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The objective of this paper is to evaluate available evidence for each step in autoimmune encephalitis management and provide expert opinion when evidence is lacking. The paper approaches autoimmune encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. Corticosteroids alone or combined with other agents (intravenous IG or plasmapheresis) were selected as a first-line therapy by 84% of responders for patients with a general presentation, 74% for patients presenting with faciobrachial dystonic seizures, 63% for NMDAR-IgG encephalitis and 48.5% for classical paraneoplastic encephalitis. Half the responders indicated they would add a second-line agent only if there was no response to more than one first-line agent, 32% indicated adding a second-line agent if there was no response to one first-line agent, while only 15% indicated using a second-line agent in all patients. As for the preferred second-line agent, 80% of responders chose rituximab while only 10% chose cyclophosphamide in a clinical scenario with unknown antibodies. Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.

Background and purposeNeurohospitalist neurology is a fast-growing subspecialty with a variety of practice settings featuring neurohospitalist models of care. Since inception, the subspecialty has responded to new challenges in resident training, hospital reimbursement, practice, and burnout.MethodsTo characterize neurohospitalists' current practice and perspectives, we surveyed the neurohospitalists and trainees affiliated with the Neurohospitalist Society using an electronic survey distributed through the society listserv.ResultsOf 501 individuals surveyed by e-mail, 119 began the survey (23.8% response rate), with 88.2% self-identifying as neurohospitalists. Most neurohospitalists (63%) are 10 years or less out of training, devoting 70% of their professional time to inpatient clinical activities while also performing administrative or teaching activities. Only 38% are employed by an academic department. Call schedules are common, with 75% of neurohospitalists participating in a hospital or emergency call schedule, while 55% provide telemedicine services. The majority (97%) of neurohospitalists primarily care for adults, most commonly treating patients with cerebrovascular disease, seizures, and delirium/encephalopathy. The majority (87%) are overall pleased with their work, but 36% report having experienced burnout.ConclusionsNeurohospitalists are a diverse group of neurologists primarily practicing in the inpatient setting while performing a variety of additional activities. They provide a wide array of clinical expertise for acute neurological diseases and neurological emergencies that require hospitalization, including stroke, seizure, and encephalopathy. Neurohospitalists in general are very pleased with their work, while burnout, as in neurology and other areas of medicine, remains a concern.

IntroductionReducing unplanned hospital readmissions has become a national focus due to the Centers for Medicare and Medicaid Services' (CMS) penalties for hospitals with high rates. A first step in reducing unplanned readmission is to understand which patients are at high risk for readmission, which readmissions are planned, and how well planned readmissions are currently captured in comparison to patient-level chart review.MethodsWe examined all 5455 inpatient neurology admissions over a 2-year period to University of California San Francisco Medical Center and Johns Hopkins Hospital via chart review. We collected information such as patient age, procedure codes, diagnosis codes, all-payer diagnosis-related group, observed length of stay (oLOS), and expected length of stay. We performed multivariate logistic modeling to determine predictors of readmission. Discharge summaries were reviewed for evidence that a subsequent readmission was planned.ResultsA total of 353 (6.5%) discharges were readmitted within 30 days. Fifty-five (15.6%) of the 353 readmissions were planned, most often for a neurosurgical procedure (41.8%) or immunotherapy (23.6%). Only 8 of these readmissions would have been classified as planned using current CMS methodology. Patient age (odds ratio [OR] = 1.01 for each 10-year increase, P < .001) and estimated length of stay (OR = 1.04, P = .002) were associated with a greater likelihood of readmission, whereas index admission oLOS was not.ConclusionsMany neurologic readmissions are planned; however, these are often classified by current CMS methodology as unplanned and penalized accordingly. Modifications of the CMS lists for potentially planned neurological and neurosurgical procedures and for acute discharge neurologic diagnoses should be considered.

Introduction: Autoimmune encephalitis (AE) comprises a heterogeneous group of autoantibody‐mediated disorders targeting the brain parenchyma. Therapeutic plasma exchange (TPE), one of several first‐line therapies for AE, is often initiated when AE is suspected, albeit prior to an established diagnosis. We sought to characterize the role of TPE in the treatment of suspected AE. Methods: A single‐center, retrospective analysis was performed of adults (≥18 years) who underwent at least one TPE procedure for "suspected AE." The following parameters were extracted and evaluated descriptively: clinicopathologic characteristics, treatment course, TPE‐related adverse events, outcomes (e.g., modified Rankin scale [mRS]), and diagnosis once investigation was complete. Results: A total of 37 patients (median age 56 years, range 28–77 years, 62.2% male) were evaluated. Autoimmune antibody testing was positive in serum for 43.2% (n = 16) and cerebrospinal fluid for 29.7% (n = 11). Patients underwent a median of five TPE procedures (range 3–16), with 97.3% (n = 36) via a central line and 21.6% (n = 8) requiring at least one unit of plasma as replacement fluid. Fifteen patients (40.5%) experienced at least one TPE‐related adverse event. Compared with mRS at admission, the mRS at discharge was improved in 21.6% (n = 8), unchanged in 59.5% (n = 22), or worse in 18.9% (n = 7). Final diagnosis of AE was determined to be definite in 48.6% (n = 18), probable in 8.1% (n = 3) and possible in 27.0% (n = 10). Six (16.2%) patients were ultimately determined to have an alternate etiology. Conclusion: Empiric TPE for suspected AE is generally well‐tolerated. However, its efficacy remains uncertain in the absence of controlled trials, particularly in the setting of seronegative disease. [ABSTRACT FROM AUTHOR]

Objective: Brain 18F‐FDG PET/CT is a useful diagnostic in evaluating patients with suspected autoimmune encephalitis (AE). Specific patterns of brain dysmetabolism have been reported in anti‐NMDAR and anti‐LGI1 AE, and the degree of dysmetabolism may correlate with clinical functional status.18FDG‐PET/CT abnormalities have not yet been described in seronegative AE. Methods: We conducted a cross‐sectional analysis of brain18FDG‐PET/CT data in people with seronegative AE treated at the Johns Hopkins Hospital. Utilizing NeuroQ™ software, the Z‐scores of 47 brain regions were calculated relative to healthy controls, then visually and statistically compared for probable and possible AE per clinical consensus diagnostic criteria to previous data from anti‐NMDAR and anti‐LGI1 cohorts. Results: Eight probable seronegative AE and nine possible seronegative AE were identified. The group only differed in frequency of abnormal brain MRI, which was seen in all of the probable seronegative AE patients. Both seronegative groups had similar overall patterns of brain dysmetabolism. A common pattern of frontal lobe hypometabolism and medial temporal lobe hypermetabolism was observed in patients with probable and possible seronegative AE, as well as anti‐NMDAR and anti‐LGI1 AE as part of their respective characteristic patterns of dysmetabolism. Four patients had multiple brain18FDG‐PET/CT scans, with changes in number and severity of abnormal brain regions mirroring clinical status. Conclusions: A18FDG‐PET/CT pattern of frontal lobe hypometabolism and medial temporal lobe hypermetabolism could represent a common potential biomarker of AE, which along with additional clinical data may facilitate earlier diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Supplemental Digital Content is Available in the Text. Background: Anti-NMDA receptor (NMDAR) encephalitis patients have been reported to exhibit visual dysfunction without retinal thinning. The objective of our study was to examine the involvement of the visual pathway structure and function in anti-NMDAR encephalitis by assessing postrecovery visual function and retinal structure, and acute-phase occipital cortex function. Methods: In this cross-sectional study, patients diagnosed with anti-NMDAR encephalitis per consensus criteria underwent postrecovery visual acuity (VA) testing and optical coherence tomography (OCT) with automated retinal layer segmentation. Clinical data and acute-phase brain 18F-fluorodeoxyglucose (FDG) PET/CT (performed within 90 days of symptom onset, assessed qualitatively and semi-quantitatively) were retrospectively analyzed. VA and OCT measures were compared between anti-NMDAR and age, sex, and race-matched healthy controls (HC). When available, FDG-PET/CT metabolism patterns were analyzed for correlations with VA, and OCT measures. Results: A total of 16 anti-NMDAR (32 eyes) and 32 HC (64 eyes) were included in the study. Anti-NMDAR exhibited lower low-contrast VA (2.5% contrast: −4.4 letters [95% CI; −8.5 to −0.3]; P = 0.04, 1.25% contrast: −6.8 letters [95%CI; −12 to −1.7]; P = 0.01) compared with HC, but no differences were found on OCT-derived retinal layer thicknesses. Acute-phase FDG-PET/CT medial occipital cortex metabolism did not correlate with follow-up low-contrast VA or ganglion cell/inner plexiform layer thickness (GCIPL) (n = 7, 2.5% contrast: r = −0.31; P = 0.50, 1.25% contrast: r = −0.34; P = 0.45, GCIPL: r = −0.04; P = 0.94). Conclusions: Although the visual system seems to be involved in anti-NMDAR encephalitis, no retinal structural or occipital cortex functional abnormalities seem to be responsible for the visual dysfunction. When detected acutely, occipital lobe hypometabolism in anti-NMDAR encephalitis does not seem to associate with subsequent retrograde trans-synaptic degenerative phenomena, potentially reflecting reversible neuronal/synaptic dysfunction in the acute phase of the illness rather than neuronal degeneration. [ABSTRACT FROM AUTHOR]

Background: The presence of co-existent neuronal antibodies (neuronal-IgG) in patients with myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG1) is not yet well understood. Objectives: The aim of this study was to investigate the co-existence of a broad range of neuronal-IgG in MOG-IgG1+ patients. Methods: MOG-IgG1+ patients were tested for 17 neuronal-IgGs in cerebrospinal fluid (CSF) and serum including NMDA-R-IgG, AMPA-R-IgG, GABAB-R-IgG, LGI1-IgG, CASPR2-IgG, GABAA-R-IgG, GAD65-IgG, mGLUR1-IgG, DPPX-IgG, CRMP5-IgG, amphiphysin-IgG, PCA1,2,Tr, and ANNA1,2,3. Clinical and radiological features of MOG-IgG1+ with NMDA-R-IgG in CSF were compared to a control cohort of MOG-IgG1+ patients without NMDA-R-IgG. Results: A total of 376 MOG-IgG1+ patients underwent testing for neuronal-IgGs. Serum testing for neuronal-IgGs (113 adults, 142 children) identified one child with NMDA-R-IgG (0.7%), one child with CASPR2-IgG (0.7%), one adult with LGI1-IgG (0.9%) and one adult with GABAA-R-IgG (0.9%). CSF testing for neuronal-IgGs (97 adults, 169 children) identified seven children (4%) and seven adults (7%) with NMDA-R-IgG, and one adult with GABAA-R-IgG (1%). The MOG-IgG1+/NMDA-R-IgG+ patients had a median age of 17 (range: 2–39) years. Features associated with MOG-IgG1+/NMDA-R-IgG+ included encephalopathy (p = 0.001), seizures (p = 0.045), and leptomeningeal enhancement (p = 0.045). Conclusion: NMDA-R-IgG was the most frequently detected neuronal-IgG to co-exist with MOG-IgG1. MOG-IgG1+/NMDA-R-IgG+ patients most often presented with encephalopathy and seizures. Testing for MOG-IgG1 and NMDA-R-IgG may be warranted in patients with encephalopathy and inflammatory demyelinating syndromes. [ABSTRACT FROM AUTHOR]