or retired at or before that time.

In collaboration with the American Academy of Pediatrics, Child Neurology Society, and Society for Critical Care Medicine, the American Academy of Neurology formulated an updated, evidence-informed consensus-based guideline for pediatric and adult brain death/death by neurologic criteria (BD/DNC) determination. In comparison with the prior guidelines, the revisions and additions in this guideline, which are summarized in this review, are intended to (1) ensure recommendations are conservative, yet practical, and emphasize circumstances in which BD/DNC determination should be delayed or deferred, so as to minimize the risk of a false-positive BD/DNC determination; and (2) provide guidance about aspects of BD/DNC determination that clinicians find challenging and/or controversial...

STUDY OBJECTIVES: Accurate diagnosis of isolated rapid eye movement (REM) sleep behavior disorder (iRBD) is crucial due to its injury potential and neurological prognosis. We aimed to analyze visual and automated REM sleep without atonia (RSWA) diagnostic thresholds applicable in varying clinical presentations in a contemporary cohort of iRBD patients using submentalis (SM) and individual bilateral flexor digitorum superficialis (FDS) and anterior tibialis (AT) electromyography limb recordings during polysomnography...

BACKGROUND AND OBJECTIVES: The purpose of this guideline is to update the 2010 American Academy of Neurology (AAN) brain death/death by neurologic criteria (BD/DNC) guideline for adults and the 2011 American Academy of Pediatrics, Child Neurology Society, and Society of Critical Care Medicine guideline for infants and children and to clarify the BD/DNC determination process by integrating guidance for adults and children into a single guideline. Updates in this guideline include guidance related to conducting the BD/DNC evaluation in the context of extracorporeal membrane oxygenation, targeted temperature management, and primary infratentorial injury...

STUDY OBJECTIVES: McLeod syndrome (MLS) is a very rare multisystemic neurodegenerative disease linked to mutations in the XK gene. It has cardiac, neurologic, and neuromuscular manifestations and shares similarities with Huntington's disease. The aim of this study was to evaluate sleep patterns of patients affected by MLS. METHODS: This retrospective case series of 4 males who underwent diagnostic polysomnography (mean age 53.8 ± 2.5 y) includes subjective and objective evaluation of sleep using the Epworth Sleepiness Scale (ESS), genetic tests, documentation of clinical course and features, and laboratory-based full-night attended video-polysomnography...

OBJECTIVE: This preliminary study examines the effects of bilingualism on a modified confrontational naming task (CNT) in native and non-native English speakers. The broad objective was to explore specific aspects of bilingualism that affected performance, to better inform good clinical practice in neuropsychology for linguistically diverse populations. A-priori hypotheses included: 1) Age of English acquisition (AOA) predicts CNT performance; 2) There would be a significant group diff between native and non-native English speakers; and 3) There would be significant group differences between multilingual and bilinguals on CNT performance...

OBJECTIVE: To demonstrate the feasibility of cognitive and psychological characterization after stroke during post-discharge neurology visit as part of standard care. METHOD: From January 1, to April 29, 2023, 33 patients were evaluated using the MoCA and screening tests for aphasia, spatial neglect, depression, and anxiety during their neurology outpatient visit. Neuropsychological measures evaluating attention, processing speed, language, visuospatial, memory, and executive function abilities were also administered...

BACKGROUND AND OBJECTIVES: Global developmental delay/intellectual disability (GDD/ID) are among the most common neurologic conditions evaluated by child neurologists in the United States. No recent neurology-specific guidelines for GDD/ID diagnostic evaluation exist, which could lead to practice variability. We assessed current practices in GDD/ID diagnostic evaluation among US child neurologists, including drivers of exome sequencing (ES). METHODS: A 19-item online anonymous survey was distributed between April 2021 and September 2021 to 953 eligible child neurologists by email and/or online platforms through the American Academy of Neurology and Child Neurology Society...

Although research studies have begun to demonstrate relationships between disorders of consciousness and brain network biomarkers, there are limited data on the practical aspects of obtaining such network biomarkers to potentially guide care. As the state of knowledge continues to evolve, guidelines from professional societies such as the American and European Academies of Neurology and many experts have advocated that the risk-benefit ratio for the assessment of network biomarkers has begun to favor their application toward potentially detecting covert consciousness...

BACKGROUND AND OBJECTIVES: Impulse control disorders (ICD) are a group of behaviors in Parkinson disease (PD), (compulsive buying, gambling, binge eating, craving sweets, and hypersexuality) that occur in up to 20% of individuals with PD, sometimes with devastating results. We sought to determine the rate of ICD screening based on 2020 quality measures for PD care by the American Academy of Neurology. METHODS: We conducted a quality improvement project to document and improve physician ICD screening in a tertiary movement disorder program...

BACKGROUND: First-time seizures comprise 15% to 35% of all seizure-related complaints in the emergency department (ED). Emergent neuroimaging of first-time seizure patients is recommended by both the American Academy of Neurology and American College of Emergency Physicians. However, most of these studies are over 10 years old and included both adults and children. OBJECTIVES: The goal of this investigation is to determine the rate of abnormal neuroimaging in adults with first-time seizures presenting to the ED during the current era...

In this brief case report on paroxysmal sleep-related movements, we describe an adolescent patient's presentation of brief jerking movements during sleep and the accompanying differential diagnosis. In examining the patient's overnight electroencephalogram (EEG), we utilize hallmark sleep architecture to provide reassurance to the patient and her family.

STUDY OBJECTIVES: Behavioral insomnia of childhood (BIC) and obstructive sleep apnea (OSA) are highly prevalent conditions affecting 10-20% and 1-5% of children respectively. Studies in adults and adolescents have suggested that comorbid insomnia and OSA (COMISA) may have distinct clinical characteristics. The association between the two conditions in the pediatric population has not been thoroughly investigated. This study aimed to examine the association between BIC and OSA in young children...

Diagnostic, prognostic, and therapeutic procedures for patients with prolonged disorders of consciousness (pDoCs) vary significantly across countries and clinical settings, likely due to organizational factors (e.g., research vs. non-academic hospitals), expertise and availability of resources (e.g., financial and human). Two international guidelines, one from the European Academy of Neurology (EAN) and one from the American Academy of Neurology (AAN) in collaboration with the American Congress of Rehabilitation Medicine (ACRM) and the National Institute on Disability, Independent Living, and Rehabilitation Research (NIDILRR), were developed to facilitate consistent practice among professionals working with this challenging patient population...

PURPOSE: To assess the demographic characteristics and geographic distribution of neuro-ophthalmologists practicing in the United States. DESIGN: A cross-sectional study. PARTICIPANTS: Neuro-ophthalmologists across the United States. METHODS: In this cross-sectional study, public databases from the American Academy of Ophthalmology, North American Neuro-ophthalmology Society, American Neurological Association, and American Academy of Neurology were used to identify neuro-ophthalmologists in the United States as of April 2023...

OBJECTIVE: To assess the association between language proficiency and absolute dominance on language versus visual-perceptual formatted and executive versus delayed memory neuropsychological measures in bilingual adults. METHODS: Data from 55 bilingual, conversationally fluent, neurologically intact, Mexican American, consecutive, adults tested in separate sessions in Spanish and English in a counterbalanced order were analyzed. Age, years of education, self-reported language proficiency, Woodcock-Muñoz Language Survey-Revised (WMLS-R) picture vocabulary measures of language proficiency, and dominance (absolute Spanish-English WMLS-R difference scores) were correlated with 11 measures from La Batería Neuropsicólogica en Español and its original English language tests...

BACKGROUND: Optometrists are often the first providers to evaluate patients with acute vision loss and are often the first to diagnose a central retinal artery occlusion (CRAO). How quickly these patients present to the optometrist, are diagnosed, and referred for evaluation are major factors influencing the possibility of acute therapeutic intervention. Our aim was to survey the U.S. optometric community to determine current optometric practice patterns for management of CRAO. METHODS: An anonymous seven-question survey was emailed in 2020 to the 5,101 members of the American Academy of Optometry and the 26,502 members of the American Optometric Association...

Brain health is crucial to optimizing both the function and well-being of every person at each stage of life and is key to both individual and social progress. As a concept, brain health is complex and requires a multidisciplinary collaborative approach between many professional and public organizations to bring into effect meaningful change. Neurologists are uniquely positioned to serve as specialists in brain health and to advance the newly evolving field of preventive neurology, which aims to identify individuals at high risk of brain disorders and other neurologic conditions and offer strategies to mitigate disease emergence or progression...

After more than 2 decades of recommending an escalating strategy for the treatment of most patients with multiple sclerosis, there has recently been considerable interest in the use of high-efficacy therapies in the early stage of the disease. Early intervention with induction/high-efficacy disease-modifying therapy may have the best risk-benefit profile for patients with relapsing-remitting multiple sclerosis who are young and have active disease, numerous focal T2 lesions on spinal and brain magnetic resonance imaging, and no irreversible disability...