Barriers to increasing paid parental leave in U.S. neurology residencies: a survey of program directors.

BACKGROUND: The American Board of Psychiatry and Neurology (ABPN) and the Accreditation Council for Graduate Medical Education (ACGME) require that residency programs allow at least 6 weeks of parental leave. The American Medical Association (AMA) recommends 12 weeks of paid parental leave. Despite these recommendations, there is little information about parental leave policies across U.S. neurology residencies. The objective of our study was to assess parental leave policies in U.S. adult neurology residencies and barriers to increasing the duration of leave. METHODS: We distributed an anonymous online survey to U.S. adult neurology program directors (PDs) to assess demographics, components and length of parental leave, perceived impact on residents' clinical training and academic development, and barriers to increasing the length of leave. RESULTS: We contacted 163 PDs and received 54 responses (response rate of 33%). 87% reported policies for both childbearing and non-childbearing residents. The average maximal length of leave allowed without extension of training was 8.5 weeks (range 0-13) for childbearing and 6.2 weeks (range 0-13) for non-childbearing residents. Most PDs felt that parental leave had a positive impact on resident wellness and neutral impact on clinical competency, academic opportunities, and career development. The most common barriers to providing a 12-week paid policy were concerns about equity in the program (82%), staffing of clinical services (80%), and impact on clinical training (78%). CONCLUSIONS: Although most programs in our study have parental leave policies, there is significant variability. Policies to improve parental leave should focus on addressing common barriers, such as additional solutions to staffing clinical services.

Midbrain lesion-induced disconjugate gaze: a unifying circuit mechanism of ocular alignment?

BACKGROUND: Disconjugate eye movements are essential for depth perception in frontal-eyed species, but their underlying neural substrates are largely unknown. Lesions in the midbrain can cause disconjugate eye movements. While vertically disconjugate eye movements have been linked to defective visuo-vestibular integration, the pathophysiology and neuroanatomy of horizontally disconjugate eye movements remains elusive. METHODS: A patient with a solitary focal midbrain lesion was examined using detailed clinical ocular motor assessments, binocular videooculography and diffusion-weighted MRI, which was co-registered to a high-resolution cytoarchitectonic MR-atlas. RESULTS: The patient exhibited both vertically and horizontally disconjugate eye alignment and nystagmus. Binocular videooculography showed a strong correlation of vertical and horizontal oscillations during fixation but not in darkness. Oscillation intensities and waveforms were modulated by fixation, illumination, and gaze position, suggesting shared visual- and vestibular-related mechanisms. The lesion was mapped to a functionally ill-defined area of the dorsal midbrain, adjacent to the posterior commissure and sparing nuclei with known roles in vertical gaze control. CONCLUSION: A circumscribed region in the dorsal midbrain appears to be a key node for disconjugate eye movements in both vertical and horizontal planes. Lesioning this area produces a unique ocular motor syndrome mirroring hallmarks of developmental strabismus and nystagmus. Further circuit-level studies could offer pivotal insights into shared pathomechanisms of acquired and developmental disorders affecting eye alignment.

Teaching Video NeuroImage: Monocular Rotational Nystagmus Caused by Superior Oblique Myokymia.

A 61-year-old man presented with 2 days of "flickering" vision. The symptom resolved with closure of the left eye. Examination demonstrated involuntary high-frequency, low-amplitude intorting movements of the left eye, consistent with superior oblique myokymia (Video 1). Ocular ductions were full, and there were no abnormal movements of the right eye.

Network Localization of Awareness in Visual and Motor Anosognosia.

OBJECTIVE: Unawareness of a deficit, anosognosia, can occur for visual or motor deficits and lends insight into awareness itself; however, lesions associated with anosognosia occur in many different brain locations. METHODS: We analyzed 267 lesion locations associated with either vision loss (with and without awareness) or weakness (with and without awareness). The network of brain regions connected to each lesion location was computed using resting-state functional connectivity from 1,000 healthy subjects. Both domain specific and cross-modal associations with awareness were identified. RESULTS: The domain-specific network for visual anosognosia demonstrated connectivity to visual association cortex and posterior cingulate while motor anosognosia was defined by insula, supplementary motor area, and anterior cingulate connectivity. A cross-modal anosognosia network was defined by connectivity to the hippocampus and precuneus (false discovery rate p < 0.05). INTERPRETATION: Our results identify distinct network connections associated with visual and motor anosognosia and a shared, cross-modal network for awareness of deficits centered on memory-related brain structures. ANN NEUROL 2023;94:434-441.

Fluorescein Angiography Findings in Susac Syndrome: A Multicenter Retrospective Case Series.

BACKGROUND: Susac syndrome is a vasculopathy, resulting in the classic triad of branch retinal artery occlusion (BRAO), inner ear ischemia, and brain ischemia. In this retrospective chart review, we characterize fluorescein angiography (FA) findings and other ancillary studies in Susac syndrome, including the appearance of persistent disease activity and the occurrence of new subclinical disease on FA. METHODS: This multicenter, retrospective case series was institutional review board-approved and included patients with the complete triad of Susac syndrome evaluated with FA, contrasted MRI of the brain, and audiometry from 2010 to 2020. The medical records were reviewed for these ancillary tests, along with demographics, symptoms, visual acuity, visual field defects, and findings on fundoscopy. Clinical relapse was defined as any objective evidence of disease activity during the follow-up period after initial induction of clinical quiescence. The main outcome measure was the sensitivity of ancillary testing, including FA, MRI, and audiometry, to detect relapse. RESULTS: Twenty of the 31 (64%) patients had the complete triad of brain, retinal, and vestibulocochlear involvement from Susac syndrome and were included. Median age at diagnosis was 43.5 years (range 21-63), and 14 (70%) were women. Hearing loss occurred in 20 (100%), encephalopathy in 13 (65%), vertigo in 15 (75%), and headaches in 19 (95%) throughout the course of follow-up. Median visual acuity at both onset and final visit was 20/20 in both eyes. Seventeen (85%) had BRAO at baseline, and 10 (50%) experienced subsequent BRAO during follow-up. FA revealed nonspecific leakage from previous arteriolar damage in 20 (100%), including in patients who were otherwise in remission. Of the 11 episodes of disease activity in which all testing modalities were performed, visual field testing/fundoscopy was abnormal in 4 (36.4%), MRI brain in 2 (18.2%), audiogram in 8 (72.7%), and FA in 9 (81.8%). CONCLUSIONS: New leakage on FA is the most sensitive marker of active disease. Persistent leakage represents previous damage, whereas new areas of leakage suggest ongoing disease activity that requires consideration of modifying immunosuppressive therapy.

Adding Nocturnal Advanced Practice Providers to an Academic Inpatient Neurology Service Improves Residents' Educational Experience.

Background and Objectives: In the inpatient academic medical center, increased demand for clinical services often equates to an increased workload for trainees, which could have a positive or negative impact on their educational experience. In 2020, our academic medical center hired Advanced Practice Providers (APPs) to provide continuous additional overnight coverage for our neurology ward teaching service. We hypothesized that adding APPs and reducing overnight clinical workload for residents would have a positive impact on resident education. Methods: We performed a mixed-methods, prospective study that included needs-assessments by residents, semi-structured interviews with both residents and APPs, and surveys to residents and nursing staff. In addition, we collected quantitative data such as hours of sleep, number of admissions, and number of pages to capture the impact of APPs on resident overnight shifts. Results: The addition of APPs overnight increased the median hours of sleep overnight from 1 hour to 3 hours (P < .001) and decreased the median number of pages overnight from 31.5 to 17 (P < .001). The median number of patients the resident was responsible for cross-covering overnight decreased from 24 patients to 14 patients (P < .001). The majority of resident responses (94%) agreed that the addition of APPs benefited their education by reducing workload and increasing time allotted to reading and formulating plans for overnight admissions. 88% of residents agreed that the addition of APPs improved quality of life and reduced risk of burnout. Conclusion: Advanced Practice Providers significantly reduced resident workload, leading residents to report improvements in the educational experience overnight and reduced perceived risk of burnout.

Treatment outcomes of first-ever episode of severe optic neuritis.

BACKGROUND: Severe optic neuritis (ON) is an acute inflammatory attack of the optic nerve(s) leading to severe visual loss that may occur in isolation or as part of a relapsing neuroinflammatory disease, such neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD), or more rarely multiple sclerosis (MS). In cases of first-ever severe ON of uncertain etiology best treatment strategies remain unclear. METHODS: We reviewed records of all patients with a documented diagnosis of ON between 2004 and 2019 at Mass General Brigham (MGB) and Johns Hopkins University (JHU) hospitals. Out of 381 patients identified, 90 (23.6%) satisfied the study criteria for severe ON with visual acuity (VA) equal to or worse than 20/200 (logMAR=1) at nadir in the affected eye and had sufficient follow-up data. Treatment strategies with corticosteroids only or treatment escalation with therapeutic plasma exchange (PLEX) after steroids were compared and evaluated for differences in visual outcomes at follow-up. RESULTS: Of the 90 patients with severe optic neuritis, 71(78.9%) received corticosteroids only, and 19 (17.0%) underwent PLEX following corticosteroids. Of the 71 patients who received steroids without escalation to PLEX, 30 patients (42.2%) achieved complete recovery (VA 20/20 on the affected eye), whereas 35 (49.3%) had a partial recovery and 6 (8.4%) had no recovery. Among the 19 corticosteroid non-responders patients who underwent escalation treatment, 13 (68.4%) made complete recovery, 6 (31.6%) had partial visual recoveries (p=0.0434). The median delta logMAR of patients who underwent escalation of care was -1.2 compared with 2.0 for the ones who did not (p=0.0208). A change of delta logmar 2.0 is equivalent of going from hand motion to light perception and the positive delta value refers to intra-attack worsening. Other than not responding to steroids, patients who underwent PLEX tended to have more severe ON with significantly worse nadir visual acuity compared with those who received corticosteroids alone (logMAR 3.12 (min 2.0 - max 5.0) vs. 2.17 (min 1.3 - max 3.0); p=0.004). CONCLUSION: In our cohort of first-ever severe optic neuritis of unknown etiology, patients that did not respond adequately to corticosteroids benefited from treatment escalation to PLEX, followed in most cases by Rituximab, regardless of final etiology. Randomized controlled trials are needed to confirm the best treatment strategies.

Clinical Reasoning: A 68-Year-Old Man With Palmar Rash, Leg Pain, and Inability to Walk.

We report a 68-year-old man with palmar rash, leg pain, and inability to walk, whose neurologic examination localized to multiple roots, leading to the diagnosis of polyradiculopathy. Polyradiculopathy has both structural and nonstructural etiologies. Structural etiologies include spinal stenosis, tethered cord syndrome, tumors, and leptomeningeal metastasis. Nonstructural etiologies include inflammatory, infectious, metabolic, and other neoplastic processes. His palmar rash in conjunction with neurologic signs/symptoms raised the suspicion for an infectious etiology, and eventually, serology and lumbar puncture confirmed the final diagnosis. In this report, we will further discuss how we determined the localization of polyradiculopathy, its specific etiologies, management decisions, and a transient complication before he achieved a significant improvement at 6 months.

Does Tocilizumab Influence Ophthalmic Outcomes in Giant Cell Arteritis?

BACKGROUND: Despite appropriate use of corticosteroids, an important minority of patients with giant cell arteritis (GCA) develop progressive vision loss during the initial stages of the disease or during corticosteroid tapering. Tocilizumab is the only clearly effective adjunctive treatment to corticosteroids in the management of GCA, but questions regarding its efficacy specifically in the neuro-ophthalmic population and its role in mitigating vision loss have not been broached until recently. EVIDENCE ACQUISITION: The authors queried Pubmed using the search terms "GCA" and "tocilizumab" in order to identify English-language publications either explicitly designed to evaluate the influence of tocilizumab on the ophthalmic manifestations of GCA or those which reported, but were not primarily focused on, ophthalmic outcomes. RESULTS: Recent retrospective analyses of populations similar to those encountered in neuro-ophthalmic practice suggest that tocilizumab is effective in decreasing the frequency of GCA relapse, the proportion of flares involving visual manifestations of GCA, and the likelihood of permanent vision loss. Data regarding the utility of tocilizumab to curtail vision loss at the time of diagnosis are limited to case reports. CONCLUSIONS: Compared with conventional corticosteroid monotherapy, treatment of GCA with both corticosteroids and tocilizumab may decrease the likelihood of permanent vision loss. Further prospective, collaborative investigation between rheumatologists and neuro-ophthalmologists is required to clarify the ophthalmic and socioeconomic impact of tocilizumab on the treatment of GCA.

Development and Impact of a Progressive Parental Leave Policy in a Neurology Residency.

Appropriate parental leave policies remain an unmet need in graduate medical education. Although legal and institutional guidelines allow for policies that support parental leave, there are many challenges and perceived barriers to consider in developing and implementing a successful policy. In 2018, we revised the parental leave policy for our neurology residency. Here we describe the development of our policy, measure its effects, and offer guidelines for other programs to develop a similar approach. We propose solutions to commonly encountered problems, focusing on training and education, staffing of clinical services, evolving legal requirements, resident well-being and equity, and financial support.

Network Localization of Unconscious Visual Perception in Blindsight.

OBJECTIVE: Blindsight is a disorder where brain injury causes loss of conscious but not unconscious visual perception. Prior studies have produced conflicting results regarding the neuroanatomical pathways involved in this unconscious perception. METHODS: We performed a systematic literature search to identify lesion locations causing visual field loss in patients with blindsight (n = 34) and patients without blindsight (n = 35). Resting state functional connectivity between each lesion location and all other brain voxels was computed using a large connectome database (n = 1,000). Connections significantly associated with blindsight (vs no blindsight) were identified. RESULTS: Functional connectivity between lesion locations and the ipsilesional medial pulvinar was significantly associated with blindsight (family wise error p = 0.029). No significant connectivity differences were found to other brain regions previously implicated in blindsight. This finding was independent of methods (eg, flipping lesions to the left or right) and stimulus type (moving vs static). INTERPRETATION: Connectivity to the ipsilesional medial pulvinar best differentiates lesion locations associated with blindsight versus those without blindsight. Our results align with recent data from animal models and provide insight into the neuroanatomical substrate of unconscious visual abilities in patients. ANN NEUROL 2022;91:217-224.

Ophthalmic Implications of Chimeric Antigen Receptor T-Cell Therapy.

Chimeric antigen receptor (CAR) T-cell therapy is a revolutionary addition to the burgeoning field of immunotherapy. CAR T-cells are engineered by combining a T-cell receptor with the antigen-binding site of an immunoglobulin that allows the hybrid cell to target antigens of interest. CAR T-cell therapy has been approved to treat various hematologic malignancies, including relapsed or refractory B-cell acute lymphoblastic leukemia and diffuse large B-cell lymphoma. While the treatment efficacy is exciting, challenges remain in understanding the unique spectrum of adverse effects of CAR T-cell therapy, including cytokine release syndrome and neurotoxicity. Innovative research is underway to expand this therapy into solid tumors and fields beyond hematology and oncology. To date, there has been limited research into ophthalmic uses and considerations of CAR T-cell therapy. This review focuses on preclinical investigations into CAR T-cell therapy for retinoblastoma and uveal melanoma, as well as ophthalmic complications of CAR T-cell therapy.

Optic Neuropathy Due to Compression by an Ectatic Internal Carotid Artery Within the Orbital Apex.

ABSTRACT: Neurovascular compression is a rare but potentially treatable cause of optic neuropathy. Although incidental contact of the cisternal optic nerve and internal carotid artery (ICA) is common, compressive optic neuropathy occurring within the orbital apex has not been comprehensively described. We report a case of intra-orbital and intracanalicular optic nerve compression due to an ectatic ICA in a patient with congenital absence of the contralateral ICA. This report describes the complementary roles of advanced neuroimaging and neuro-ophthalmologic examination in rendering the diagnosis.

Bilateral occipital metastases: Visual deficits and management considerations.

BACKGROUND: Metastases to the bilateral occipital lobes pose a difficult clinical scenario due to risk of debilitating visual loss. We sought to characterize clinical outcomes following different treatment modalities to help guide management in this challenging situation. METHODS: We retrospectively reviewed brain metastases patients treated at a single institution between 2008 and 2017 and assessed visual symptoms before and after treatment, the tumor and peritumoral edema volumes before treatment, and clinical outcomes including mortality. RESULTS: Eighteen patients with metastases affecting both occipital lobes were identified. Lung cancer represented the most common primary (n = 10). Visual deficits were present in 12 patients at the time of diagnosis of bilateral occipital metastases (67%). Patients received radiotherapy (n = 5) or combined surgical resection and radiotherapy (n = 13). Among symptomatic patients, two received radiation and 10 received combined surgery and radiation. Nine patients had improved visual symptoms after treatment with no new visual deficits reported as a result of treatment. Among asymptomatic patients, three were treated with radiation alone and three with resection and radiation. Three of these patients developed new visual symptoms following treatment, including one patient with Balint's syndrome. CONCLUSION: Patients with symptomatic bilateral occipital lobe metastases may experience visual improvement following intervention, especially if symptoms stem from compression or edema. Those without visual symptoms are at risk of developing new visual deficits during treatment, which should be included in the decision-making process and when counseling patients. Visual deficits improved after surgery in the majority of patients, with no cases of immediate visual deterioration.

What You Need to Know About AQP4, MOG, and NMOSD.

Neuromyelitis optica (NMO) is an antibody-mediated inflammatory disease of the central nervous system with a predilection for the optic nerves, spinal cord, and certain brain regions. While NMO was previously considered a variant of multiple sclerosis (MS), it is now known to have distinct clinical, pathological, and immunological features. The identification of AQP4-IgG, a pathogenic antibody against aquaporin-4 (AQP4), delineated NMO from MS and markedly advanced insights into the unique features of this disease. The specificity of this antibody has allowed an expanded view of the clinical presentations of NMO-spectrum disorders (NMOSD), without requiring all the clinical features that were previously essential to make a clinical diagnosis. Early, accurate diagnosis of patients with NMOSD permits treatment with appropriate acute and long-term immunosuppressive agents that are critical to mitigate the risk of disability associated with this disease. More recently, a subset of patients with the NMOSD phenotype have been found to have autoantibodies targeting myelin oligodendrocyte glycoprotein (MOG), which has a different pathogenesis and expected outcome. Better understanding of the distinct pathophysiology of these disorders has laid the foundation for targeted efforts to develop novel, disease-specific treatments. In this review, we discuss the revised diagnostic criteria for NMOSD, appraise the diagnostic significance of the AQP4-IgG and MOG-IgG tests, review evidence supporting the use of available treatments for acute episodes and long-term disease modification, and highlight key emerging immunotherapies.