Autoimmune limbic encephalitis

An autoimmune condition characterized by INFLAMMATION of the LIMBIC SYSTEM, though other brain regions may also be affected. Patients usually present with severe short-term memory loss; however, confusion, psychiatric symptoms, and seizures may also occur. It may result from an underlying neoplasm (paraneoplastic limbic encephalitis) or be idiopathic.

Networked: 82 relevant articles (4 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Systemic Scleroderma (Systemic Sclerosis)
2.	Seizures (Absence Seizure)
3.	Limbic Encephalitis (Paraneoplastic Limbic Encephalitis)
4.	Glioma (Gliomas)
5.	Encephalitis (Encephalitis, Rasmussen)

Experts

1.	Melzer, Nico: 4 articles (05/2022 - 01/2020)
2.	Watanabe, Osamu: 4 articles (03/2017 - 01/2013)
3.	Spatola, Marianna: 3 articles (01/2022 - 06/2014)
4.	Dalmau, Josep: 3 articles (11/2018 - 06/2014)
5.	Dik, Andre: 2 articles (05/2022 - 12/2021)
6.	Golombeck, Kristin S: 2 articles (05/2022 - 12/2021)
7.	Meuth, Sven G: 2 articles (05/2022 - 12/2021)
8.	Mönig, Constanze: 2 articles (05/2022 - 12/2021)
9.	Räuber, Saskia: 2 articles (05/2022 - 12/2021)
10.	Strippel, Christine: 2 articles (05/2022 - 12/2021)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Autoimmune limbic encephalitis:

1.	Immunosuppressive Agents (Immunosuppressants)IBA 08/01/2018 - "We report the case of a patient affected by systemic sclerosis (SSc) who developed autoimmune limbic encephalitis, which improved under immunosuppressant agents and high-dose intravenous immunoglobulins. " 06/01/2010 - "We present a case of autoimmune limbic encephalitis associated with voltage-gated potassium channel autoantibodies that led to rapid cognitive deterioration and bizarre behaviour, which subsequently improved following immunosuppressant therapy. "
2.	AntibodiesIBA 09/01/2022 - "The definitive diagnosis of AE was based on the results of neuronal antibodies, and the diagnostic criteria of definite autoimmune limbic encephalitis proposed by Graus et al. used as the reference standard for antibody-negative AE. First, we automatically recognized and extracted symptoms for all HPI texts in EMRs by training a dataset of 552 cases. " 10/01/2021 - "Serum antiganglioside antibodies in patients with autoimmune limbic encephalitis." 09/01/2020 - "These symptoms were compatible with a clinical diagnosis of autoimmune limbic encephalitis, although no specific antibodies were detected and the initial MRI was normal. " 01/01/2020 - "The clinical and neuroradiological picture and the detection of anti-CASPR2 antibodies in serum oriented the diagnosis towards autoimmune limbic encephalitis. " 12/15/2019 - "We discuss the prognostic and pathogenic value of high titer anti-GAD65 antibodies and its variations in a case of autoimmune limbic encephalitis."
3.	AutoantibodiesIBA 11/01/2013 - "Cerebral FDG-PET and MRI findings in autoimmune limbic encephalitis: correlation with autoantibody types." 01/01/2023 - "It is assumed that autoimmune limbic encephalitis (ALE) demonstrates distinct neuropsychological manifestations with differential responses to immunotherapy according to which associated autoantibody (AAB), if any, is identified. " 01/01/2023 - "Review and meta-analysis of neuropsychological findings in autoimmune limbic encephalitis with autoantibodies against LGI1, CASPR2, and GAD65 and their response to immunotherapy." 05/01/2019 - "This report describes a case of feline autoimmune limbic encephalitis and hippocampal necrosis that were presumably associated with DCC autoantibodies." 11/01/2018 - "Human autoantibodies against LGI1 associate with a form of autoimmune limbic encephalitis characterized by severe but treatable memory impairment and frequent faciobrachial dystonic seizures. "
4.	Voltage-Gated Potassium Channels (Voltage-Gated Potassium Channel)IBA 09/01/2021 - "Key Words: Autoimmune limbic encephalitis, Voltage-gated potassium channels, Cognitive dysfunction." 10/01/2013 - "Autoimmune limbic encephalitis (LE) associated with voltage gated potassium channel antibodies (VGKC-Abs) in children is more common than previously thought and is not always paraneoplastic. " 06/01/2010 - "We present a case of autoimmune limbic encephalitis associated with voltage-gated potassium channel autoantibodies that led to rapid cognitive deterioration and bizarre behaviour, which subsequently improved following immunosuppressant therapy. " 10/01/2017 - "We describe a case of autoimmune limbic encephalitis associated with positive voltage gated potassium channel (VGKC) antibodies and positive leucine-rich glioma inactivated protein 1 antibodies (LGI1). " 03/01/2017 - "Several types of autoantibodies have been reported in autoimmune limbic encephalitis (LE), such as antibodies against the voltage-gated potassium channel (VGKC) complex including leucine-rich glioma inactivated 1 (LGI1). "
5.	tocilizumab (atlizumab)FDA Link 01/01/2014 - "This case highlights the possibility of the development of encephalopathy after treatment with tocilizumab, which may also induce autoimmune limbic encephalitis. " 01/01/2020 - "These data support the efficacy of tocilizumab in autoimmune limbic encephalitis associated with anti-CASPR2 antibodies, which has been sporadically reported in the literature." 01/01/2023 - "Autoimmune limbic encephalitis combined with leptomeningeal metastases of non-small cell lung cancer: treatment response to osimertinib, immunoglobulin, rituximab, and tocilizumab."
6.	Intravenous Immunoglobulins (IVIG)FDA Link 09/01/2018 - "A total of 625 patients who were 18 years of age or older were treated with intravenous immunoglobulin for autoimmune encephalitis, of whom 398 were determined as having 'highly likely' or 'definite' autoimmune/limbic encephalitis. " 08/01/2018 - "We report the case of a patient affected by systemic sclerosis (SSc) who developed autoimmune limbic encephalitis, which improved under immunosuppressant agents and high-dose intravenous immunoglobulins. "
7.	Proteins (Proteins, Gene)FDA Link 12/13/2017 - "Dysfunction of these genes and encoded proteins is associated with neurological disorders such as genetic epilepsy or autoimmune limbic encephalitis in animals and human. " 01/01/2023 - "Previous studies suggested that autoimmune limbic encephalitis with antibodies against contactin-associated protein-like 2 (CASPR2-encephalitis) is clinically heterogeneous and progresses slowly, preventing its early recognition. " 01/01/2017 - "Autoimmune limbic encephalitis with anti-contactin-associated protein-like 2 antibody secondary to pembrolizumab therapy." 01/01/2017 - "We report a case of autoimmune limbic encephalitis with Contactin-Associated Protein-like 2 (CASPR2) antibody secondary to Pembrolizumab therapy for metastatic melanoma." 01/01/2023 - "Autoantibodies to leucine-rich glioma inactivated protein 1 (LGI1) cause an autoimmune limbic encephalitis with frequent focal seizures and anterograde memory dysfunction. "
8.	AMPA Receptors (AMPA Receptor)IBA 06/15/2014 - "We report serial cerebral (18)FDG PET studies in a woman with AMPA receptor (AMPA-R) autoimmune limbic encephalitis. " 01/01/2021 - "This case highlighted that autoimmune limbic encephalitis is an important differential diagnosis for patients with typical symptoms even when the MRI and antibodies are normal, and more attention should be paid to the relapse of anti-AMPA receptor encephalitis." 01/01/2021 - "We described a case of a 47-year-old Chinese man having anti-AMPA receptor limbic encephalitis initially presented with cognitive decline, undetectable antibodies, and normal imaging findings in magnetic resonance image (MRI) and then developed into typical autoimmune limbic encephalitis a few months later with a course of multiple relapses. "
9.	Rituximab (Mabthera)FDA Link 01/01/2016 - "Rituximab treatment for autoimmune limbic encephalitis in an institutional cohort." 01/01/2016 - "This study provides Class IV evidence that rituximab improves mRS scores for patients with autoimmune limbic encephalitis who fail first-line therapy." 01/01/2016 - "To determine efficacy and safety of rituximab treatment as a second-line immunotherapy treatment for autoimmune limbic encephalitis (ALE) and to determine factors associated with functional improvement and favorable outcome following rituximab treatment. " 01/01/2023 - "Autoimmune limbic encephalitis combined with leptomeningeal metastases of non-small cell lung cancer: treatment response to osimertinib, immunoglobulin, rituximab, and tocilizumab."
10.	anti-leucine-rich glioma-inactivated 1 autoantibodyIBA 01/01/2013 - "Our study suggests that an autoimmune limbic encephalitis exists in cats and that VGKC-complex/LGI1 antibodies may play a role in this disorder, as they are thought to in humans." 01/01/2022 - "LGI1 antibodies are detected in subjects with autoimmune limbic encephalitis and peripheral nerve hyperexcitability syndromes (PNHSs) such as Isaacs and Morvan syndromes. "

Therapies and Procedures

1.	Therapeutics 01/01/2020 - "Investigating immune cells in autoimmune limbic encephalitis (LE) will contribute to our understanding of its pathophysiology and may help to develop appropriate therapies. " 09/01/2012 - "Although immunosuppression is the first-line therapy for autoimmune limbic encephalitis, this case suggests that, in selected cases, a lasting response can be achieved with surgery." 10/26/2021 - "Patients with temporal lobe epilepsy caused by autoimmune limbic encephalitis (AI-TLE) clinically resemble patients with temporal lobe epilepsy with non-autoimmune etiologies (NAI-TLE) but have a different prognosis and require specific adjusted therapies. " 01/01/2016 - "This study provides Class IV evidence that rituximab improves mRS scores for patients with autoimmune limbic encephalitis who fail first-line therapy." 01/01/2018 - "This article reports a case of fatal anti-Hu-associated autoimmune limbic encephalitis presenting within 8 weeks following anti-PD1 therapy in a patient with myxoid chondrosarcoma and pre-existing anti-Hu antibodies. "
2.	Immunotherapy 05/16/2022 - "Neuropsychological Performance in Autoimmune Limbic Encephalitis: Evidence from an Immunotherapy-Naïve Cohort." 01/01/2017 - "Suspected antibody negative autoimmune limbic encephalitis: outcome of immunotherapy." 01/01/2023 - "It is assumed that autoimmune limbic encephalitis (ALE) demonstrates distinct neuropsychological manifestations with differential responses to immunotherapy according to which associated autoantibody (AAB), if any, is identified. " 01/01/2023 - "Review and meta-analysis of neuropsychological findings in autoimmune limbic encephalitis with autoantibodies against LGI1, CASPR2, and GAD65 and their response to immunotherapy." 01/01/2016 - "To determine efficacy and safety of rituximab treatment as a second-line immunotherapy treatment for autoimmune limbic encephalitis (ALE) and to determine factors associated with functional improvement and favorable outcome following rituximab treatment. "
3.	Hematopoietic Stem Cell Transplantation 11/01/2023 - "Autoimmune limbic encephalitis (LE) is a rare, but devastating complication of allogeneic hematopoietic stem cell transplantation (HSCT). " 11/01/2023 - "Autoimmune Limbic Encephalitis in Patients with Hematologic Malignancies after Haploidentical Hematopoietic Stem Cell Transplantation with Post-Transplant Cyclophosphamide."
4.	Anesthesia 06/01/2014 - "Autoimmune limbic encephalitis and anesthesia."
5.	Aftercare (After-Treatment) 01/01/2014 - "This case highlights the possibility of the development of encephalopathy after treatment with tocilizumab, which may also induce autoimmune limbic encephalitis. "