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View article: Treatment Preferences of Neurologists in Generalized Myasthenia Gravis: A Conjoint Analysis Study
Treatment Preferences of Neurologists in Generalized Myasthenia Gravis: A Conjoint Analysis Study Open
View article: Clinical Characterization and Long-Term Outcome in Children and Adults With Anti-AMPA Receptor Encephalitis
Clinical Characterization and Long-Term Outcome in Children and Adults With Anti-AMPA Receptor Encephalitis Open
Children and adults with anti-AMPAR encephalitis show distinct clinical-radiologic features. At long-term follow-up, 68% of patients, all adults, have neurologic sequelae, with failure to respond to first-line immunotherapy being associate…
View article: Brain Metabolite Levels in the Post-Acute Stage of Anti-NMDA Receptor Encephalitis and Schizophrenia: A Longitudinal Case-Control Study
Brain Metabolite Levels in the Post-Acute Stage of Anti-NMDA Receptor Encephalitis and Schizophrenia: A Longitudinal Case-Control Study Open
View article: Cerebrospinal 14-3-3 Protein Levels as a Neuroaxonal Biomarker in Aquaporin-4 Antibody–Positive Neuromyelitis Optica Spectrum Disorder
Cerebrospinal 14-3-3 Protein Levels as a Neuroaxonal Biomarker in Aquaporin-4 Antibody–Positive Neuromyelitis Optica Spectrum Disorder Open
This study provides Class IV evidence that in individuals presenting with acute myelitis, CSF 14-3-3 differentiates AQP4-NMOSD from MS or MOGAD with a sensitivity of 0.60 (0.30-0.80) and specificity of 0.95 (0.84-1.00).
View article: Clinical Features of Children With MOG-IgG Who Fulfill Criteria of Multiple Sclerosis and Overlapping Disorders
Clinical Features of Children With MOG-IgG Who Fulfill Criteria of Multiple Sclerosis and Overlapping Disorders Open
In this pediatric cohort, 4% of patients with MOG-IgG met criteria for MS. The clinical-radiologic spectrum ranged from typical MS to overlapping MOGAD-MS, and patients usually required high-efficacy DMT.
View article: Assessing Commercial Tissue-Based Assays for Autoimmune Neurologic Disorders (II)
Assessing Commercial Tissue-Based Assays for Autoimmune Neurologic Disorders (II) Open
The diagnostic performance of EUROIMMUN IIF-TBA in detecting NSAbs in autoimmune encephalitis is suboptimal. NMDAR antibodies, among the most common NSAbs, can be missed in 50% of cases. This commercial TBA should not be used alone as a sc…
View article: Assessing Commercial Tissue-Based Assays for Autoimmune Neurologic Disorders (I)
Assessing Commercial Tissue-Based Assays for Autoimmune Neurologic Disorders (I) Open
The performance of commercial IIF-TBAs for IC-Abs detection is suboptimal, exhibiting high false-negative rates of 25%-35%. Therefore, commercial TBAs should not be used alone for IC-Abs screening, but alongside more sensitive techniques, …
View article: ROHHAD syndrome in an adult: presence of ZSCAN1 antibodies
ROHHAD syndrome in an adult: presence of ZSCAN1 antibodies Open
Detection of ZSCAN1-abs in a ROHHAD syndrome case with an adult debut https://bit.ly/4hkcChb.
View article: Assessing therapeutic decisions in generalized myasthenia gravis: Study protocol
Assessing therapeutic decisions in generalized myasthenia gravis: Study protocol Open
Background The therapeutic landscape in generalized myasthenia gravis (gMG) has been continuously evolving in recent years, with over five products approved, each with different efficacy, safety, and administration profiles. With the avail…
View article: Novel Approaches to Treatment of Immune-Mediated Chronic Intestinal Pseudo-Obstruction
Novel Approaches to Treatment of Immune-Mediated Chronic Intestinal Pseudo-Obstruction Open
Our findings underscore the potential efficacy of rituximab and vedolizumab in the management of autoimmune CIPO refractory to first-line treatments.
View article: Complement Activation Profiles Predict Clinical Outcomes in Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease
Complement Activation Profiles Predict Clinical Outcomes in Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease Open
Our results suggest that serum and CSF levels of CFs have diagnostic and prognostic value respectively in patients with MOGAD. These findings support the use of complement inhibitors as a therapeutic approach in these patients.
View article: Long‐Term Follow Up in Anti‐Contactin‐1 Autoimmune Nodopathy
Long‐Term Follow Up in Anti‐Contactin‐1 Autoimmune Nodopathy Open
Objective To analyze long‐term clinical and biomarker features of anti‐contactin‐1 (CNTN1) autoimmune nodopathy (AN). Methods Patients with anti‐CNTN1 + autoimmune nodopathy detected in our laboratory from which clinical information was av…
View article: Spectrum of Clinical and Imaging Features of Children With GFAP Astrocytopathy
Spectrum of Clinical and Imaging Features of Children With GFAP Astrocytopathy Open
GFAP-ab-associated diseases encompass a wide spectrum of clinical presentation associated with a particular set of MRI features clearly distinct to other antibody-mediated diseases or MOGAD. We recommend that testing for GFAP-abs in serum …
View article: 21538. ENCEFALITIS AUTOINMUNE PEDIÁTRICA: DIAGNÓSTICO DIFERENCIAL Y CARACTERIZACIÓN DE PACIENTES CON ANTICUERPOS NEGATIVOS
21538. ENCEFALITIS AUTOINMUNE PEDIÁTRICA: DIAGNÓSTICO DIFERENCIAL Y CARACTERIZACIÓN DE PACIENTES CON ANTICUERPOS NEGATIVOS Open
View article: 21574. RENDIMIENTO DE LOS NUEVOS CRITERIOS DIAGNÓSTICOS DE MOGAD EN ADULTOS Y NIÑOS
21574. RENDIMIENTO DE LOS NUEVOS CRITERIOS DIAGNÓSTICOS DE MOGAD EN ADULTOS Y NIÑOS Open
View article: 21521. CARACTERIZACIÓN CLÍNICA DE 64 PACIENTES CON ANTICUERPOS CONTRA LA PROTEÍNA ÁCIDA FIBRILAR GLIAL (GFAP)
21521. CARACTERIZACIÓN CLÍNICA DE 64 PACIENTES CON ANTICUERPOS CONTRA LA PROTEÍNA ÁCIDA FIBRILAR GLIAL (GFAP) Open
View article: 20274. NODOPATÍA AUTOINMUNE ANTI-CONTACTIN-1: CARACTERÍSTICAS CLÍNICAS, BIOMARCADORES Y SEGUIMIENTO A LARGO PLAZO
20274. NODOPATÍA AUTOINMUNE ANTI-CONTACTIN-1: CARACTERÍSTICAS CLÍNICAS, BIOMARCADORES Y SEGUIMIENTO A LARGO PLAZO Open
View article: CD19-Directed CAR T-Cells in a Patient With Refractory MOGAD
CD19-Directed CAR T-Cells in a Patient With Refractory MOGAD Open
This provides Class IV evidence. It is a single observational study without controls.
View article: Long term follow-up in anti-contactin-1 autoimmune nodopathy
Long term follow-up in anti-contactin-1 autoimmune nodopathy Open
Objective To analyze long-term clinical and biomarker features of anti-contactin-1 (CNTN1) autoimmune nodopathy (AN). Methods Patients with anti-CNTN1+ AN detected in our laboratory from which clinical information was available were includ…
View article: Antibodies Against ZSCAN1 in Pediatric and Adult Patients With Non-Paraneoplastic ROHHAD Syndrome
Antibodies Against ZSCAN1 in Pediatric and Adult Patients With Non-Paraneoplastic ROHHAD Syndrome Open
Patients with ROHHAD syndrome should be investigated for the presence of ZSCAN1-abs in CSF. The antibodies do not necessarily predict the presence of a tumor. The detection of ZSCAN1-abs in an adult patient suggests that this condition als…
View article: Very Long-Term Functional Outcomes and Dependency in Children With Anti-NMDA Receptor Encephalitis
Very Long-Term Functional Outcomes and Dependency in Children With Anti-NMDA Receptor Encephalitis Open
After a minimal follow-up of 5 years, most children with NMDARe had substantial or full functional recovery, but approximately one-fifth remained with behavioral and school/working deficits. The younger the patient at disease onset, the mo…
View article: Antibody Investigations in 2,750 Children With Suspected Autoimmune Encephalitis
Antibody Investigations in 2,750 Children With Suspected Autoimmune Encephalitis Open
The repertoire of antibodies in children with AE is different from that of the adults. Except for NMDAR and MOG antibodies, many of the antibodies included in diagnostic panels are rarely positive and their up-front testing in children see…
View article: mRNA COVID-19 Vaccination Does Not Exacerbate Symptoms or Trigger Neural Antibody Responses in Multiple Sclerosis
mRNA COVID-19 Vaccination Does Not Exacerbate Symptoms or Trigger Neural Antibody Responses in Multiple Sclerosis Open
In this study, mRNA COVID-19 vaccination was safe and did not exacerbate the autoimmune disease nor triggered neural autoantibodies or immune-mediated neurologic disorders. The outcome of patients who developed SARS-CoV-2 infection was fav…
View article: Significance of Myelin Oligodendrocyte Glycoprotein Antibodies in CSF
Significance of Myelin Oligodendrocyte Glycoprotein Antibodies in CSF Open
Paired serum and CSF MOG-Abs positivity are common in MOGAD and are associated with a more severe clinical presentation. CSF-only MOG-Abs positivity can occur in patients with a phenotype suggestive of MOGAD and is associated with a worse …
View article: The 2021 European Alliance of Associations for Rheumatology/American College of Rheumatology Points to Consider for Diagnosis and Management of Autoinflammatory Type I Interferonopathies: <scp>CANDLE</scp>/<scp>PRAAS</scp>, <scp>SAVI</scp>, and <scp>AGS</scp>
The 2021 European Alliance of Associations for Rheumatology/American College of Rheumatology Points to Consider for Diagnosis and Management of Autoinflammatory Type I Interferonopathies: <span>CANDLE</span>/<span>PRAAS</span>, <span>SAVI</span>, and <span>AGS</span> Open
Objective Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome‐associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STIN…
View article: The 2021 European Alliance of Associations for Rheumatology/American College of Rheumatology points to consider for diagnosis and management of autoinflammatory type I interferonopathies: CANDLE/PRAAS, SAVI and AGS
The 2021 European Alliance of Associations for Rheumatology/American College of Rheumatology points to consider for diagnosis and management of autoinflammatory type I interferonopathies: CANDLE/PRAAS, SAVI and AGS Open
View article: Efficacy of baricitinib on chronic pericardial effusion in a patient with Aicardi–Goutières syndrome
Efficacy of baricitinib on chronic pericardial effusion in a patient with Aicardi–Goutières syndrome Open
Dear Editor, Aicardi–Goutières syndrome (AGS) was initially described as an early-onset progressive encephalopathy with severe neurological symptoms, acquired microcephaly, basal ganglia calcification, leukoencephalopathy, cerebral atrophy…
View article: Impact of COVID-19 in Immunosuppressed Children With Neuroimmunologic Disorders
Impact of COVID-19 in Immunosuppressed Children With Neuroimmunologic Disorders Open
In this cohort of children with neuroimmunologic disorders, the frequency of COVID-19 was low and not affected by immunosuppressive therapies. The main risk factors for developing COVID-19 were having cohabitants with COVID-19 and low vita…
View article: Use and Safety of Immunotherapeutic Management of <i>N</i>-Methyl-<scp>d</scp>-Aspartate Receptor Antibody Encephalitis
Use and Safety of Immunotherapeutic Management of <i>N</i>-Methyl-<span>d</span>-Aspartate Receptor Antibody Encephalitis Open
Factors influencing functional outcomes and relapse are different and need to be considered independently in development of evidence-based optimal management guidelines of patients with NMDARE.
View article: International Consensus Recommendations for the Treatment of Pediatric NMDAR Antibody Encephalitis
International Consensus Recommendations for the Treatment of Pediatric NMDAR Antibody Encephalitis Open
These international consensus recommendations for the management of pediatric NMDARE aim to standardize the treatment and provide practical guidance for clinicians, rather than absolute rules. A similar recommendation could be applicable t…