About This Special Issue
Paraneoplastic limbic encephalitis is a rare neurological disorder characterized by subacute onset, in day or up to twelve weeks, of seizures, short-term memory loss, confusion and psychiatric symptoms suggesting involvement of the limbic system. In addition to the “well”-defined classical paraneoplastic syndromes with antibodies which target intracellular proteins (e.g., antiHu, anti-Yo, anti-Ri), a new group of antibodies associated with autoimmune encephalitis and their correlating symptoms, have been defined. They interact directly with cell-surface neuronal receptors or synaptic proteins. Unlike in classical paraneoplastic syndromes, in cell-surface or synaptic antibody-syndromes the presence of a tumor is variable, they respond to multimodal immunotherapy and seem to have a better overall prognosis. Autoimmune encephalitis Associated With Antibodies Against Neuronal Cell Surface Antigens includes various forms such as N-methyl D-aspartate receptor (NMDA-R) encephalitis, Anti-AMPA-R (α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor), Glycine receptor (Gly-R), DPPX (dipeptidyl-peptidase-like protein-6), anti-contactin-associated protein-2 (CASPR2), Antibodies against metabotropic glutamate receptor 5 (mGLUR5). Autoimmune encephalitis can manifest with several distinct syndromes, complicating its recognition. The classical presentation of encephalitis consists of a subacute (days to a few weeks) progressive decrease in the level of consciousness, often with fluctuations, and altered cognition. Memory, especially retention of new information, may be impaired early in the clinical course. Patients may progress to coma.
This special issue aims to help clinicians, both neurologists and other specialists, to promptly suspect and treat paraneoplastic limbic encephalitis. We will particularly focus on presentation (typical or peculiar), diagnosis, etiologies, but also treatment of them, expecially the forms associated with Antibodies Against Neuronal Cell Surface Antigens.
Perspective or retrospective studies, case reports, updates, reviews and guidelines proposals will be accepted.
- Symptoms and Signs, Diagnosis and Management of Paraneoplastic Limbic Encephalitis
- Treatment, Outcome and Prognosis of Paraneoplastic Limbic Encephalitis