American Academy Of Neurology Research Articles

Quality measures for the care of adult patients with obstructive sleep apnea: 2022 update after measure maintenance.

Lloyd R, Morgenthaler TI, Donald R, etal. Quality measures for the care of adult patients with obstructive sleep apnea: 2022 update after measure maintenance. J Clin Sleep Med. 2022;18(11):2673-2680.

Acute Treatment of Headache (Focus on Migraine).

Acute treatments for migraine and cluster headache are necessary to abort attacks, relieve pain and associated symptoms, and restore an individual's ability to function. Acute headache treatments consist of a variety of medication and nonmedication options. In this article, we discuss the approach to acute treatment of migraine and cluster headache. We summarize the level of evidence to support each acute medication class according to recent systematic reviews and meta-analyses, as well as guideline recommendations from the American Headache Society, American Academy of Neurology, and European Federation of Neurological Society.

Viewpoint on Milestones for Fellowship Training in Movement Disorders.

Viewpoint on Milestones for Fellowship Training in Movement Disorders.

Calling All Experts: Broadening the Definition of Expert Peer Review

MODERATOR: Jonathan Schultz Director of Journal Operations American Heart Association SPEAKERS: Jonathan Schultz Patricia Baskin Executive Editor Neurology Journals, American Academy of Neurology H.E. Hinson, MD Associate Professor at Oregon Health and Science University Ashwini Sehgal, MD Professor at Case Western Reserve University REPORTER: Tony Alves Highwire Press For the session “Calling all Experts: Broadening the Definition of Expert Peer Review,” moderator Jonathan Schultz kicked off the session by noting that, although the traditional model of peer review is that an editor will send a manuscript to 2 or 3 reviewers who are experts on the topic of the manuscript, there is a growing recognition that sometimes a more specialized reviewer may need to be involved. In a presentation entitled “Technical/Methods/Reproducibility Review,” different forms of technical review were discussed. Because science articles are more complex, the American Heart Association (AHA) contracted with a team of technical editors who look at manuscripts at the revision stage and focus on the technical aspects of the manuscript like the abstract, methods, figures, and data—all elements that you need to understand reproducibility of the research. Three of the AHA’s technical reviewers answered questions posed by Schultz. They discussed that their role as a technical reviewer is to look at the content of the article to be sure that authors are providing necessary key components such as background and methods details. The best way to do that is to look at the reporting guidelines and to use standard reporting guideline checklists. It’s also important […]

Gender Representation Among Physician Authors of Practice Guidelines Developed, Endorsed, or Affirmed by the American Academy of Neurology.

To assess American Academy of Neurology (AAN)-recommended Practice Guidelines (PGs) for equity in gender representation among physician authors. This cross-sectional study included AAN-recommended PG publications from January 1, 2015, to December 31, 2020. Author degrees and gender were identified by 2 reviewers using the publication and/or online searches. Gender was determined from pronouns or photographs. Gender representation was compared with Association of American Medical Colleges (AAMC) data on academic neurologists. Data were analyzed using Z tests of 2 proportions and descriptive statistics. AAMC benchmarks report academic women neurologists represented 35% of the specialty in 2015, 38% in 2018, and 39% in 2020. We identified 68 unique PG publications with 709 physician authors, 31% (223) women, 68% (484) men, and 0.3% (2) gender could not be identified. Representation of women physicians was low among PG authors across all benchmarks, significantly so for 2018 and 2020 (p < 0.01). Among physician first authors, women were significantly underrepresented across all benchmarks (18% [12/65], p < 0.01). Representation of women physicians was lower when men physicians were first authors vs women physicians (31% [161/524] vs 43% [50/118], p = 0.02). Among subspecialties with 10+ PGs, women physician authorship was highest in child neurology (48% [57/120]) and lowest in stroke and vascular neurology (16% [18/113]). We found that women physicians were underrepresented as authors of AAN-recommended PGs. This suggests a missed opportunity for neurology because PGs that include expertise from women physicians may improve care and translation into practice. In addition, women physicians lose out on professional development from authorship. Further research is needed to understand causality and address gaps.

American Academy of Neurology (AAN) - 74th Annual Meeting: The Great Neuro Reunion. Seattle - April 2-7, 2022

American Academy of Neurology (AAN) - 74th Annual Meeting: The Great Neuro Reunion. Seattle - April 2-7, 2022

The Medicolegal and Ethical Dimensions of Physician Prescription Reluctance.

This article addresses the potential legal ramifications for neurologists caring for patients with Alzheimer disease (AD) who elect neither to prescribe aducanumab nor to refer patients with AD for treatment with aducanumab. To prevail against a neurologist for failing to prescribe aducanumab or refer for aducanumab treatment, the plaintiff would have to establish that the neurologist's failure to prescribe the medication or refer for treatment was a breach of the standard of care. The standard of care is conceptualized as the generally accepted approach to diagnosing or treating a condition. However, the controversy surrounding the US Food and Drug Administration's (FDA's) approval process for aducanumab (which was based on the drug's efficacy at reducing brain amyloidosis rather than on clinically meaningful efficacy) as well as the American Academy of Neurology (AAN) position statement on aducanumab and the recent decision by the Centers for Medicare & Medicaid Services (CMS) to limit Medicare coverage of the drug and its associated costs to patients enrolled in qualifying clinical trials indicate that aducanumab cannot yet be considered the standard of care for the treatment of AD. Although deciding not to prescribe aducanumab does not violate the standard of care, neurologists treating patients with AD and not recommending this treatment should explain to their patients and their patients' surrogate decision makers why they are not recommending the treatment.

Common risk variants for epilepsy are enriched in families previously targeted for rare monogenic variant discovery.

SummaryBackgroundThe epilepsies are highly heritable conditions that commonly follow complex inheritance. While monogenic causes have been identified in rare familial epilepsies, most familial epilepsies remain unsolved. We aimed to determine (1) whether common genetic variation contributes to familial epilepsy risk, and (2) whether that genetic risk is enriched in familial compared with non-familial (sporadic) epilepsies.MethodsUsing common variants derived from the largest epilepsy genome-wide association study, we calculated polygenic risk scores (PRS) for patients with familial epilepsy (n = 1,818 from 1,181 families), their unaffected relatives (n = 771), sporadic patients (n = 1,182), and population controls (n = 15,929). We also calculated separate PRS for genetic generalised epilepsy (GGE) and focal epilepsy. Statistical analyses used mixed-effects regression models to account for familial relatedness, sex, and ancestry.FindingsPatients with familial epilepsies had higher epilepsy PRS compared to population controls (OR 1·20, padj = 5×10−9), sporadic patients (OR 1·11, padj = 0.008), and their own unaffected relatives (OR 1·12, padj = 0.01). The top 1% of the PRS distribution was enriched 3.8-fold for individuals with familial epilepsy when compared to the lowest decile (padj = 5×10−11). Familial PRS enrichment was consistent across epilepsy type; overall, polygenic risk was greatest for the GGE clinical group. There was no significant PRS difference in familial cases with established rare variant genetic etiologies compared to unsolved familial cases.InterpretationThe aggregate effects of common genetic variants, measured as polygenic risk scores, play an important role in explaining why some families develop epilepsy, why specific family members are affected while their relatives are not, and why families manifest specific epilepsy types. Polygenic risk contributes to the complex inheritance of the epilepsies, including in individuals with a known genetic etiology.FundingNational Health and Medical Research Council of Australia, National Institutes of Health, American Academy of Neurology, Thomas B and Jeannette E Laws McCabe Fund, Mirowski Family Foundation.

Impact of Antiseizure Medications on Appetite and Weight in Children

There are numerous potential factors that may affect growth in children with epilepsy, and these must be evaluated in any child with appetite and weight concerns. Antiseizure medications (ASMs) have potential adverse effects, and many may affect appetite, thus impacting normal growth and weight gain. The aim of this review is to focus on the impact of both epilepsy and ASMs on appetite and weight in children. We systematically reviewed studies using Medline assessing the impact of ASMs on appetite and weight in children. Eligible studies included randomized controlled trials and open-label studies (open-label extension and interventional) that targeted or included the pediatric population (0-18 years of age). Each study was classified using the American Academy of Neurology (AAN) Classification of Evidence for Therapeutic Studies, and the level of evidence for impact on appetite and weight in children was graded. ASMs associated with decreased appetite and/or weight loss include fenfluramine, topiramate, zonisamide, felbamate, rufinamide, stiripentol, cannabidiol, brivaracetam and ethosuximide; ASMs with minimal impact on weight and appetite in children include oxcarbazepine, eslicarbazepine, lamotrigine, levetiracetam, lacosamide, carbamazepine, vigabatrin and clobazam. The ASM most robustly associated with increased appetite and/or weight gain is valproic acid; however, both pregabalin and perampanel may also lead to modest weight gain or increased appetite in children. Certain ASMs may impact both appetite and weight, which may lead to increased morbidity of the underlying disease and impaired adherence to the treatment regimen.

HIV Dementia: A Bibliometric Analysis and Brief Review of the Top 100 Cited Articles.

Dementia is a syndrome of cognitive impairment that affects an individual’s ability to live independently. The number of people living with dementia worldwide in 2015 was estimated at 47.47 million. The American Academy of Neurology (AAN) criteria for human immunodeficiency virus (HIV)-associated dementia (HAD) require an acquired abnormality in at least two cognitive (non-motor) domains and either an abnormality in motor function or specified neuropsychiatric/psychosocial domains. HIV is the most common cause of dementia below 60 years of age.Citation frequencies are commonly used to assess the scholarly impact of any scientific publication in bibliometric analyses. It helps depict areas of higher interest in terms of research frequency and trends of citations in the published literature and identify under-explored domains of any field, providing useful insight and guidance for future research avenues. We used the database “Web of Science” (WOS) to search for the top 100 cited articles on HIV-associated dementia. The keywords “HIV dementia” and “HIV-associated neurocognitive disorders” (HAND) were used. The list was generated by two authors after excluding articles not pertaining to HIV dementia. The articles were then assigned to authors to extract data to make tables and graphical representations. Finally, the manuscript was organized and written describing the findings of the bibliometric study.These 100 most cited articles on HIV dementia were published between years 1986 and 2016. The highest number of the articles was from 1999 (n=9). The year 1993-2007 contributed consistently two publications to the list. The articles are from 42 journals, and among them, the Annals of Neurology (n=16) and the Journal of Neurology (n=15) published most of the articles. Justin C. McArthur with 25 publications contributed the highest number of papers to the list by any author. The USA collaborated in the highest number of publications (n=87). American institutes were leading the list with the most publications. The Johns Hopkins University collaborated on 37 papers. The most widely studied aspect of HIV dementia was pathogenesis. Incidence and prevalence, clinical features, and pre- and post-highly active antiretroviral therapy (HAART) era were also discussed in the articles.Beyond America, the research should be expanded to low-income countries and those affected more by HIV. Therefore, other countries and their institutes should participate more in HIV-associated dementia research. Anticipating the rising resistance to existing antiretrovirals, we should develop new therapeutic options. There is room for research in many aspects of HIV dementia care.

Gene delivery &amp; gene editing platform evolution

Dr Rodino-Klapac is a gene therapy pioneer who has dedicated her professional life to advancing medicines designed to treat genetically based diseases. With professional experience across industry and academia, she is renowned for her contributions to molecular genetics and gene therapy that have advanced the field. She is author to a vast body of published, peer-reviewed work, the recipient of multiple awards, a National Institutes of Health (NIH) Fellow appointee, and current Board member of the Association for Regenerative Medicine, as well as a member of the American Society for Gene and Cell Therapy, the American Academy of Neurology, and the American Association for the Advancement of Science. She is the former head of the Laboratory for Gene Therapy Research at Nationwide Children’s Hospital, established the Gene Therapy Center of Excellence within Sarepta and leads the Company’s Gene Editing Innovation Center, directing a team of researchers to discover and develop novel gene replacement and gene editing therapies. She co-founded and served as chief scientific officer of Myonexus Therapeutics, a gene therapy company focused on limb-girdle muscular dystrophies acquired by Sarepta in 2019. She currently serves as Sarepta’s Executive Vice President, Chief Scientific Officer. Her work has led to 11 investigational new drug applications and she is the co-inventor of SRP-9001, an investigational micro-dystrophin gene therapy, and the inventor of five investigational gene therapies for limb-girdle muscular dystrophy. She is the inventor of over 50 US and over 70 international published patent applications. She earned her PhD in molecular genetics form the Ohio State University and graduated summa cum laude from Kings College in Wilkes-Barre, Pennsylvania, with a Bachelor of Science degree in Biology.

2022 Guideline for the Management of Patients With Spontaneous Intracerebral Hemorrhage: A Guideline From the American Heart Association/American Stroke Association.

2022 Guideline for the Management of Patients With Spontaneous Intracerebral Hemorrhage: A Guideline From the American Heart Association/American Stroke Association.

The Legacy of the TTASAAN Report - Premature Conclusions and Forgotten Promises About SPECT Neuroimaging: A Review of Policy and Practice Part II.

Brain perfusion single photon emission computed tomography (SPECT) scans were initially developed in 1970s. A key radiopharmaceutical, hexamethylpropyleneamine oxime (HMPAO), was not stabilized until 1993 and most early SPECT scans were performed on single-head gamma cameras. These early scans were of inferior quality. In 1996, the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology (TTASAAN) issued a report regarding the use of SPECT in the evaluation of neurological disorders. This two-part series explores the policies and procedures related to perfusion SPECT functional neuroimaging. In Part I, the comparison between the quality of the SPECT scans and the depth of the data for key neurological and psychiatric indications at the time of the TTASAAN report vs. the intervening 25 years were presented. In Part II, the technical aspects of perfusion SPECT neuroimaging and image processing will be explored. The role of color scales will be reviewed and the process of interpreting a SPECT scan will be presented. Interpretation of a functional brain scans requires not only anatomical knowledge, but also technical understanding on correctly performing a scan, regardless of the scanning modality. Awareness of technical limitations allows the clinician to properly interpret a functional brain scan. With this foundation, four scenarios in which perfusion SPECT neuroimaging, together with other imaging modalities and testing, lead to a narrowing of the differential diagnoses and better treatment. Lastly, recommendations for the revision of current policies and practices are made.

Scleritis in Lyme Disease

To estimate the incidence of scleritis in Lyme disease and report clinical features. Incidence rate estimate and case series. Data were collected from an electronic medical record on patients with scleritis presenting to the Wilmer Eye Institute between January 1, 2012 and December 31, 2020. A diagnosis of Lyme disease was made using the Infectious Diseases Society of America, American Academy of Neurology, and the American College of Rheumatology 2020 joint criteria plus a response to antibiotic therapy. After identifying all new-onset cases of scleritis in the database, the proportion of new-onset scleritis with Lyme disease was calculated. The proportion of Lyme disease cases with scleritis was estimated using the number of cases with Lyme disease from the Baltimore metropolitan area reported to the Centers for Disease Control and Prevention. After querying other major eye centers in the area for any cases of Lyme disease scleritis, none were identified, and the incidence of Lyme disease scleritis was estimated using published U.S. Census data for the greater Baltimore metropolitan area. Six cases of Lyme disease scleritis were identified in the 8-year time frame; 1 additional case was identified in the following year. Lyme disease scleritis accounted for 0.6% of all cases of scleritis, and 0.052% of patients with Lyme disease had scleritis. The estimated incidence of Lyme scleritis was 0.2 per 1,000,000 population per year (95% confidence interval 0-0.4), whereas the estimated incidence of Lyme disease in the area was 3 per 10,000 population per year (95% confidence interval 2.9-3.1). All scleritis cases were anterior, unilateral, without necrosis, and resolved with antibiotic use without relapse in a median of 39.5 days (range 29-57 days). Other features of Lyme disease were present in 4 of 7 patients, including a history of erythema migrans in 2 of 7 patients. Lyme disease is an uncommon cause of scleritis in endemic areas.

American and European Guidelines on Disorders of Consciousness: Ethical Challenges of Implementation.

American and European Guidelines on Disorders of Consciousness: Ethical Challenges of Implementation.

The Neurology Fellowship Application Conundrum: Finding Common Ground.

For many neurologic subspecialties, the fellowship application process begins early in the first half of the second year of neurology-specific training (PGY3 for adult neurology residents and PGY4 for child neurology residents). In 2019, the American Academy of Neurology (AAN) published a position statement recommending communication between fellowship candidates and training programs begin no sooner than March 1 of the penultimate year of training and that programs offer fellowship positions no sooner than August 1 of the final year of training. A few pilot subspecialties adopted this timeline for 2021 recruitment for positions beginning in 2022. All United States-based AAN-affiliated neurology and child neurology residents who recently completed the fellowship application process received a survey about their fellowship application experience. Of the 291 residents who responded to the survey, 96% agree that applications should not be submitted before March 1 of the penultimate year of training and 72% believe that August 1 of the final year is a reasonable time to begin offering positions. Nearly half (49%) of residents believe that there is too little time for subspecialty/clinical exposure before applying for fellowship and 88% feel the current process and timeline are stressful. Residents who applied to programs in pilot subspecialties report more time to choose, less stress, and a lower number of time-pressured offers. A large majority of residents (89%) prefer to submit a single application through a centralized system. The survey results suggest that residents who just completed the fellowship application process agree with the AAN recommended timeline for all subspecialties and that all neurology subspecialties should consider adopting a fellowship match. Programs can help facilitate a better fellowship application experience by providing earlier exposure to a broad range of neurologic subspecialties and ensure that residents are given opportunities to attend national meetings and participate in appropriately scoped scholarly endeavors.

AAN releases updated guideline for management of diabetic neuropathy

New evidence supporting the use of select oral and topical agents for the management of painful diabetic neuropathy is discussed in an updated guideline released in December 2021 by the American Academy of Neurology (AAN).

Pregabalina vs gabapentyna w leczeniu bólu neuropatycznego u pacjentów onkologicznych

Neuropathic pain affects an average of 7–10% of the population and is more often in elderly people and in women. It is a chronic pathological pain, resistant to analgesics from the first step of the analgesic ladder (paracetamol, metamizole, non-steroidal anti-inflammatory drugs). Neuropathic pain may be caused by: infectious diseases, trauma, ischemia, metabolic diseases, neurological diseases, toxic, consequences of damage to the nervous system resulting from the treatment of neoplastic disease, pressure, inflammation, as well as deficiencies. The American Academy of Neurology, Special Interest Group on Neuropathic Pain, and the European Federation of Neurological Societies recommend gabapentin/pregabalin and antidepressants as first choice in the treatment of neuropathic pain. In neuropathic pain accompanying neoplastic disease, due to its mixed nature, i.e., the coexistence of receptor pain, opioid analgesics are always used in the treatment: tramadol for moderate pain and strong opioids for high pain. The following article describes the differences in the pharmacokinetics and pharmacodynamics of gabapentin and pregabalin.

Intravenous Brivaracetam in the Management of Acute Seizures in the Hospital Setting: A Scoping Review.

BackgroundClinical considerations for drug treatment of acute seizures involve variables such as safety, tolerability, drug-drug interactions, dosage, route of administration, and alterations in pharmacokinetics because of critical illness. Therapy options that are easily and quickly administered without dilution, well tolerated, and effective are needed for the treatment of acute seizures. The objective of this review is to focus on the clinical considerations relating to the use of intravenous brivaracetam (IV BRV) for the treatment of acute seizures in the hospital, focusing on critically ill patients.MethodsThis was a scoping literature review of PubMed from inception to April 13, 2021, and search of the American Academy of Neurology (AAN) 2021 Annual Meeting website for English language publications/conference abstracts reporting the results of IV BRV use in hospitalized patients, particularly in the critical care setting. Outcomes of interest relating to the clinical pharmacology, safety, tolerability, efficacy, and effectiveness of IV BRV were reviewed and are discussed.ResultsTwelve studies were included for analysis. One study showed that plasma concentrations of IV BRV 15 min after the first dose were similar between patients receiving IV BRV as bolus or infusion. IV BRV was generally well tolerated in patients with acute seizures in the hospital setting, with a low incidence of individual TEAEs classified as behavioral disorders. IV BRV demonstrated efficacy and effectiveness and had a rapid onset, with clinical and electrophysiological improvement in seizures observed within minutes. Although outside of the approved label, findings from several studies suggest that IV BRV reduces seizures and is generally well tolerated in patients with status epilepticus.ConclusionsIV BRV shows effectiveness, and is generally well tolerated in the management of acute seizures in hospitalized patients where rapid administration is needed, representing a clinically relevant antiseizure medication for potential use in the critical care setting.

Cannabis-based medicine in treatment of patients with Gilles de la Tourette syndrome.

Gilles de la Tourette syndrome (GTS) is a childhood onset disorder characterised by the presence of motor and vocal tics. The guidelines of both the American Academy of Neurology (AAN) as well as the European Society for the Study of Tourette Syndrome (ESSTS) recommend behavioural therapy and pharmacotherapy, mainly with antipsychotics, as first line treatments for tics. In spite of these well-established therapeutic approaches, a significant number of patients are dissatisfied because of insufficient tic reduction or intolerable side effects. Previous studies have suggested that cannabis-based medicine (CBM) might be an alternative treatment in these patients. Two reviewers (KS, NS) searched the electronic database of PubMed on 1 July, 2021 for relevant studies using the search terms: ('Tourette syndrome' [MeSH Terms] OR 'Gilles de la Tourette syndrome' [MeSH Terms] OR 'tic disorders' [MeSH Terms] OR 'tics' [MeSH Terms] OR 'tic disorders'[Title/Abstract]) AND ('cannabis-based medicine' [Title/Abstract] OR 'cannabis' [Title/Abstract] OR 'dronabinol' [Title/Abstract] OR 'nabiximols' [Title/Abstract] OR 'tetrahydrocannabinol' [Title/Abstract] OR 'THC' [Title/Abstract] OR 'cannabidiol' [Title/Abstract], limit: 'humans'. These studies were further reviewed for additional relevant citations. The titles and abstracts of the studies obtained through this search were examined by two reviewers (KS, NS) in order to determine article inclusion. Discrepancies were addressed by the reviewers through discussion and eventually conversation with the senior reviewer (KMV). Although the amount of evidence supporting the use of CBM in GTS is growing, the majority of studies are still limited to case reports, case series, and open uncontrolled studies. To date, only two small randomised controlled trials (RCTs) using tetrahydrocannabinol (THC, dronabinol) have been published demonstrating the safety and efficacy of this intervention in the treatment of tics in patients with GTS. On the other hand, another RCT with Lu AG06466 (formerly known as ABX-1431), a modulator of endocannabinoid neurotransmission, has failed to prove effective in the therapy of GTS. Accordingly, under the guidelines of both the ESSTS and the AAN, treatment with CBM is categorised as an experimental intervention that should be applied to patients who are otherwise treatment-resistant. Increasing evidence suggests that CBM is efficacious in the treatment of tics and psychiatric comorbidities in patients with GTS. The results of ongoing larger RCTs, such as CANNA-TICS (ClinicalTrials.gov Identifier: NCT03087201), will further clarify the role of CBM in the treatment of patients with GTS.