Objective: To report the clinical features of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in patients Յ 18 years old. Methods: Information was obtained by the authors or referring physicians. Antibodies were determined by immunocytochemistry and enzyme-linked immunosorbent assay (ELISA) using HEK293 cells ectopically expressing NR1. Results: Over an 8-month period, 81 patients (12 male) with anti-NMDAR encephalitis were identified. Thirty-two (40%) were Յ18 years old (youngest 23 months, median 14 years); 6 were male. The frequency of ovarian teratomas was 56% in women Ͼ18 years old, 31% in girls Յ18 years old ( p ϭ 0.05), and 9% in girls Յ14 years old ( p ϭ 0.008). None of the male patients had tumors. Of 32 patients Յ18 years old, 87.5% presented with behavioral or personality change, sometimes associated with seizures and frequent sleep dysfunction; 9.5% with dyskinesias or dystonia; and 3% with speech reduction. On admission, 53% had severe speech deficits. Eventually, 77% developed seizures, 84% stereotyped movements, 86% autonomic instability, and 23% hypoventilation. Responses to immunotherapy were slow and variable. Overall, 74% had full or substantial recovery after immunotherapy or tumor removal. Neurological relapses occurred in 25%. At the last follow-up, full recovery occurred more frequently in patients who had a teratoma that was removed (5/8) than in those without a teratoma (4/23; p ϭ 0.03). Interpretation: Anti-NMDAR encephalitis is increasingly recognized in children, comprising 40% of all cases. Younger patients are less likely to have tumors. Behavioral and speech problems, seizures, and abnormal movements are common early symptoms. The phenotype resembles that of the adults, although dysautonomia and hypoventilation are less frequent or severe in children.Ann Neurol 2009;66:11-18 Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a recently described disorder with a well defined set of clinical features.1 The associated syndrome has been characterized in adults, frequently young women with teratomas of the ovary who develop changes of mood, behavior, and personality, resembling acute psychosis. The clinical picture usually progresses to include seizures, decreased level of consciousness, dyskinesias, autonomic instability, and hypoventilation.2-5 Despite the severity of the disorder, patients often improve with immunotherapy and removal of the teratoma. 1,3,6 These findings and the discovery that all patients have serum and cerebrospinal fluid (CSF) antibodies that react with the cell surface of neurons suggested an immune-mediated pathogenesis. 1,7,8 Further studies demonstrated that the target antigen of patients' antibodies was the NR1 subunit of the NMDAR. Additionally, application of antibodies into cultures of hippocampal neurons resulted in a significant decrease of postsynaptic NMDAR clusters that was reversed after antibody removal. 9 A recent series of 100 patients showed that the disorder also occurs in patients without teratoma, and that men and children c...

Background-This study assesses the utility of a hybrid optical instrument for noninvasive transcranial monitoring in the neurointensive care unit. The instrument is based on diffuse correlation spectroscopy (DCS) for measurement of cerebral blood flow (CBF), and near-infrared spectroscopy (NIRS) for measurement of oxy-and deoxy-hemoglobin concentration. DCS/NIRS measurements of CBF and oxygenation from frontal lobes are compared with concurrent xenon-enhanced computed tomography (XeCT) in patients during induced blood pressure changes and carbon dioxide arterial partial pressure variation.

ObjectiveMyelin oligodendrocyte glycoprotein–immunoglobulin G (MOG-IgG) associated disorder (MOGAD) often manifests with recurrent CNS demyelinating attacks. The optimal treatment for reducing relapses is unknown. To help determine the efficacy of long-term immunotherapy in preventing relapse in patients with MOGAD, we conducted a multicenter retrospective study to determine the rate of relapses on various treatments.MethodsWe determined the frequency of relapses in patients receiving various forms of long-term immunotherapy for MOGAD. Inclusion criteria were history of ≥1 CNS demyelinating attacks, MOG-IgG seropositivity, and immunotherapy for ≥6 months. Patients were reviewed for CNS demyelinating attacks before and during long-term immunotherapy.ResultsSeventy patients were included. The median age at initial CNS demyelinating attack was 29 years (range 3–61 years; 33% <18 years), and 59% were female. The median annualized relapse rate (ARR) before treatment was 1.6. On maintenance immunotherapy, the proportion of patients with relapse was as follows: mycophenolate mofetil 74% (14 of 19; ARR 0.67), rituximab 61% (22 of 36; ARR 0.59), azathioprine 59% (13 of 22; ARR 0.2), and IV immunoglobulin (IVIG) 20% (2 of 10; ARR 0). The overall median ARR on these 4 treatments was 0.3. All 9 patients treated with multiple sclerosis (MS) disease-modifying agents had a breakthrough relapse on treatment (ARR 1.5).ConclusionThis large retrospective multicenter study of patients with MOGAD suggests that maintenance immunotherapy reduces recurrent CNS demyelinating attacks, with the lowest ARR being associated with maintenance IVIG therapy. Traditional MS disease-modifying agents appear to be ineffective. Prospective randomized controlled studies are required to validate these conclusions.

Purpose To estimate the proportion of patients presenting with isolated third, fourth or sixth cranial nerve palsies of presumed microvascular origin versus other causes. Design Prospective, multi-center observational case series. Participants One hundred and nine patients, 50 years of age or older with acute isolated ocular motor nerve palsy. Testing Magnetic resonance imaging (MRI) of the brain Main outcome measures Causes of acute isolated ocular motor nerve palsy (presumed microvascular or other) as determined with early MRI and clinical assessment. Results Among 109 patients enrolled in the study, there were 22 patients with cranial nerve III palsy, 25 patients with cranial nerve IV palsy and 62 patients with cranial nerve VI palsy. A cause other than presumed microvascular ischemia was identified in 18 patients (16.5 %, 95% confidence interval (CI): 10.7–24.6%). The presence of one or more vasculopathic risk factors (diabetes, hypertension, hypercholesterolemia, coronary artery disease, myocardial infarction, stroke and smoking) was significantly associated with a presumed microvascular cause (p=0.003, Fisher’s exact test). Vasculopathic risk factors were also present in 61% of patients (11/18) with other causes. In the group of patients who had vasculopathic risk factors only, with no other significant medical condition, 10% of patients (8/80) were found to have other causes including midbrain infarction, neoplasms, inflammation, pituitary apoplexy and giant cell arteritis (GCA). Excluding patients with third cranial nerve palsies and those with GCA the incidence of other causes for isolated fourth and sixth cranial nerve palsies was 4.7% (3/64). Conclusions In our series of patients with acute isolated ocular motor nerve palsies, substantial proportion of patients had other causes including neoplasm, GCA and brainstem infarction. Brain MRI and laboratory work–up has a role in the initial evaluation of older patients with isolated acute ocular motor nerve palsies regardless of whether vascular risk factors are present or not.

Background: During the COVID-19 pandemic, telehealth modalities have come to prominence as a strategy for providing patient care when in-person care provision opportunities are limited. The degree of adoption by neuroophthalmologists has not been quantified. Methods: Telehealth utilization pre-COVID-19 and peri-COVID-19 was surveyed among practicing neuroophthalmologists in and outside the United States using an online platform. Demographics, perceived benefits, barriers, and utility for different neuro-ophthalmic conditions were collected. Data collection occurred over a 2-week period in May 2020. Results: Two hundred eight practicing neuroophthalmologists (81.3% United States, 50.2% females, age range ,35 to .65, mode 35-44 years) participated in the survey. Utilization of all telehealth modalities increased from pre-COVID to peri-COVID (video visit 3.9%-68.3%, P , 0.0005, remote interpretation of testing 26.7%-32.2%, P = 0.09, online second opinion 7.9%-15.3%, P = 0.001, and interprofessional e-consult 4.4%-18.7%, P , 0.0005, McNemar). The majority selected access, continuity, and patient efficiency of care as benefits and data quality as a barrier. Telehealth was felt to be most helpful for conditions relying on history, external examination, and previously collected ancillary testing and not helpful for conditions requiring funduscopic examination. Conclusions: Telehealth modality usage by neuroophthalmologists increased during the COVID-19 pandemic. Identified benefits have relevance both during and beyond COVID-19. Further work is needed to address barriers in their current and future states to maintain these modalities as viable care delivery options.