Josep Dalmau
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View article: Cytokine and Chemokine Profiles in Anti-LGI1 Encephalitis
Cytokine and Chemokine Profiles in Anti-LGI1 Encephalitis Open
In this study, we show that the acute-phase IL-6 CSF/serum ratio and serum IL-35 levels in immunotherapy-naive patients with anti-LGI1 encephalitis are associated with disease severity and poor outcomes, respectively, highlighting their po…
View article: Autoimmune neuropsychiatric disorders manifesting with psychosis
Autoimmune neuropsychiatric disorders manifesting with psychosis Open
The increasing recognition of a new category of encephalitides that occur in association with antibodies against neuronal surface proteins has prompted the use of terms like "autoimmune psychosis" and "autoimmune psychiatric disorders." Ho…
View article: Progressive Encephalomyelitis With Rigidity and Myoclonus With Glycine Receptor Antibodies
Progressive Encephalomyelitis With Rigidity and Myoclonus With Glycine Receptor Antibodies Open
GlyR antibody-mediated PERM is a rapidly progressive and severe disease that predominantly affects men and frequently presents with brainstem involvement. Its distinct demographic and clinical features suggest that it should be considered …
View article: Autoimmune encephalitis
Autoimmune encephalitis Open
Autoimmune encephalitis (AE) is a treatable neuro-inflammatory disorder that is increasing in incidence. AE can be associated with malignancy (paraneoplastic), but in many patients no tumour is present. The disease presentation of AE can b…
View article: Caregivers’ perspectives on long-term cognitive, psychiatric, psychological, and social outcomes in NMDAR encephalitis: Initial findings from the multi-center prospective SAPIENCE study
Caregivers’ perspectives on long-term cognitive, psychiatric, psychological, and social outcomes in NMDAR encephalitis: Initial findings from the multi-center prospective SAPIENCE study Open
The SAPIENCE study explores the long-term psychological, social, and systemic challenges faced by caregivers of individuals with NMDAR encephalitis. It aims to inform patientand caregiver-centered strategies that support recovery and wellb…
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: Autologous Hematopoietic Stem Cell Transplantation for Paraneoplastic Cerebellar Degeneration
Autologous Hematopoietic Stem Cell Transplantation for Paraneoplastic Cerebellar Degeneration Open
This is a single observational study without controls and provides Class IV evidence.
View article: Brain atrophy patterns in anti-IgLON5 disease
Brain atrophy patterns in anti-IgLON5 disease Open
Anti-IgLON5 disease is an autoimmune encephalitis that presents with a heterogenous clinical phenotype, including sleep disorders, movement abnormalities and bulbar involvement. It is characterised by autoantibodies against IgLON5, 85% ass…
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: 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: Acknowledgment to Reviewers
Acknowledgment to Reviewers Open
View article: Animal models of autoimmune encephalitis
Animal models of autoimmune encephalitis Open
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: A Phase-2B Double-Blind Randomized International Prospective Trial of Inebilizumab in NMDAR Encephalitis: The ExTINGUISH Trial
A Phase-2B Double-Blind Randomized International Prospective Trial of Inebilizumab in NMDAR Encephalitis: The ExTINGUISH Trial Open
The ExTINGUISH trial will determine the safety and efficacy of inebilizumab as a treatment of NMDAR encephalitis while evaluating the utility of clinical, cognitive, and quality-of-life outcome measures and supporting standardized collecti…
View article: Ten Years of <i>Neurology</i> ® <i>Neuroimmunology & Neuroinflammation</i>
Ten Years of <i>Neurology</i> ® <i>Neuroimmunology & Neuroinflammation</i> Open
View article: Anti-IgLON5 Disease 10 Years Later
Anti-IgLON5 Disease 10 Years Later Open
Anti-IgLON5 disease was identified 10 years ago, thanks to the discovery of IgLON5 antibodies and the joint effort of specialists in sleep medicine, neuroimmunology, and neuropathology. Without this collaboration, it would have been imposs…
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: Neuropathological spectrum of anti-IgLON5 disease and stages of brainstem tau pathology: updated neuropathological research criteria of the disease-related tauopathy
Neuropathological spectrum of anti-IgLON5 disease and stages of brainstem tau pathology: updated neuropathological research criteria of the disease-related tauopathy Open
Anti-IgLON5 disease is a unique condition that bridges autoimmunity and neurodegeneration. Since its initial description 10 years ago, an increasing number of autopsies has led to the observation of a broader spectrum of neuropathologies u…
View article: Assessment of long-term psychosocial outcomes in N-methyl-D-aspartate receptor encephalitis – the SAPIENCE study protocol
Assessment of long-term psychosocial outcomes in N-methyl-D-aspartate receptor encephalitis – the SAPIENCE study protocol 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: Neuro-immunobiology and treatment assessment in a mouse model of anti-NMDAR encephalitis
Neuro-immunobiology and treatment assessment in a mouse model of anti-NMDAR encephalitis Open
Anti-N-methyl- D -aspartate receptor (NMDAR) encephalitis is a disorder mediated by autoantibodies against the GluN1 subunit of NMDAR. It occurs with severe neuropsychiatric symptoms that often improve with immunotherapy. Clinical studies …
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: Cognitive profile, neuroimaging and fluid biomarkers in post-acute COVID-19 syndrome
Cognitive profile, neuroimaging and fluid biomarkers in post-acute COVID-19 syndrome Open
We aimed to characterize the cognitive profile of post-acute COVID-19 syndrome (PACS) patients with cognitive complaints, exploring the influence of biological and psychological factors. Participants with confirmed SARS-CoV-2 infection and…
View article: Acknowledgment to Reviewers
Acknowledgment to Reviewers Open
View article: Positive Allosteric Modulation of NMDARs Prevents the Altered Surface Dynamics Caused by Patients' Antibodies
Positive Allosteric Modulation of NMDARs Prevents the Altered Surface Dynamics Caused by Patients' Antibodies Open
We demonstrate that SGE-301 upregulates NMDAR surface diffusion and antagonizes the pathogenic effects of patients' IgG on NMDAR membrane organization. These findings suggest a potential therapeutic strategy for NMDARe.
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: STEM CELL SOLUTION TO GAD65 ANTIBODIES INDUCED HURRICANE OF TYPE 1 DIABETES AND NEUROLOGICAL DISORDERS
STEM CELL SOLUTION TO GAD65 ANTIBODIES INDUCED HURRICANE OF TYPE 1 DIABETES AND NEUROLOGICAL DISORDERS Open
The encounter of disorders associated with antibodies to neuronal enzymes caused a paradigm shift in understanding the CNS autoimmunity.The autoimmune disorders targeting the 65kDa isoform of glutamic acid decarboxylase (GAD65) not only co…
View article: HLA-DQB1*05 subtypes and not DRB1*10:01 mediates risk in anti-IgLON5 disease
HLA-DQB1*05 subtypes and not DRB1*10:01 mediates risk in anti-IgLON5 disease Open
Anti-IgLON5 disease is a rare and likely underdiagnosed subtype of autoimmune encephalitis. The disease displays a heterogeneous phenotype that includes sleep, movement and bulbar-associated dysfunction. The presence of IgLON5-antibodies i…
View article: Episodic recruitment of attractor dynamics in frontal cortex reveals distinct mechanisms for forgetting and lack of cognitive control in short-term memory
Episodic recruitment of attractor dynamics in frontal cortex reveals distinct mechanisms for forgetting and lack of cognitive control in short-term memory Open
Short-term memory (STM) is prone to failure, especially during prolonged memory maintenance or under limited cognitive control. Despite predictive mechanistic frameworks based on persistent neural activity and attractor states, a direct as…