Human T-lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a progressive neurologic disorder characterized by chronic inflammation in the CNS. HTLV-1-specific cytotoxic CD8 T lymphocytes (CTLs) play a crucial role in the pathogenesis of HAM/TSP; however, the dynamics of CTLs in the CSF remain poorly understood. The aim of this study was to investigate the accumulation of HTLV-1-specific CTLs in the CSF of patients with HAM/TSP and to evaluate their association with neuroinflammation and neural damage.

Optical coherence tomography angiography (OCTA) is widely used for evaluating retinal vessels. The aim of this study was to assess the influence of OCTA quality, as measured by the previously introduced OSCAR-MP criteria, on OCTA outcome parameters and the reliability of test-retest results.

Chimeric antigen receptor (CAR) T cells are genetically modified T cells expressing CARs, initially developed to recognize tumor antigens and kill cancer cells that evade T-cell recognition. Because of their impressive success in hemato-oncologic malignancies, CAR T cells are being repurposed with redesigned constructs for safety and sustained efficacy to target refractory systemic autoimmune or neurologic diseases. The CD19 CAR T cells-targeting those CD19-positive, antibody-secreting, long-lived plasma cells, and plasmablasts-are now extensively explored in refractory neuroautoimmunities with promising benefits based on case series in patients with myasthenia gravis (MG), stiff person syndrome (SPS), neuromyelitis, myositis, and multiple sclerosis; some patients with MG and SPS with steadily progressive and disabling disease refractory to all available therapies, including rituximab and new biologics, exhibit impressive clinical improvements with long-lasting benefits. The review, triggered by these early results and ongoing trials, addresses what these cells are and why they show effectiveness not only in antibody-mediated B-cell neurologic diseases unresponsive to available anti-B-cell agents but also in patients with nonpathogenic antibodies, implying effects even beyond B cells; points out that CARs are "living cells" penetrating physiologic barriers, such as the blood-brain barrier, expanding within tissues to memory cells ensuring sustained effects; describes the process and challenges of preparing and administering CAR T cells and their safety profile stressing the differences in toxicities when treating autoimmunites vs malignancies; and highlights that CD19 CAR T cells can successfully target even 2 different autoimmune diseases in the same patient, such as SPS and MG, offering promising prospects of changing the therapeutic algorithm in all neuroautoimmunities with potential for achieving even an immune reset shifting immunity to a healthy state.

Progressive multifocal leukoencephalopathy (PML) is a rare, often fatal CNS infection caused by reactivation of JC virus, typically in immunocompromised patients. No effective antiviral therapy has been established. We report a case of PML in a patient with multiple sclerosis (MS) treated with fingolimod, who received oral tenofovir alafenamide fumarate (TAF).

Autoimmune nodopathy with anti-contactin1 (CNTN1) autoantibodies is a rare sensory-motor neuropathy characterized by subacute-onset sensory ataxia and variable disease courses. Comorbidities such as glomerulonephritis and diabetes mellitus are observed in some patients. The diversity in clinical presentation may reflect differences in the targeted CNTN1 epitopes.

Immune effector cell-associated neurotoxicity syndrome (ICANS) is typically a monophasic condition with a variable onset after chimeric antigen receptor T-cell (CAR T-cell) therapy. We report an exceedingly rare case of late-onset ICANS relapse, highlighting challenges in its recognition and management.

Spinal cord leptomeningeal enhancement (LME) can be observed in children with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and with seronegative myelitis. We investigated whether the presence of spinal cord LME in MOGAD and seronegative myelitis is associated with distinct clinical, CSF, and MRI findings.

Antibody-associated neurologic diseases often present with movement disorders (MDs). The frequency and clinical course of specific MDs in most antibody-associated disease subtypes remain largely unknown.

Aquaporin (AQP)-4 (AQP4)-seropositive neuromyelitis optica (NMO) frequently coexists with rheumatologic autoimmune conditions, including Sjögren syndrome and systemic lupus erythematosus, 2 conditions that share a common association with NMO. AQP4 is a member of a family of ubiquitously expressed water channels. Its immunodominant pathogenic T-cell epitope, which binds MHC II with exceptionally high affinity, is homologous to sequences in other AQPs, including AQP5, a candidate target autoantigen in Sjögren syndrome. Thus, we hypothesized that self-antigen molecular mimicry exists between AQPs and contributes to autoimmunity.

Paramagnetic rim lesions (PRLs), visible on susceptibility-based imaging (SbI), reflect chronic active inflammation in multiple sclerosis (MS). In adult-onset MS, PRLs are associated with a more aggressive disease course.The objectives of this study were to assess the prevalence of PRLs in children with MS and to examine how baseline PRL count relates to clinical disability and brain tissue volume loss, both cross-sectionally and over short-term follow-up.

Multifocal motor neuropathy (MMN) is an asymmetric motor neuropathy driven by IgM autoantibodies targeting gangliosides, particularly GM1. In this study, we investigated the relationship between IgM seropositivity, complement activation, and clinical parameters using an induced pluripotent stem cell (iPSC)-derived motor neuron (MN) model of MMN.

In multiple sclerosis (MS), a variety of immunosuppressive treatments are available. While effective, these approaches often lead to sustained impairment of essential components of the immune system, posing long-term safety concerns. Consequently, there is a growing interest in alternative therapeutic approaches that selectively limit pathogenic B-cell functions while preserving their physiologic roles. In this study, we investigated the therapeutic potential of inhibiting the enzyme Bruton tyrosine kinase (BTK), a key signaling molecule in both B-cell and myeloid cell activation.

The aim of this study was to elucidate the clinical spectrum and mechanisms of autoimmune nodopathy (AN) with autoantibodies against leucine-rich repeat leucin-rich glioma inactivated 1 (LGI) family member 4 (LGI4) localized at juxtaparanodes and satellite glia in the dorsal root ganglion.

Intrathecal synthesis of immunoglobulin G (IgG) is a key feature of multiple sclerosis (MS) and a prognostic marker for the disease course. Although previous studies identified 2 genetic regions-the major histocompatibility complex (MHC) region on chromosome 6 and the immunoglobulin heavy chain constant (IGHC) locus on chromosome 14-associated with intrathecal IgG synthesis in MS, the genetic underpinnings remain insufficiently understood.

Differentiating multiple sclerosis (MS) from antibody (Ab)-defined diseases, such as neuromyelitis optica spectrum disorders (NMOSDs), remains challenging, particularly as Ab levels decline. -glycans play a key role in immunity, with changes in branching and fucosylation linked to T/B-cell function and MS onset while increased -acetylglucosamine residues correlate with disease progression. Despite growing recognition of glycosylation in neuroinflammation, direct comparisons of the -glycome between MS and Ab-defined diseases are lacking. This study aims to assess whether plasma -glycome profiling can effectively differentiate these conditions and their subtypes.

Understanding the immunologic changes induced by immune reconstitution therapies (IRTs) is key to optimizing multiple sclerosis (MS) treatment. We evaluate lymphocyte dynamics and their association with secondary autoimmune disease (SAD) and its recurrence after treatment with alemtuzumab (ALZ), autologous hematopoietic stem cell transplantation (AHSCT), and cladribine tablets (CladT).

Anti-leucine-rich glioma-inactivated 1 (anti-LGI1) encephalitis is the most common antibody-mediated encephalitis in adults older than 50 years. In addition to antibody effects, cytokines and chemokines drive neuroinflammation in other autoimmune encephalitides. However, their role in anti-LGI1 encephalitis is underexplored. We evaluated cytokine profiles in serum and CSF, correlating them with acute severity and long-term outcome.

Histiocytoses are diverse hematopoietic diseases with disabling neurologic involvement. Recently, targeted mitogen-activated protein kinase pathway inhibitors have been used with clinical and radiologic response; however, some patients are unable to tolerate these treatments or have isolated and/or refractory neurologic, ocular, or head and neck (NOHN) disease. Intra-arterial administration of chemotherapy has conferred favorable responses in various neoplasms; however, treatment and outcomes across histiocytosis subtypes have not been examined.