Myoclonic status epilepticus after severe hyperthermia in a patient with coronavirus disease 2019.

Myoclonic status epilepticus (MSE) is a sign of severe neurologic injury in cardiac arrest patients. To our knowledge, MSE has not been described as a result of prolonged hyperpyrexia. A 56-yearold man with coronavirus disease 2019 presented with acute respiratory distress syndrome, septic/hypovolemic shock, and presumed community-acquired pneumonia. Five days after presentation, he developed a sustained fever of 42.1°C that did not respond to acetaminophen or ice water gastric lavage. After several hours, he was placed on surface cooling. Three hours after fever resolution, new multifocal myoclonus was noted in the patient's arms and trunk. Electroencephalography showed midline spikes consistent with MSE, which resolved with 40 mg/kg of levetiracetam. This case demonstrates that severe hyperthermia can cause cortical injury significant enough to trigger MSE and should be treated emergently using the most aggressive measures available. Providers should have a low threshold for electroencephalography in intubated patients with a recent history of hyperpyrexia.

Multi-Residency Implementation of an Online Movement Disorders Curriculum Based on Real Patient Videos.

Background: In-person didactic education in residency has numerous challenges including inconsistent availability of faculty and residents, limited engagement potential, and non-congruity with clinical exposure. Methods: An online curriculum in movement disorders was implemented across nine neurology residency programs (six intervention, three control), with the objective to determine feasibility, acceptability, and knowledge growth from the curriculum. Residents in the intervention group completed ten modules and a survey. All groups completed pre-, immediate post-, and delayed post-tests. Results: Eighty-six of 138 eligible housestaff (62.3%) in the intervention group completed some modules and 74 completed at least half of modules. Seventy-four, 49, and 30 residents completed the pre-, immediate post-, and delayed post-tests respectively. Twenty-five of 42 eligible control residents (59.5%) completed at least one test. Mean pre-test scores were not significantly different between groups (6.33 vs. 6.92, p = 0.18); the intervention group had significantly higher scores on immediate post- (8.00 vs. 6.79, p = 0.001) and delayed post-tests (7.92 vs. 6.92, p = 0.01). Residents liked having a framework for movement disorders, appreciated the interactivity, and wanted more modules. Residents completed the curriculum over variable periods of time (1-174 days), and at different times of day. Discussion: This curriculum was feasible to implement across multiple residency programs. Intervention group residents showed sustained knowledge benefit after participating, and residents took advantage of its flexibility in their patterns of module completion. Similar curricula may help to standardize certain types of clinical learning and exposure across residency programs. Highlights: Interactive online tools for resident didactic learning are valuable to residents. Residents learn from interactive online curricula, find the format engaging, and take advantage of the flexibility of online educational tools. Beginner learners appreciate algorithms that help them to approach a new topic.

Implications of the Gut Microbiome in Parkinson's Disease.

Parkinson's disease is a common neurodegenerative disorder that presents with nonmotor and motor symptoms. The nonmotor manifestations of Parkinson's disease often begin years before the motor symptoms. Autopsy studies, including both Parkinson's disease patients and matched controls, demonstrated that α-synuclein aggregates in Parkinson's disease patients can be found in both the substantia nigra and the enteric nervous system. Therefore, it has been hypothesized that the pathological process that leads eventually to Parkinson's disease might initially take place in the enteric nervous system years before the appearance of motor features. The gut microbiome plays essential roles in the development and maintenance of different body systems. Dysbiosis of the normal gut microbiome is thought to be associated with pathophysiologic changes not only in the gastrointestinal system itself but also in the enteric and central nervous systems. These changes are thought to ultimately cause loss of dopaminergic neurons via various mechanisms including the release of neurotoxins into the systemic circulation, decreased production of neuroprotective factors, and triggering inflammatory and autoimmune responses. In this review, we review the gut microbiome changes in Parkinson's disease and discuss the mechanisms by which gut microbiome dysbiosis may be a contributing factor to the pathophysiology of Parkinson's disease. © 2020 International Parkinson and Movement Disorder Society.

An online curriculum in movement disorders for neurology housestaff.

INTRODUCTION: In many neurology residency programs, outpatient neurology subspecialties are underrepresented. Trainee exposure to these subspecialties, including movement disorders, is limited by paucity and variability of clinical experiences. We designed a structured educational tool to address this variability and allow for standardization of elements of movement disorders teaching. METHODS: We designed and implemented a web-based curriculum in movement disorders for neurology housestaff, in order to improve participant knowledge. The curriculum includes an introduction with a structured framework for the description of abnormal movements and 10 interactive modules focusing on common movement disorders. The curriculum was piloted with nine neurology housestaff at Yale-New Haven Hospital. Evaluation of the curriculum was performed using pre- and post-tests, a survey, and semi-structured interviews. RESULTS: The mean pre-test score was 0.7 (±0.19), and the mean post-test score was 0.95 (±0.05) (t = 3.27). Surveys demonstrated mean Likert values >4/5 for all questions in all categories (knowledge acquisition, quantity, enthusiasm and technical). Semi-structured interviews revealed the following themes: 1) the modules increased participant comfort with the topic, 2) the format was engaging, and 3) the curriculum accommodated different learning styles. All participants remarked that the structured framework was a particular strength. CONCLUSION: We have created, implemented, and evaluated a foundational curriculum in movement disorders for neurology trainees, using readily-available technology. Housestaff responded positively to the curriculum, both in terms of content and format. This curriculum can be implemented in a variety of educational settings, as a central component of a standardized approach to movement disorders teaching.

Successful Treatment of Primary Orthostatic Tremor Using Perampanel.

Background: Primary orthostatic tremor (POT) remains a therapeutic conundrum. Various medication classes have been tried, yielding modest results at best. Case Report: A 62-year-old female with a 13-year history of POT, refractory to clonazepam up to 20 mg/day, was treated with perampanel 1-2 mg/day. She reported 90% subjective symptomatic improvement. Discussion: This case highlights the potential for use of perampanel, a novel AMPA (α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) receptor antagonist for the treatment of POT. There has been one prior report citing its use for POT with complete resolution of symptoms. We encourage further studies to highlight its efficacy for POT.

The Future of the Lecture in Neurology Education.

The lecture has been a core pedagogical method since the early days of formal medical education. Although approaches to formal lectures have evolved over the years, there has been ongoing debate about the role that lectures should play in modern medical education. Arguably, traditional lectures do not align well with modern learning theory, and do not take full advantage of our current knowledge of how people learn. In many modern medical curricula, lectures have been replaced by self-study activities, including video-based lectures, computer-based learning modules, and other self-directed learning. We argue that scheduled "together time" is still important, particularly in neurology education, where there is a strong emphasis on clinical reasoning. We outline alternative teaching methods that effectively use this time, including the flipped classroom, just-in-time teaching, problem-based learning, and team-based learning. We discuss ways in which these approaches may be particularly conducive to components of neurology education.

Brain circuits and neurochemical systems in essential tremor: insights into current and future pharmacotherapeutic approaches.

INTRODUCTION: There are few medications that are available for the treatment of essential tremor (ET) and they are only moderately effective. Areas covered: Data were obtained from a PubMed search. Original articles, review articles, and clinical guidelines were included. Two disease models for ET have been proposed: 1) the olivary model, which attributes ET to a pathological pacemaker in the inferior olivary nucleus, and 2) the cerebellar degeneration model, which postulates that ET originates in the cerebellum and could be related to deficient or abnormal Purkinje cell (PC) output. Underlying biochemical dysfunction in T-type calcium channels (T-tCaC) may loosely be linked to the first model and deficiency/abnormality in γ-aminobutyric acid (GABA) neurotransmission, to the second. Expert commentary: Human data points robustly to the role of GABA in ET. Numerous medications that target the GABA system have been tried, with variable success. Given the many different types of GABA-ergic neurons, and the multitude of GABAA receptor subtypes, a given medication could have competing/cancelling effects. It would seem that influencing GABA receptors broadly is not as effective as targeting certain GABAA receptor subtypes. Future research should seek to identify molecular candidates that have a more targeted effect within the GABA system.

A Neurology Clerkship Curriculum Using Video-Based Lectures and Just-in-Time Teaching (JiTT).

Introduction: Just-in-time teaching is an educational strategy that involves tailoring in-session learning activities based on student performance in presession assessments. We implemented this strategy in a third-year neurology clerkship. Methods: Linked to core neurology clerkship lectures, eight brief video-based lectures and knowledge assessments were developed. Students watched videos and completed multiple-choice questions, and results were provided to faculty, who were given the opportunity to adjust the in-person lecture accordingly. Feedback was obtained by surveys of students and faculty lecturers and from student focus groups and faculty. Student performance on the end-of-clerkship examination was analyzed. Results: Between October 2016 and April 2017, 135 students participated in the curriculum, and 56 students (41.5%) responded to the surveys. Most students agreed or strongly agreed that the new curriculum enhanced their learning and promoted their sense of responsibility in learning the content. Faculty agreed that this pedagogy helped prepare students for class. Most students watched the entire video-based lecture, although there was a trend toward decreased audience retention with longer lectures. There were no significant changes in performance on the end-of-clerkship examination after implementation of just-in-time teaching. In focus groups, students emphasized the importance of tying just-in-time teaching activities to the lecture and providing video-based lectures well in advance of the lectures. Discussion: Just-in-time teaching using video-based lectures is an acceptable and feasible method to augment learning during a neurology clinical clerkship. We believe this method could be used in other neurology clerkships with similar success.

Positional Tremor and its Treatment.

BACKGROUND: Positional tremors arise when a patient's tremor is brought on during specific positioning of the involved body part. They can be distinguished from postural tremor, wherein a patient's tremor is elicited in any posture, and from task-specific tremor, wherein a patient's tremor occurs only during a certain task. CASES: We describe two cases of positional tremor that are markedly improved with botulinum toxin injection. DISCUSSION: The term "positional" is a valuable descriptor for tremors. In patients with positional tremor, botulinum toxin may be beneficial for treatment. Lidocaine injection provides a transient way to test for the appropriateness of botulinum toxin injection in these patients.

Tic Exacerbation in Adults with Tourette Syndrome: A Case Series.

BACKGROUND: Tourette syndrome (TS) has been described as peaking in adolescence with subsequent regression. We report patients who were diagnosed with TS during childhood who experienced a latent period (significant reduction in or absence of tics) followed by tic re-emergence in adulthood. METHODS: We performed a retrospective chart review of outpatients over age 21 seen at the Yale neurology clinic between January 2012 and July 2016 who were diagnosed with childhood-onset tics, and who experienced a latent period of greater than 1 year followed by an exacerbation. RESULTS: Sixteen patients were identified. The mean latent period was 16 years. Ten patients (62.5%) identified an exacerbation trigger, most commonly changes in substance use (five patients). Seven patients (43.8%) reported worsening of tics since childhood. Six patients (37.5%) had received pharmacological intervention for tics as children, and 15 patients (93.8%) as adults. Six of 15 patients (40.0%) had an effective response from those pharmacological intervention(s). DISCUSSION: Our study demonstrates that the decline in symptoms as patients age may represent temporary improvement. The latent period lasted years in our patients, different from the more rapid waxing and waning in children. A change in substance use was an important trigger. Requests for pharmacological intervention were not necessarily correlated with worsening tic severity.

Movement Disorders in the Intensive Care Unit.

Movement disorders are common; yet to those without subspecialty experience they can be difficult to recognize, categorize, and manage. Although most frequently encountered in an outpatient setting, patients with movement disorders are commonly hospitalized and often admitted to the intensive care unit, leading to possible confusion about their proper care. In addition, movement disorders can arise in critical care settings for several reasons, including medication side effects, substance withdrawal, or development of a new syndrome. In this review, the authors explore the management of critically ill patients with movement disorders, new or established, including differential diagnosis, work-up, and treatment.

Treatment of Chronic Migraine with Focus on Botulinum Neurotoxins.

Migraine is the most common neurological disorder, and contributes to disability and large healthcare costs in the United States and the world. The treatment of migraine until recently has focused on medications, both abortive and prophylactic, but treatment of chronic migraine has been revolutionized with the introduction of botulinum toxin injection therapy. In this review, we explore the current understanding of migraine pathophysiology, and the evolution of the use of botulinum toxin therapy including proposed pathophysiological mechanisms through animal data. We also discuss the similarities and differences between three injection techniques.

An outbreak of Eastern equine encephalitis virus in free-ranging white-tailed deer in Michigan.

Eastern equine encephalitis (EEE) virus has been recognized as affecting horses and humans in the eastern United States for 70 yr. Evidence of exposure with EEE virus has been reported in a variety of free-ranging wild birds and mammals but cases of clinical disease are much less commonly reported. In Michigan, reports of outbreaks of EEE virus in equine species extend back more than a half century. We report diagnosis of EEE virus infection of multiple free-ranging white-tailed deer (Odocoileus virginianus) from three Michigan counties during late summer of 2005. Infection was confirmed in seven of 30 deer collected based on reported neurologic signs and results from immunohistochemistry, polymerase chain reaction, and/or virus isolation. One of the deer also was infected with West Nile virus and an eighth deer had microscopic lesions in the cerebrum consistent with those reported for EEE. To our knowledge, this is the first report of multiple cases of EEE in free-ranging white-tailed deer, and highlights several issues of significance to wildlife managers and public health officials.