OBSOLETE: Limbic encephalitis with nCMAgs antibodies

Limbic encephalitis with neuronal cell membrane antigen (nCMAgs) antibodies was a clinical classification used to describe a form of autoimmune limbic encephalitis in which antibodies target antigens located on the surface of neuronal cell membranes. This entry in Orphanet (code 163914) is now designated as OBSOLETE, meaning the condition has been reclassified or merged into more specific diagnostic entities. As understanding of autoimmune encephalitis advanced, distinct antibody-mediated conditions were identified, including anti-NMDA receptor encephalitis, anti-LGI1 encephalitis, anti-CASPR2 encephalitis, and anti-AMPA receptor encephalitis, among others. These conditions were previously grouped under the broader umbrella of limbic encephalitis with nCMAgs antibodies. Limbic encephalitis generally affects the limbic system of the brain, leading to symptoms such as subacute onset of short-term memory loss, seizures, confusion, psychiatric symptoms (including anxiety, depression, and behavioral changes), and sleep disturbances. Some patients may also develop movement disorders or autonomic dysfunction depending on the specific antibody involved. The condition can occur as a paraneoplastic syndrome associated with underlying tumors or as a primary autoimmune disorder without cancer. Treatment typically involves immunotherapy, including corticosteroids, intravenous immunoglobulin (IVIG), and plasma exchange as first-line therapies. Second-line treatments may include rituximab or cyclophosphamide. If an underlying tumor is identified, its removal is a critical component of management. Patients with antibodies against neuronal surface antigens generally have a better prognosis and response to immunotherapy compared to those with antibodies targeting intracellular antigens. Because this entry is obsolete, patients and clinicians should refer to the specific antibody-defined subtypes for current diagnostic and treatment guidance.

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