Autoimmune neurology encompasses a wide spectrum of immune-mediated disorders with various neurologic manifestations. New neuronal antibodies are discovered each year; thus, the field continues to rapidly evolve including advancements in the understanding of underlying pathophysiology, associated clinical phenotypes, and treatment approaches. Advancements in the diagnostic workup and antibody testing of these disorders have grown to make once dogmatic thinking now irrelevant; for example, Voltage-Gated Potassium Channel (VGKC) antibodies are no longer tested as evidence has shown this antibody to be clinically insignificant in the absence of positive leucine-rich glioma inactivated 1 (LGI1) and/or contactin-associated protein-like 2 (Caspr2) antibodies. Even beyond the numerous autoantibody assays now available, our repertoire of ancillary testing continues to expand within the realms of neuroimaging as well as peripheral blood and cerebrospinal fluid biomarkers.
Furthermore, the various neoplastic, infectious, and genetic predisposing factors and triggers of neurological autoimmunity continue to become more refined with the hope of improved therapeutic targeting. For example, viruses have been associated with triggering central nervous system antibody-mediated autoimmune disease, as seen with herpes simplex virus encephalitis and post-infectious anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Additionally, paraneoplastic neurologic conditions may be the initial manifestation of occult malignancy or even be detected prior to tumor, requiring repeat malignancy screenings in anticipation of tumor diagnosis. As such, diagnostic workup of autoimmune neurologic conditions often necessitates investigation of underlying infections, immunodeficiency, and occult malignancy.
Lastly, with increased awareness and diagnosis of autoimmune neurologic syndromes, an increasing number of immunosuppressed patients are now at risk for infectious complications with little data to guide immunotherapy decisions. As the field advances, our treatment approaches evolve. Unfortunately, the lack of meaningful clinical outcome measures in autoimmune encephalitis and other autoimmune neurological disorders hinders the evaluation of treatment strategies. Identifying meaningful outcome measures and biomarkers of disease activity is required to understand the burden of disease and to provide adequate clinical measures to inform future treatment trials.
This Research Topic aims to improve the understanding of novel neuronal autoantibody syndromes, provide an update on our current understanding of the etiology of these disorders, and provide clinical data to guide clinicians in evaluating the appropriate treatment strategy with an understanding of associated infectious risks. Both Review and Original Research articles will be considered for submission. Case reports/series are appropriate for submission if they provide data regarding a newly identified autoimmune neurologic disorder, a novel presentation of an autoimmune neurologic disorder, or data regarding patient outcomes and/or complications of immunotherapy for autoimmune neurologic disorders.
Autoimmune neurology encompasses a wide spectrum of immune-mediated disorders with various neurologic manifestations. New neuronal antibodies are discovered each year; thus, the field continues to rapidly evolve including advancements in the understanding of underlying pathophysiology, associated clinical phenotypes, and treatment approaches. Advancements in the diagnostic workup and antibody testing of these disorders have grown to make once dogmatic thinking now irrelevant; for example, Voltage-Gated Potassium Channel (VGKC) antibodies are no longer tested as evidence has shown this antibody to be clinically insignificant in the absence of positive leucine-rich glioma inactivated 1 (LGI1) and/or contactin-associated protein-like 2 (Caspr2) antibodies. Even beyond the numerous autoantibody assays now available, our repertoire of ancillary testing continues to expand within the realms of neuroimaging as well as peripheral blood and cerebrospinal fluid biomarkers.
Furthermore, the various neoplastic, infectious, and genetic predisposing factors and triggers of neurological autoimmunity continue to become more refined with the hope of improved therapeutic targeting. For example, viruses have been associated with triggering central nervous system antibody-mediated autoimmune disease, as seen with herpes simplex virus encephalitis and post-infectious anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Additionally, paraneoplastic neurologic conditions may be the initial manifestation of occult malignancy or even be detected prior to tumor, requiring repeat malignancy screenings in anticipation of tumor diagnosis. As such, diagnostic workup of autoimmune neurologic conditions often necessitates investigation of underlying infections, immunodeficiency, and occult malignancy.
Lastly, with increased awareness and diagnosis of autoimmune neurologic syndromes, an increasing number of immunosuppressed patients are now at risk for infectious complications with little data to guide immunotherapy decisions. As the field advances, our treatment approaches evolve. Unfortunately, the lack of meaningful clinical outcome measures in autoimmune encephalitis and other autoimmune neurological disorders hinders the evaluation of treatment strategies. Identifying meaningful outcome measures and biomarkers of disease activity is required to understand the burden of disease and to provide adequate clinical measures to inform future treatment trials.
This Research Topic aims to improve the understanding of novel neuronal autoantibody syndromes, provide an update on our current understanding of the etiology of these disorders, and provide clinical data to guide clinicians in evaluating the appropriate treatment strategy with an understanding of associated infectious risks. Both Review and Original Research articles will be considered for submission. Case reports/series are appropriate for submission if they provide data regarding a newly identified autoimmune neurologic disorder, a novel presentation of an autoimmune neurologic disorder, or data regarding patient outcomes and/or complications of immunotherapy for autoimmune neurologic disorders.