ABSTRACT
BACKGROUND: Isolated psychiatric symptoms can be the initial symptom of pediatric anti-N-methyl-d-aspartate (NMDA) receptor autoimmune encephalitis (pNMDARE). Here we report on the prevalence of isolated psychiatric symptoms in pNMDARE. We also assess whether initial neurodiagnostic tests (brain magnetic resonance imaging [MRI], electroencephalography [EEG], and/or cerebrospinal fluid [CSF] white blood cell count) are abnormal in children with isolated psychiatric symptoms and pNMDARE. METHODS: This multicenter retrospective cohort study from CONNECT (Conquering Neuroinflammation and Epilepsies Consortium) from 14 institutions included children under age 18 years who were diagnosed with pNMDARE. Descriptive statistics using means, medians, and comparisons for continuous versus discrete data was performed. RESULTS: Of 249 children included, 12 (5%) had only psychiatric symptoms without other typical clinical features of autoimmune encephalitis at presentation. All but one (11 of 12 = 92%) had at least one abnormal finding on initial ancillary testing: eight of 12 (67%) had an abnormal EEG, six of 12 (50%) had an abnormal MRI, and five of 12 (42%) demonstrated CSF pleocytosis. The single patient with a normal MRI, EEG, and CSF profile had low positive CSF NMDA antibody (titer of 1:1), and symptoms improved without immunotherapy. CONCLUSIONS: Isolated first-episode psychiatric symptoms in pNMDARE are uncommon, and the majority of children will exhibit additional neurodiagnostic abnormalities. Delaying immunotherapy in a child with isolated psychiatric symptoms and normal neurodiagnostic testing may be warranted while awaiting confirmatory antibody testing.
ABSTRACT
Treatment of youth concussion during the acute phase continues to evolve, and this has led to the emergence of guidelines to direct care. While symptoms after concussion typically resolve in 14-28 days, a portion (â¼20%) of adolescents endorse persistent post-concussive symptoms (PPCS) beyond normal resolution. This report outlines a study implemented in response to the National Institute of Neurological Diseases and Stroke call for the development and initial clinical validation of objective biological measures to predict risk of PPCS in adolescents. We describe our plans for recruitment of a Development cohort of 11- to 17-year-old youth with concussion, and collection of autonomic, neurocognitive, biofluid, and imaging biomarkers. The most promising of these measures will then be validated in a separate Validation cohort of youth with concussion, and a final, clinically useful algorithm will be developed and disseminated. Upon completion of this study, we will have generated a battery of measures predictive of high risk for PPCS, which will allow for identification and testing of interventions to prevent PPCS in the most high-risk youth.
Subject(s)
Brain Concussion , Post-Concussion Syndrome , Humans , Adolescent , Child , Post-Concussion Syndrome/diagnosis , Endophenotypes , Brain Concussion/psychologyABSTRACT
BACKGROUND: The COVID-19 pandemic altered how residency interviews occur. Despite 2 years of web-based interviews, these are still perceived as inferior to in-person experiences. Showcasing a program and location is critical for recruitment; however, it is difficult to highlight the program's location and community digitally. This article presents the authors' viewpoints on designing and implementing a virtual second look for residency applicants. OBJECTIVE: Our objective was to host a web-based event to feature the benefits of living in Winston-Salem, North Carolina, for residency applicants, enhance recruitment efforts, and ensure a successful residency match. The goal was to cover topics that interested all applicants, highlight how Winston-Salem is a special place to live, involve current residents, and engage community members. METHODS: Three programs-child neurology, neurology, and family medicine were chosen for a pilot virtual second look. All residency program directors' were asked to recommend community contacts and help identify residents and faculty who may serve as content experts on one of the topics in the panel discussions. A total of 24 community leaders from restaurants, venues, schools, and businesses were contacted, and 18 agreed to participate. The panel discussions included living in and raising a family in Winston-Salem, experiencing Winston-Salem arts and music, where to eat and drink like a local, and enjoying sports and outdoors in the area. The 2-hour event was hosted on Zoom. Postevent feedback assessments were automatically sent to each registrant through Research Electronic Data Capture (REDCap). This study was deemed exempt from Wake Forest University Health Sciences institutional review board review (IRB00088703). RESULTS: There were 51 registrants for the event, and 28 of 48 registrants provided postevent feedback, which was positive. The authors found in the MATCH residency results that 2 of 2 child neurology positions, 4 of 6 adult neurology positions, and 1 of 10 family medicine positions attended our second look event. One adult neurology resident who did not participate was an internal candidate. All respondents agreed or strongly agreed that the session was valuable, well organized, and met their expectations or goals. Furthermore, all respondents gained new information during this web-based event not obtained during their interview day. CONCLUSIONS: The virtual second look event for residency attendees featured the benefits of living in Winston-Salem, and the perspectives of current residents. Feedback from the session was overall positive; however, a top desire would be devoting more time for the applicants to ask questions directly to the community leaders and our resident trainees. This program could be reproducible by other institutions. It could be broadened to a graduate medical education-wide virtual second look event where all medical and surgical programs could opt to participate, facilitating an equitable opportunity for prospective applicants.
ABSTRACT
Social media has changed the way we communicate and interact. Unsurprisingly, it has also changed how we teach and learn. Younger generations of learners have transitioned from traditional educational sources to digital ones. Medical educators need to adapt to trends in medical education and develop fluency in the digital methods used by medical learners today. This is part two of a two-part series on social media and digital education in neurology. This article provides an overview of how social media can be used as a teaching tool in medical education and provides an overview in which it is grounded. We offer practical strategies on how social media can promote lifelong learning, educator development, educator support, and foster educator identity with accompanying neurology-specific examples. We also review considerations for incorporating social media into teaching and learning practices and future directions for integrating these tools in neurology education.
ABSTRACT
SCN2A, a gene that codes for a sodium channel highly expressed in the cerebellum, has been linked to a heterogeneous phenotype, including episodic ataxia (EA) and epilepsy, among other symptoms1. Given the rarity of SCN2A-associated EA and its recent description, it is important the genotype-phenotype relationship of SCN2A-associated EA be better defined for prognosis and optimizing future management. Thus, we describe a 2-year-old boy with a SCN2A variant causing an initial prolonged episode of profound ataxia lasting 4 months, cerebellar atrophy, and persistent mild ataxia with episodic exacerbations. Due to the patient's lack of early epilepsy, prolonged initial episode of ataxia, and cerebellar atrophy, this case broadens the scope of the SCN2A variant phenotype. SCN2A should be considered as a cause of early onset ataxia in children with targeted testing or as part of Whole Exome Sequencing (WES) in patients with new onset persistent or EA with or without seizures.
ABSTRACT
Episodic ataxia type 2 (EA2) is a rare autosomal dominant disorder associated with mutations of the CACNA1A gene.1 Because there is no curative therapy available, EA2 is typically managed symptomatically. First line treatment has typically been with acetazolamide.2 Dalfampridine has also been noted to decrease the frequency and duration of ataxic attacks in patients ranging in age from adolescence through adulthood.3, 4 The efficacy and dosing of dalfampridine has not yet been studied in younger pediatric populations. The lack of published experience in younger children can and has led to these patients going without potentially safe and effective treatment. Thus, we describe an 8-year-old girl with EA2 and a confirmed CACNA1A gene mutation whose symptoms had been previously unrelieved by acetazolamide. She was subsequently trialed on dalfampridine and experienced symptomatic relief at a dose of 0.3â mg/kg.
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ABSTRACT: We report the recognition of a diagnosis of leukoencephalopathy with vanishing white matter, also known as vanishing white matter disease in an adolescent male patient after a sports-related concussion. The patient's atypical symptoms after the concussion led to imaging and subsequent neurological consultation. The objective of this clinical case is to highlight the importance of considering imaging in patients who present with atypical symptoms that may be present after a concussion and to raise awareness of this rare disorder which can present after head trauma.
Subject(s)
Athletic Injuries , Brain Concussion , Leukoencephalopathies , White Matter , Adolescent , Athletic Injuries/diagnosis , Brain Concussion/diagnosis , Humans , Leukoencephalopathies/diagnostic imaging , Male , White Matter/diagnostic imaging , White Matter/pathologyABSTRACT
Acute disseminated encephalomyelitis (ADEM) was originally described in the medical literature more than 200 years ago. However, consensus clinical diagnostic criteria are less than 15 years old. Accurate diagnostic testing for myelin oligodendrocyte glycoprotein (MOG) autoantibodies has only become clinically available in the last 3-5 years and has facilitated a rapidly evolving understanding of patients with recurrent demyelination following ADEM. The field is working to optimize treatment for these patients with hopes of prospective treatment studies in the not too distant future. New imaging data suggest that even monophasic demyelination may have long-term impacts that were previously unrecognized. Recent developments in the literature are described in order to guide practice for providers who treat both adults and children with monophasic and recurrent forms of ADEM with and without MOG antibodies.
Subject(s)
Encephalomyelitis, Acute Disseminated , Adult , Autoantibodies , Child , Consensus , Humans , Myelin-Oligodendrocyte Glycoprotein , Prospective StudiesABSTRACT
BACKGROUND: Gait impairment is common after concussion. "Dizziness" is associated with prolonged recovery after concussion. Most often each of these symptoms is considered to be of vestibular origin. However, whether or not there are psychological etiologies of gait instability that complicate concussion recovery has been less fully explored. METHODS: A chart review was performed of all patients seen between 2017 and 2019 in a weekly pediatric traumatic brain injury clinic. RESULTS: Of eighty-four patients with traumatic brain injury, five were found to have developed a classic psychogenic gait. All were adolescents at the time of diagnosis. Recognition of this complication led to modification/initiation of physical therapy and/or mental health intervention and eventual resolution for all patients. CONCLUSION: Although uncommon, psychogenic gait disorders may complicate concussion recovery in adolescents likely at a higher rate than their occurrence in the general population. This association has not been previously reported. Accurate diagnosis of psychogenic gait likely improves outcomes in this subset of patients with concussion.
Subject(s)
Brain Concussion/complications , Gait Disorders, Neurologic/etiology , Somatoform Disorders/etiology , Adolescent , Brain Concussion/rehabilitation , Child , Female , Gait Disorders, Neurologic/diagnosis , Gait Disorders, Neurologic/rehabilitation , Humans , Male , Retrospective Studies , Somatoform Disorders/diagnosis , Somatoform Disorders/rehabilitationABSTRACT
PURPOSE OF REVIEW: Diagnostic criteria for pediatric-onset multiple sclerosis (POMS) and related demyelinating disorders have been updated, neuroimaging studies have revealed new insights, biological assays identify patients with specific antibodies that influence both diagnosis and treatment, clinical trials are informing on treatment efficacy and safety, and longitudinal studies of neurological, cognitive and quality of life outcomes are informing on the impact of these diseases. We provide updates to assist providers caring for these children. RECENT FINDINGS: The recent 2017 McDonald Criteria for MS provide a simplified means to confirm diagnosis at onset and over time, and have been shown to be equally applicable for POMS. MRI analyses demonstrate that brain volume is reduced at onset, and that both volumetric and tissue integrity measures decline over time, indicating that POMS shares the degenerative aspects that also characterize adult-onset disease. The presence of myelin oligodendrocyte glycoprotein (MOG) antibodies at onset is detected in more than 50% of children with acute disseminated encephalomyelitis. When persistent over time, they are associated with relapsing disease. The first randomized clinical trials of disease supports superiority of fingolimod over subcutaneous interferon beta 1a, and demonstrated a favorable safety profile. Finally, while Expanded Disability Status Scale (EDSS) scores remain low in the first 10 years post-onset, POMS is associated with high rates of patient-reported fatigue and reduced engagement in exercise and carries a risk for cognitive impairment. The past 15 years have borne witness to a marked expansion in recognition and research in POMS. There are now more specific diagnostic criteria, antibodies to CNS proteins appear to define diagnostically distinct disorders, clinical trials have successfully launched and one has completed, and we are gaining increasing appreciation of the impact of MS and related disorders on the lived experience of children and adolescents.