Consensus Recommendations for the Management of Neurosarcoidosis: A Delphi Survey of Experts Across the United States.

Background And Objectives: Neurosarcoidosis poses a diagnostic and management challenge due to its rarity, phenotypic variability, and lack of randomized controlled studies to guide treatment selection. Recommendations for management based on expert opinion are useful in clinical practice and provide a framework for designing prospective studies.

Methods: In this Delphi survey study, specialists with experience in managing patients with neurosarcoidosis were invited to anonymously complete 2 surveys about key elements of evaluation, diagnosis, treatment, monitoring, and long-term management of neurosarcoidosis.

Symmetric leukoencephalopathy associated with systemic lupus erythematosus: A systematic review of a distinctive neurorheumatologic syndrome.

Background: A symmetric leukoencephalopathy can occur in the context of systemic lupus erythematosus (SLE), often as a first manifestation of underlying rheumatologic disease. Recognition of this distinctive syndrome can prompt investigation for SLE when undiagnosed, or prompt treatment initiation when the diagnosis is already known. Earlier recognition of this syndrome could lead to more effective treatment of the disease.

Neurologic Complications of COVID-19.

Objective: This article describes the spectrum of neurologic complications occurring in acute or postacute infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) as well as the neurologic risks and benefits of vaccination against SARS-CoV-2.

Latest Developments: Early in the COVID-19 pandemic, reports of neurologic complications of COVID-19 began to surface. A variety of neurologic conditions have since been reported in association with COVID-19.

Imaging of Central Nervous System Autoimmune, Paraneoplastic, and Neuro-rheumatologic Disorders.

Objective: This article provides an overview of the imaging modalities used in the evaluation of central nervous system (CNS) autoimmune, paraneoplastic, and neuro-rheumatologic disorders. An approach is outlined for interpreting imaging findings in this context, synthesizing a differential diagnosis based on certain imaging patterns, and choosing further imaging for specific diseases.

Latest Developments: The rapid discovery of new neuronal and glial autoantibodies has revolutionized the autoimmune neurology field and has elucidated imaging patterns characteristic of certain antibody-associated diseases.

Curriculum Innovations: Improving Residents' Knowledge and Interest in Outpatient Neurology Through an Interactive Patient-Centered Didactic Series.

Introduction And Problem Statement: Neurology residency training is inpatient focused, underemphasizing outpatient disorders. We implemented a novel didactic series of facilitated discussions between a patient and their outpatient neurologist to expose residents to outpatient topics and management skills.

Objectives: (1) Improve residents' understanding of the roles and responsibilities of the neurologist in the outpatient setting; (2) share with residents the patient's perspective of living with chronic neurologic disease; and (3) improve residents' understanding of what effective shared decision making entails.

Septin-5 and -7-IgGs: Neurologic, Serologic, and Pathophysiologic Characteristics.

Background And Objectives: We sought to determine clinical significance of neuronal septin autoimmunity and evaluate for potential IgG effects.

Methods: Septin-IgGs were detected by indirect immunofluorescence assays (IFAs; mouse tissue and cell based) or Western blot. IgG binding to (and internalization of) extracellular septin epitopes were evaluated for by live rat hippocampal neuron assay.

Palato-Pharyngo-Laryngeal Rhythmic Myoclonus in Neuro-Behcet Syndrome: A Case Report.

Objective: Behcet's syndrome (BS) is a chronic, relapsing multisystemic inflammatory perivasculitis and can affect any tissue, including the nervous system. Neuro-Behcet's syndrome (NBS) most commonly affects the CNS parenchyma and presents with a subacute brainstem syndrome that includes cranial neuropathies. Here we describe a rare case of palato-pharyngo-laryngeal myoclonus as a manifestation of NBS and discuss it from a laryngology perspective.

Multiple sclerosis patients have an altered gut mycobiome and increased fungal to bacterial richness.

Trillions of microbes such as bacteria, fungi, and viruses exist in the healthy human gut microbiome. Although gut bacterial dysbiosis has been extensively studied in multiple sclerosis (MS), the significance of the fungal microbiome (mycobiome) is an understudied and neglected part of the intestinal microbiome in MS. The aim of this study was to characterize the gut mycobiome of patients with relapsing-remitting multiple sclerosis (RRMS), compare it to healthy controls, and examine its association with changes in the bacterial microbiome.

A national survey of Neurology Vice Chairs For Education.

Objectives: A growing number of Neurology Departments have appointed a Neurology Vice Chair for Education (NVCE), yet the roles and responsibilities of an NVCE have not been previously described in the literature.

Methods: A group of current NVCEs developed a survey that was sent to all NVCEs in the US via a secure, anonymous survey. Questions included roles and responsibilities, sources of support, metrics to determine success, faculty development, basic demographics and education scholarship engagement.

Clinical approach to neuro-rheumatology.

Recognizing the neurologic manifestations of systemic rheumatologic diseases and certain isolated autoimmune neurologic diseases poses challenges to the clinician. Using a systematic approach allows the clinician to diagnose these conditions more readily and to initiate treatment more rapidly. Specific neurological syndromes frequently associated with rheumatologic or specific autoimmune conditions can suggest the diagnosis.

Neurosarcoidosis: Pathophysiology, Diagnosis, and Treatment.

Although often regarded as a protean illness with myriad clinical and imaging manifestations, neurosarcoidosis typically presents as recognizable syndromes that can be approached in a rational, systematic fashion. Understanding of neurosarcoidosis has progressed significantly in recent years, including updated diagnostic criteria and advances in treatment. The diagnosis of neurosarcoidosis is established by the clinical syndrome, imaging and histopathological findings, and exclusion of other causes.

Single Center Experience with Isolated Spinal Cord Neurosarcoidosis.

Introduction: Isolated spinal cord neurosarcoidosis is extremely rare. The potential implications of long-term immunosuppressant therapy make correct diagnosis imperative. However, there are challenges inherent in isolated spinal cord involvement that require a multidisciplinary approach.

Vascular Involvement in Neurosarcoidosis: Early Experiences From Intracranial Vessel Wall Imaging.

Background And Objectives: Cerebrovascular manifestations in neurosarcoidosis (NS) were previously considered rare but are being increasingly recognized. We report our preliminary experience in patients with NS who underwent high-resolution vessel wall imaging (VWI).

Methods: A total of 13 consecutive patients with NS underwent VWI.

Consensus disease definitions for neurologic immune-related adverse events of immune checkpoint inhibitors.

Expanding the US Food and Drug Administration-approved indications for immune checkpoint inhibitors in patients with cancer has resulted in therapeutic success and immune-related adverse events (irAEs). Neurologic irAEs (irAE-Ns) have an incidence of 1%-12% and a high fatality rate relative to other irAEs. Lack of standardized disease definitions and accurate phenotyping leads to syndrome misclassification and impedes development of evidence-based treatments and translational research.

Immune-Mediated Myelopathies: A Review of Etiologies, Diagnostic Approach, and Therapeutic Management.

Myelopathy is a broad term used to describe a heterogeneous group of disorders that affects the spinal cord; the focus of this article will be a subgroup of these disorders with an autoimmune and inflammatory-based pathology. Symptoms typically develop over hours or days and then worsen over a matter of days to weeks, but sometimes can have a more insidious or subacute presentation, which can make the diagnosis more puzzling. Despite relatively low incidence rates, almost a third of affected patients are left with severely disabling symptoms.

Unilateral Relapsing Primary Angiitis of the CNS: An Entity Suggesting Differences in the Immune Response Between the Cerebral Hemispheres.

Objective: To determine whether studying patients with strictly unilateral relapsing primary angiitis of the CNS (UR-PACNS) can support hemispheric differences in immune response mechanisms, we reviewed characteristics of a group of such patients.

Methods: We surveiled our institution for patients with UR-PACNS, after characterizing one such case. We defined UR-PACNS as PACNS with clinical and radiographic relapses strictly recurring in 1 brain hemisphere, with or without hemiatrophy.

CNS vasculopathies: Challenging mimickers of primary angiitis of the central nervous system.

Primary angiitis of the central nervous system (CNS) is an inflammatory vasculopathy affecting the brain and spinal cord. It is a difficult diagnosis to make because of its insidious nonspecific course and its multiple mimics. This review identifies and discusses some noninfectious mimickers of primary CNS angiitis, including: reversible cerebral vasoconstriction syndrome, Sneddon's Syndrome, amyloid-beta-related angiopathy, Susac Syndrome, and neurosarcoidosis.

Altered expression of SIRPγ on the T-cells of relapsing remitting multiple sclerosis and type 1 diabetes patients could potentiate effector responses from T-cells.

Factors regulating self-antigen directed immune-responses in autoimmunity are poorly understood. Signal regulatory protein gamma (SIRPγ) is a human T-cell specific protein with genetic variants associated with type 1 diabetes (T1D). SIRPγ's function in the immune system remains unclear.

Association Between Immunosuppressive Treatment and Outcomes of Cerebral Amyloid Angiopathy-Related Inflammation.

Importance: Cerebral amyloid angiopathy-related inflammation (CAA-ri), a distinct subtype of cerebral amyloid angiopathy, is characterized by an autoimmune reaction to cerebrovascular β-amyloid deposits. Outcomes and response to immunosuppressive therapy for CAA-ri are poorly understood.

Objective: To identify clinical, neuroimaging, laboratory, pathologic, or treatment-related associations with outcomes after an episode of CAA-ri.

Diagnostic Testing in Central Nervous System Infection.

Patients with central nervous system (CNS) infection experience very high levels of morbidity and mortality, in part because of the many challenges inherent to the diagnosis of CNS infection and identification of a causative pathogen. The clinical presentation of CNS infection is nonspecific, so clinicians must often order and interpret many diagnostic tests in parallel. This can be a daunting task given the large number of potential pathogens and the availability of different testing modalities.

Building a neuroinfectious disease consensus curriculum.

Objective: To delineate a comprehensive curriculum for fellowship training in neuroinfectious diseases, we conducted a modified Delphi approach to reach consensus among 11 experts in the field.

Methods: The authors invited a diverse range of experts from the American Academy of Neurology Neuro-Infectious Diseases (AAN Neuro-ID) Section to participate in a consensus process using a modified Delphi technique.

Results: A comprehensive list of topics was generated with 101 initial items.

A Consensus Guideline to Support Resident-as-Teacher Programs and Enhance the Culture of Teaching and Learning.

Background: Methods for assessing residents as teachers are limited, and it can be difficult to discern optimal curricula for training residents as educators. A guideline may be a tool to assess resident-as-teacher programs and to help enhance a culture of teaching and learning.

Objective: We developed a consensus guideline to assess academic medical centers' resident-as-teacher programs and teaching environments.