Figure 2 NMDAR-Ab levels, clinical syndromes, and therapy in 8 informative patients with white matter syndromes in association with NMDAR-Ab NMDAR-Ab levels,

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Figure 3 Brain MRI findings in patients with MOG-Ab Extensive brain lesions with large diameter (A and B), posterior reversible encephalopathy–like lesions.

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Figure 2 GlyR antibody binding

Figure 2 Association of serum IgG reactivity with MRI measures of disease severity Association of serum IgG reactivity with MRI measures of disease severity.

Figure 2 Serums of patients with stiff-person syndrome spectrum disorders containing GlyRα1-Binding IgG specifically induces internalization of GlyRα1.

Figure 4 Correlation of age with [11C](R)-PK11195 binding in the normal-appearing white matter (NAWM) and thalami Correlation of age with [11C](R)-PK11195.

Figure 4 Proposed therapy scheme for anti-LGI1 encephalitis

Figure 1 Percent positivity by clinical feature Overall, 6

Figure Brain MRI of the patient throughout the disease course(A) Brain MRI at the time of cerebral toxoplasmosis diagnosis (a) and after 1 month of toxoplasmosis.

Figure 2 Temporal distribution of MOG antibody in serum of 2 relapsing patients with demyelinating diseases Temporal distribution of MOG antibody in serum.

Figure Clinical course of acute neuritis and NMDA receptor (NMDAR) encephalitis, sural nerve biopsy, and detection of NMDAR antibodies(A) Approximately.

Figure 4 Relation of neuropsychological deficits and intrathecal immune cell subsets in GABAB receptor antibody–associated limbic encephalitis Relation.

Figure 3 Immune response to neoantigen: Geometric mean titers of antirabies antibody levels over timeAt days 31 and 38, all subjects achieved antibody.

Figure 2 Expression of GABAA receptor and LGI1 by patient's thymomaTissue sections of the patient's thymoma incubated with biotinylated immunoglobulin.

Figure 3 Multifocal visual-evoked potentials in optic neuritis Figure shows the visual-evoked potentials (VEPs) in 52 sectors of the retina. Multifocal.

Figure. Patient imaging

Figure 1 Reactivity of the patients' antibodies with rat brain and HEK cell-based assays Rat hippocampal dentate gyrus neuropils were stained with patient.

Figure 1 Flow diagram of the assays and the samples that were evaluatedA total of 1,109 samples were initially screened at a serum dilution of 1:20 for.

Figure 2 Clinical course and response to therapy in 6 Australian patients with HMGCR antibodies The clinical course, creatine kinase (CK) levels, Medical.

Figure 1 MRI, pathology, and EEG findings(A) Axial fluid-attenuated inversion recovery (FLAIR) MRI sequences of the brain showing right frontal and parietal.

Figure 1 Evolution of visual function after acute optic neuritis Figure shows the measurement of high-contrast visual acuity (VA) using the Early Treatment.

Figure 3 Gene expression in CSF cell pellets

Figure 2 Correlation between total IgG levels and anti-AQP4 IgG titer

Figure 1 8-Iso-PGF2α levels in CSF of patients with MS and controlsCSF 8-iso-prostaglandin F2α (8-iso-PGF2α) levels were estimated using an ELISA. (A)

Figure 3 EEG demonstrating ictal seizure discharges in a patient with faciobrachial dystonic seizures The EEG of a 56-year-old woman with faciobrachial.

Figure 2 Concordance of results for commercial cell-based assay (CBA) and fluorescence-activated cell sorting (FACS) assays, FACS titers, and disease activity.

Figure. Groups 1–3, patients tested, and test results (viral PCR and antibodies)‏ Groups 1–3, patients tested, and test results (viral PCR and antibodies)

Figure 1 White matter lesion central vein visibility in MS and absence in small vessel disease (SVD)‏ White matter lesion central vein visibility in MS.

Figure 3. Brain imaging and neuropathologic studies in patient PT-5 diagnosed with progressive multifocal leukoencephalopathy Brain imaging and neuropathologic.

Figure 1 Evolution of blood cell counts during 18-month treatment and follow-up (A) Mean white blood cell count, (B) mean lymphocyte count, (C) mean eosinophil.

Figure 4 Pattern of relapse in patients with MOG-Ab Five myelin oligodendrocyte glycoprotein antibody (MOG-Ab)–positive patients experienced a relapse,

Figure 4 Aquaporin-4 immunoglobulin G (AQP4-IgG) index in time-matched paired serum-CSF specimens: 3 attack/preattack pairs and 7 bridge/remission pairs.

Figure 1 Patients with acute anti–NMDA receptor encephalitis have marked hypometabolism of the visual cortical brain region correlating with the medial.

Figure 4 Confirmatory cohorts to assess MOG-IgG1 assay(A) All 81 aquaporin-4 (AQP4)- seropositive patients (blue) from the Oxford National neuromyelitis.

Figure Clinical and radiologic course(A) The T2 contrast-enhanced sequence on day 3 shows an extensive central cord lesion extending from C2 to T7. Clinical.

Figure Time course of the patient's diagnostics and treatment

Figure 2 Relative concentration (in mAb equivalents) of JCV Ab in samples tested prior to IVIg (“pre-IVIg”), within 30 days of IVIg (“during IVIg”), and.

Figure 1. Antibodies to MOG in a proportion of adult patients with MS

Figure 1 Distribution of MOG IgG antibody in pediatric demyelinating diseases Distribution of MOG IgG antibody in pediatric demyelinating diseases (A)

Figure 1 Annual trend in specimen type submitted as first sample for aquaporin-4 immunoglobulin G testing (serum only vs CSF only vs both) from 101,065.

Figure 1 Clinical findings and CNS immunoreactivity of CSF IgA of a patient with limbic encephalitis (A) Cranial MRI of a 69-year-old man with limbic encephalitis.

Figure 1 B cells and plasma cells accumulate in the CSF in GABAB receptor antibody–associated LE B cells and plasma cells accumulate in the CSF in GABAB.

Figure 1 Examples illustrating gating strategy for fluorescence-activated cell sorting (FACS)‏ Examples illustrating gating strategy for fluorescence-activated.

Figure 1 Association between serum levels of IL-18 and hippocampal volume in patients with schizophrenia Scatter plots show a positive correlation between.

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Figure 1 Relapse and rituximab historyThe figure shows rituximab (RTX) treatment history as well as relapse history for 100 days prior to the first RTX.

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Figure Multiple MRI abnormalities as correlates of diverse adverse events and neurologic impairment during the course of severe NMDAR-E Multiple MRI abnormalities.

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Figure 1 Radiologic features of patients with white matter syndromes in association with NMDA receptor antibodies Radiologic features of patients with.

Figure 2 Summary of the utility of MOG-Abs and OCB testing in predicting pediatric disease course at onset compared to clinical follow-up at 1 yearFollowing.

Figure Imaging, histology/immunohistochemistry, and schematic course of treatment with corresponding clinical and radiologic disease activity Imaging,

Figure Forty-two months of follow-up of clinical symptoms in parallel with therapeutic interventions and antibody titers/B-cell count Forty-two months.

Figure. Model supporting noradrenergic signaling in stiff-person syndrome (SPS) pathophysiology and clinical exacerbations Model supporting noradrenergic.

Figure 1 Full-length MOG cell-based assay using a serum dilution of 1:160 as a cutoff for positivity (red line in both plots)(A) Myelin olidgodendrocyte.

Figure 3 Effect of IVIg on endogenous relative concentration (in mAb equivalents) of JCV AbSix patients shown in this figure have had John Cunningham virus.

Figure Clinical course and overview of the treatment protocols♦ = neuroradiologic evidence of new disease activity; ★ = CD19+ reconstitution. Clinical.

Figure Spinal cord imaging (A, B) Sagittal and axial T2-weighted cervical spine MRI demonstrating hyperintensities in the central gray matter of patient.

Figure 1 Classical pathway and lectin pathway activity in patients with multifocal motor neuropathy and controls Classical pathway (CP) activity (A) and.

Figure 2 Overview of apheresis therapies

Figure 2 Detection of atypical anti-neuronal antibodies Immunohistofluorescence assay on rat brain sagittal slices incubated with the patient's CSF and.

Figure 1 Follow-up periods of 33 anti–3-hydroxy-3-methylglutaryl coenzyme A reductase autoantibody (HMGCR Ab+) myopathy patients in relation to cancer.

Figure Clinical course and CSF/serum NMDA receptor (NMDAR) antibody (ab) titers of mother and infant Titers were measured with immunohistochemistry of.

Figure 2 Cell-based assay demonstrating differential binding of AChR antibodies to the adult and fetal receptorsThe fetal (gamma subunit specific) and.

Figure 1 Cell gating and binding curve from FACS experiments and M23 and M1 antibody titers during relapses and remission Cell gating for fluorescence-activated.

Figure 1 MRIs (case 1)‏ MRIs (case 1) An enlarging T2 lesion in the cerebral white matter near the angular gyrus and a new lesion in the left middle cerebellar.

Figure A 57-year-old man with relapsing-remitting MS (RRMS) and new-onset ataxia A 57-year-old man with relapsing-remitting MS (RRMS) and new-onset ataxia.

Figure 1 Numbers/seropositivity rates of IVIg-naive and IVIg-exposed STRATIFY-2 enrollees* = % of enrollment samples, ** = date of IVIg and/or concentration.

Figure 3 A receiver operating characteristic curve of days to IVMP as a predictor of failure to regain 0.2 logMAR (20/30) vision (AUC 0.84, p < 0.001)‏

Figure 1 Standard treatment scheme and antibody titer changes

Presentation transcript:

Figure 2 NMDAR-Ab levels, clinical syndromes, and therapy in 8 informative patients with white matter syndromes in association with NMDAR-Ab NMDAR-Ab levels, clinical syndromes, and therapy in 8 informative patients with white matter syndromes in association with NMDAR-Ab (A–C) Cases 1–3 presented initially with brainstem encephalitis; Cases 1 and 2 relapsed with encephalopathy, psychiatric features, movement disorder, and dysautonomia. Case 2 had additional seizures. In both, the diagnosis of NMDAR-Ab encephalitis was made at the time of relapse. Case 3 had a monophasic illness and did not have any of the clinical characteristics of NMDAR-Ab encephalitis. (D, E) Cases 4 and 5 presented with herpes simplex virus encephalitis (HSVE) and then had a neurologic relapse, which correlated with raised NMDAR-Abs in both serum and CSF and demonstrated a clinical response to immunotherapy with reduction of antibody levels. (F) Case 8 presented with optic neuritis and poor visual recovery, which prompted a neuroinflammatory screen and the identification of the antibody positivity. She only received a course of steroids 1 year into her illness and 3 years later had a neurologic relapse. (G) Case 9 presented initially with 2 episodes of acute disseminated encephalomyelitis (ADEM) and relapsed at 1 year with optic neuritis. He was also MOG-Ab positive, which remain detectable even when NMDAR-Abs are no longer detectable and when the patient had clinically recovered. (H) Case 10 had recurrent episodes of hyperventilation, dizziness, and double vision; did not receive any treatment; and both her clinical and radiologic features remained unchanged. Ab = antibody; CSF Ab × 10 = NMDAR antibody titers in CSF (all between 1:20 and 1:50) multiplied by 10 to provide visibility in comparison to serum levels; IVIg = IV immunoglobulin; IVMP = IV methylprednisolone; LeukoE = radiologic leukoencephalopathy; Lt = left; MMF = mycophenolate mofetil; NMDAR = NMDA receptor; ON = optic neuritis; PLEX = plasma exchange; Rt = right; Serum Ab titer = titer measured by endpoint dilution. Yael Hacohen et al. Neurol Neuroimmunol Neuroinflamm 2014;1:e2 © 2014 American Academy of Neurology