108 results match your criteria: "Neuroimmunology Clinic[Affiliation]"

Article Synopsis
  • MOG antibodies are linked to specific types of CNS demyelinating syndromes that differ from multiple sclerosis and aquaporin-4-seropositive neuromyelitis optica spectrum disorder.* -
  • A proposed set of diagnostic criteria for MOG antibody-associated disease (MOGAD) emphasizes that the presence of MOG-IgG is crucial and typically associates with conditions like acute disseminated encephalomyelitis, optic neuritis, or transverse myelitis.* -
  • Validating these criteria could enhance the diagnosis of MOGAD, which is important for understanding long-term clinical outcomes and for refining clinical trial criteria, though not all multiple sclerosis patients need to be tested for MOG-IgG.*
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The repertoire of CSF antiviral antibodies in patients with neuroinflammatory diseases.

Sci Adv

January 2023

Viral Immunology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA.

Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system (CNS). Although various viruses have been proposed to contribute to MS pathology, the etiology of MS remains unknown. Since intrathecal antibody synthesis is well documented in chronic viral infection and neuroinflammatory diseases, we hypothesized whether the patterns of antigen-specific antibody responses associated with various viral exposures may define patients with CNS chronic immune dysregulation.

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There are clinical and radiological phenotypes characteristic of neurosarcoidosis. Histopathologic confirmation is preferred, however, biopsy is associated with a significant risk of morbidity when only eloquent neural structures are involved and where there is no systemic disease. We present a series of patients with isolated neurosarcoidosis and suggest circumstances where an empirical, closely monitored, trial of tumour-necrosis-factor-alpha inhibitor therapy can improve outcome and diagnostic confidence.

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Visual evoked potentials (VEP) index visual pathway functioning, and are often used for clinical assessment and as outcome measures in people with multiple sclerosis (PwMS). VEPs may also reflect broader neural disturbances that extend beyond the visual system, but this possibility requires further investigation. In the present study, we examined the hypothesis that delayed latency of the P100 component of the VEP would be associated with broader structural changes in the brain in PwMS.

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The diagnostic signature of Susac's syndrome.

J Clin Neurosci

October 2022

Neuroimmunology Clinic, Concord Repatriation General Hospital, University of Sydney, Australia; Brain & Mind Centre, University of Sydney, NSW, Australia. Electronic address:

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The purpose of the present study is to evaluate the efficacy of combination immunotherapy with Propes and Inflamafertin in GDFC adults with natural killer (NK) and/or natural killer T-lymphocyte (NKT) cell deficiency. This single-centre, retrospective, controlled, non-randomized clinical trial analysed medical records of 212 adult GDFC patients aged 19-50 years (study group [SG]). SG received Propes at a dose of 2 ml intramuscularly every other day at night for three consecutive months and Inflamafertin at a dose of 2 ml IM every other day at night for three consecutive months in rotation with Propes.

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Co-expression of anti-NMDAR and anti-GAD65 antibodies. A case of autoimmune encephalitis in a post-COVID-19 patient.

Neurologia (Engl Ed)

July 2022

Department of Internal Medicine, Hospital de Especialidades "Dr. Antonio Fraga Mouret", Centro Médico Nacional La Raza, Mexico City, Mexico; Division of Postgraduate Studies, Universidad Nacional Autonoma de Mexico City, Mexico, Mexico. Electronic address:

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Background: Lytic infection of oligodendrocytes by the human JC polyomavirus (JCPyV) results in the demyelinating disease called progressive multifocal leukoencephalopathy (PML). The detection of viral DNA in the cerebrospinal fluid (CSF) by PCR is an important diagnostic tool and, in conjunction with defined radiological and clinical features, can provide diagnosis of definite PML, avoiding the need for brain biopsy. The main aim of this study is to compare the droplet digital PCR (ddPCR) assay with the gold standard quantitative PCR (qPCR) for the quantification of JC viral loads in clinical samples.

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Background: The "central vein sign" (CVS), a linear hypointensity on T2*-weighted imaging corresponding to a central vein/venule, is associated with multiple sclerosis (MS) lesions. The effect of lesion-size exclusion criteria on MS diagnostic accuracy has not been extensively studied.

Objective: Investigate the optimal lesion-size exclusion criteria for CVS use in MS diagnosis.

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Background: Haemorrhagic demyelinating lesions are rare, and little is known about the demyelinating diseases with which they are associated, or how lesional haemorrhage affects treatment and outcomes.

Objective: To examine the clinical characteristics and outcomes of patients with demyelinating lesions and magnetic resonance imaging (MRI) evidence of haemorrhage seen at the Mayo clinic between 1990 and 2018.

Methods: The Mayo Clinic's medical-record diagnostic-linkage system was used to identify patients with CNS demyelinating disease and parenchymal haemorrhage on brain MRI cross-referenced against a database of patients with pathologically confirmed CNS demyelinating disease.

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Longitudinal assessment of the relationship between visual evoked potentials and cognitive performance in multiple sclerosis.

Clin Neurophysiol

May 2022

South Shore Neurologic Associates, 712 Main Street, Islip, Patchogue, NY, USA. Electronic address:

Objective: Visual evoked potentials (VEPs) can provide insight into disease activity in people with multiple sclerosis (PwMS). However, few studies have tracked concurrent changes in VEPs and cognitive functioning over time in MS. To address this, we examined the longitudinal relationship between VEP and cognitive performance in PwMS over a two-year period.

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MS-Driven Metabolic Alterations Are Recapitulated in iPSC-Derived Astrocytes.

Ann Neurol

May 2022

Transplantation Immunobiology Laboratory, Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, São Paulo, Brazil.

Article Synopsis
  • Astrocytes play a crucial role in multiple sclerosis (MS) pathology, prompting a study to analyze these cells from patients rather than relying solely on mouse models.
  • Researchers used induced pluripotent stem cell-derived astrocytes from patients with MS and healthy controls to evaluate mitochondrial function and metabolism through various techniques.
  • The study found significant differences in MS astrocytes, including alterations in gene expression linked to neurodegeneration, increased oxidative stress markers, and distinct metabolic changes, providing new insights for future MS therapies.
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Central Vein Sign Profile of Newly Developing Lesions in Multiple Sclerosis: A 3-Year Longitudinal Study.

Neurol Neuroimmunol Neuroinflamm

March 2022

From the Translational Neuroradiology Section (O.A.-L., V.L., S.M., E.S.B., P.S., D.S.R.), National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD; Department of Neurology (O.A.-L., P.S.), Cedars-Sinai Medical Center, Los Angeles, CA; Section on Neural Function (S.R.), National Institute of Mental Health, NIH, Bethesda, MD; Neuroimmunology Clinic (J.O., I.C.), National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD; and Center for Neuroscience and Regenerative Medicine (D.L.P.), the Henry M. Jackson Foundation for the Advancement of Military Medicine, Bethesda, MD.

Background And Objectives: The central vein sign (CVS), a central linear hypointensity within lesions on T2*-weighted imaging, has been established as a sensitive and specific biomarker for the diagnosis of multiple sclerosis (MS). However, the CVS has not yet been comprehensively studied in newly developing MS lesions. We aimed to identify the CVS profiles of new white matter lesions in patients with MS followed over time and investigate demographic and clinical risk factors associated with new CVS+ or CVS- lesion development.

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Background And Purpose: Miller Fisher syndrome (MFS) is a subtype of Guillain-Barré syndrome characterized by the triad of ophthalmoparesis, areflexia, and ataxia. Although cases of MFS have been associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, no studies have synthesized the clinical characteristics of patients with this condition.

Methods: In this rapid systematic review, we searched the PubMed database to identify studies on MFS associated with SARS-CoV-2 infection.

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Clinical trial of raltegravir, an integrase inhibitor, in HAM/TSP.

Ann Clin Transl Neurol

October 2021

Viral Immunology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, 20892, USA.

Objective: Human T-cell lymphotropic virus 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic, progressive myelopathy. A high proviral load (PVL) is one of the main risk factors for HAM/TSP. Recently, it was shown that raltegravir could inhibit cell-free and cell-to-cell transmission of HTLV-1 in vitro.

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Complement component 3 from astrocytes mediates retinal ganglion cell loss during neuroinflammation.

Acta Neuropathol

November 2021

Division of Neuroimmunology and Neurological Infections, Johns Hopkins Hospital, Pathology Building 509, 600 N. Wolfe St., Baltimore, Md, 21287, USA.

Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system (CNS) characterized by varying degrees of secondary neurodegeneration. Retinal ganglion cells (RGC) are lost in MS in association with optic neuritis but the mechanisms of neuronal injury remain unclear. Complement component C3 has been implicated in retinal and cerebral synaptic pathology that may precede neurodegeneration.

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7T MRI Differentiates Remyelinated from Demyelinated Multiple Sclerosis Lesions.

Ann Neurol

October 2021

Translational Neuroradiology Section, National Institute of Neurological disorders and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, MD.

Objective: To noninvasively assess myelin status in chronic white matter lesions of multiple sclerosis (MS), we developed and evaluated a simple classification scheme based on T1 relaxation time maps derived from 7-tesla postmortem and in vivo MRI.

Methods: Using the MP2RAGE MRI sequence, we classified 36 lesions from 4 postmortem MS brains as "long-T1," "short-T1," and "mixed-T1" by visual comparison to neocortex. Within these groups, we compared T1 times to histologically derived measures of myelin and axons.

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BK virus-specific T cells for immunotherapy of progressive multifocal leukoencephalopathy: an open-label, single-cohort pilot study.

Lancet Neurol

August 2021

Hematology Branch, Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA; Herbert Irving Comprehensive Cancer Center, Columbia University Medical Center, New York, NY, USA.

Background: Progressive multifocal leukoencephalopathy, a rare disease of the CNS caused by JC virus and occurring in immunosuppressed people, is typically fatal unless adaptive immunity is restored. JC virus is a member of the human polyomavirus family and is closely related to the BK virus. We hypothesised that use of partly HLA-matched donor-derived BK virus-specific T cells for immunotherapy in progressive multifocal leukoencephalopathy would be feasible and safe.

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Cervical and thoracic cord atrophy in multiple sclerosis phenotypes: Quantification and correlation with clinical disability.

Neuroimage Clin

July 2021

Viral Immunology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, United States. Electronic address:

Objective: We sought to characterize spinal cord atrophy along the entire spinal cord in the major multiple sclerosis (MS) phenotypes, and evaluate its correlation with clinical disability.

Methods: Axial T-weighted images were automatically reformatted at each point along the cord. Spinal cord cross-sectional area (SCCSA) were calculated from C1-T10 vertebral body levels and profile plots were compared across phenotypes.

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Underlying mechanisms on the association between severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and neurologic complications are still poorly understood. Cases of Guillain-Barré Syndrome (GBS) have been linked to the SARS-CoV-2 infection as the result of dysregulated immune response with damage in neuronal tissues. In the current report, we present the first pediatric case of GBS with detection of SARS-CoV-2 in the cerebrospinal fluid (CFS).

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SASH3 variants cause a novel form of X-linked combined immunodeficiency with immune dysregulation.

Blood

September 2021

Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD.

Article Synopsis
  • The SASH3 protein is an adaptor involved in orchestrating signaling in lymphocytes, and its gene is located on the X-chromosome.
  • Researchers found 3 harmful mutations in the SASH3 gene in 4 unrelated male patients who displayed symptoms of combined immunodeficiency and immune dysregulation, leading to infections and autoimmune issues.
  • The study reveals that SASH3 is crucial for proper lymphocyte function and survival, suggesting a new form of X-linked combined immunodeficiency in humans similar to defects seen in mouse models.
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Effect of Teriflunomide on Cells From Patients With Human T-cell Lymphotropic Virus Type 1-Associated Neurologic Disease.

Neurol Neuroimmunol Neuroinflamm

May 2021

From the Viral Immunology Section (Y.E.-A., N.N., S.J.), National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD; Joan Ohayon, Neuroimmunology Clinic (J.O.), National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD; and Sanofi (M.M., J.C., T.J.T.), Cambridge, MA.

Objective: To test the hypothesis that teriflunomide can reduce ex vivo spontaneous proliferation of peripheral blood mononuclear cells (PBMCs) from patients with human T-cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP).

Methods: PBMCs from patients with HAM/TSP were cultured in the presence and absence of teriflunomide and assessed for cell viability, lymphocyte proliferation, activation markers, HTLV-1 and HTLV-1 messenger ribonucleic acid (mRNA) expression, and HTLV-1 Tax protein expression.

Results: In culture, teriflunomide did not affect cell viability.

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