Limbic Encephalitis (Paraneoplastic Limbic Encephalitis)

A paraneoplastic syndrome marked by degeneration of neurons in the LIMBIC SYSTEM. Clinical features include HALLUCINATIONS, loss of EPISODIC MEMORY; ANOSMIA; AGEUSIA; TEMPORAL LOBE EPILEPSY; DEMENTIA; and affective disturbance (depression). Circulating anti-neuronal antibodies (e.g., anti-Hu; anti-Yo; anti-Ri; and anti-Ma2) and small cell lung carcinomas or testicular carcinoma are frequently associated with this syndrome.

Also Known As:

Networked: 779 relevant articles (29 outcomes, 18 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Small Cell Lung Carcinoma (Small Cell Lung Cancer)
2.	Neoplasms (Cancer)
3.	Seizures (Absence Seizure)
4.	Thymoma (Thymic Carcinoma)
5.	Glioma (Gliomas)

Experts

1.	Vincent, Angela: 40 articles (01/2018 - 06/2003)
2.	Takahashi, Yukitoshi: 31 articles (01/2021 - 09/2005)
3.	Dalmau, Josep: 21 articles (01/2021 - 08/2005)
4.	Lang, Bethan: 18 articles (06/2018 - 06/2003)
5.	Graus, Francesc: 17 articles (01/2021 - 05/2003)
6.	Honnorat, Jérôme: 15 articles (03/2022 - 11/2009)
7.	Irani, Sarosh R: 15 articles (01/2022 - 04/2010)
8.	Bien, Christian G: 13 articles (03/2022 - 03/2004)
9.	Tanaka, Keiko: 12 articles (05/2021 - 09/2002)
10.	Vincent, A: 12 articles (02/2015 - 07/2001)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Limbic Encephalitis:

1.	AntibodiesIBA 07/01/2023 - "The commercial assays used in this study did not provide unequivocal evidence for presence of neural antibodies in dogs including one dog with histopathologically proven limbic encephalitis. " 08/01/2013 - "This study provides evidence for a potential association between antibodies and limbic encephalitis. " 01/01/2023 - "'Limbic encephalitis' with GAD antibodies should be subsumed under GAD-TLE. " 01/01/2023 - "It has been questioned whether 'limbic encephalitis with GAD antibodies' is a meaningful diagnostic entity. " 09/29/2022 - "Paraneoplastic antibodies were also ordered, with anti-Ma2 antibodies eventually returning positive, consistent with anti-Ma2 paraneoplastic limbic encephalitis. "
2.	SteroidsIBA 09/01/2020 - "The patient made a full clinical recovery but relapsed clinically upon steroid tapering, while hypersignal in the left mesial temporal suggestive of limbic encephalitis was observed on repeated MRI. " 12/15/2019 - "Limbic encephalitis was diagnosed and high dose steroids treatment started. " 09/15/2017 - "After initially responded to steroid treatment, the patient developed limbic encephalitis. " 05/01/2003 - "Steroid-responsive limbic encephalitis." 01/01/2014 - "The outcome of anti-VGKCrelated limbic encephalitis is improved with early treatment employing steroids or immunotherapy. "
3.	Intravenous Immunoglobulins (IVIG)FDA Link 01/01/2019 - "Limbic encephalitis was suspected and the patient was treated with intravenous immunoglobulin (IVIG) for five days. " 07/01/2010 - "We diagnosed him with limbic encephalitis and peripheral neuropathy due to paraneoplastic neurological syndrome and treated him with two courses of intravenous immunoglobulin (IVIg) (400 mg/kg, 5 days). " 01/01/2022 - "Clinical workup including magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) examination revealed findings suspicious for limbic encephalitis (LE), successfully treated with intravenous immunoglobulins and intravenous corticosteroids. " 08/01/2020 - ": We conclude that rituximab may be tried in sero-negative limbic encephalitis if IVMP combined with IVIg failed, and delayed use of rituximab could also be effective." 01/01/2012 - "He was diagnosed as limbic encephalitis, and was administered high-dose intravenous methylprednisolone and immune adsorption plasma therapy followed by intravenous immunoglobulin therapy. "
4.	AutoantibodiesIBA 11/23/2023 - "After searching for autoantibodies, she was diagnosed with anti-VGKC complex antibody-associated limbic encephalitis. " 10/01/2022 - "In XLP1-associated limbic encephalitis, anti-AMPAR autoantibody production by the dysregulated immune system due to SAP deficiency might be a pathogenic mechanism of central nervous system manifestations. " 10/01/2021 - "Analysis of autoantibody spectrum and human herpesvirus 6 in adult patients with 'early' versus 'late' diagnosis of 'possible limbic encephalitis'." 01/01/2021 - "Limbic encephalitis (LE) comprises a spectrum of inflammatory changes in affected brain structures including the presence of autoantibodies and lymphoid cells. " 01/01/2020 - "In this context, certain autoantibodies (autoABs) were often associated with limbic encephalitis (LE). "
5.	Voltage-Gated Potassium Channels (Voltage-Gated Potassium Channel)IBA 05/01/2022 - "A primary diagnosis of voltage-gated potassium channel limbic encephalitis (VGKC-LE) was made. " 01/01/2021 - "Voltage-gated potassium channel antibody-associated limbic encephalitis was rare, and always associated with high antibody titres. " 12/09/2019 - "Review of a previous admission with hyponatraemia raised the possibility of voltage-gated potassium channel antibody-associated limbic encephalitis, although subsequent investigations deemed this unlikely as a cause of hyponatraemia. " 09/01/2019 - "We report a case of anti-voltage-gated potassium channel (VGKC) limbic encephalitis in a 47-year-old man presenting with a 2-year history of psychiatric features. " 09/01/2019 - "Anti-Voltage-gated Potassium Channel Limbic Encephalitis with Psychiatric Features: a Case Report."
6.	Glutamate Decarboxylase (Decarboxylase, Glutamate)IBA 01/01/2023 - "In cases with established glutamic acid decarboxylase antibody-associated limbic encephalitis, psychotic symptoms were uncommon and identifiable by clinical assessment. " 01/01/2023 - "Second, literature review of all published cases and case series of glutamic acid decarboxylase antibody-associated limbic encephalitis was assessed for frequency and description of psychotic symptoms. " 12/01/2020 - "Psychiatric symptoms in anti glutamic acid decarboxylase associated limbic encephalitis in adults: a systematic review." 08/01/2016 - "With the increased availability of laboratory tests, glutamic acid decarboxylase (GAD) antibody-positive limbic encephalitis has become an emerging diagnosis. " 08/01/2016 - "The diagnosis of glutamic acid decarboxylase 65 (GAD65) mediated limbic encephalitis relied on cerebral magnetic resonance imaging lesions and high serological and cerebrospinal fluid GAD65-antibodies titers. "
7.	Adrenal Cortex Hormones (Corticosteroids)IBA 04/01/2003 - "The rapid improvement in the patient's mental and cognitive functions after corticosteroid treatment and the abnormality shown on the single photon emission computed tomography suggested a diagnosis of paraneoplastic limbic encephalitis. " 01/01/2022 - "Clinical workup including magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) examination revealed findings suspicious for limbic encephalitis (LE), successfully treated with intravenous immunoglobulins and intravenous corticosteroids. " 06/01/2014 - "Here we describe 3 children presenting with limbic encephalitis with elevated thyroid antibodies that did not respond to corticosteroids alone and required more aggressive immunotherapy, mirroring the slower treatment response that is more frequently seen in other immune-mediated forms of limbic encephalitis. " 05/01/2007 - "We report two men with limbic encephalitis (LE) refractory to corticosteroids, IVIg and plasma exchange. " 06/01/2014 - "Elevated antithyroid antibodies have also been reported in a range of neurological syndromes with encephalopathy, such as limbic encephalitis, often collectively termed Hashimoto encephalopathy, a condition whereby corticosteroids responsiveness with a complete recovery is commonly observed. "
8.	Rituximab (Mabthera)FDA Link 01/01/2022 - "Rituximab treatment for a child with antibody-negative limbic encephalitis." 08/01/2020 - "It is still unclear if rituximab can benefit sero-negative limbic encephalitis.Case" 08/01/2020 - "Synergistic effect of rituximab with anti-epileptic drugs in treating sero-negative limbic encephalitis: a case report." 08/01/2020 - " presentation: Here, we report that a sero-negative limbic encephalitis patient with refractory seizures was treated successfully with rituximab combined with anti-epileptic drugs. " 01/01/2013 - "Improvement of paraneoplastic limbic encephalitis after systemic treatment with rituximab in a patient with B-cell chronic lymphocytic leukemia."
9.	Immunosuppressive Agents (Immunosuppressants)IBA 01/01/2023 - "The condition of two patients worsened despite immunosuppressants possibly related to their autoimmune diagnoses (limbic encephalitis n = 1, giant cell arteritis n = 1). " 01/01/2005 - "On the contrary, immunosuppressant treatment is not usually effective in limbic encephalitis associated to anti-Hu antibodies." 04/01/2010 - "We describe a 61-year-old patient with clinical evidence of limbic encephalitis who improved with anticonvulsant treatment only, that is, without the use of immunosuppressive agents. " 02/01/2021 - "Steroid-responsive encephalopathy is a general term for diseases that are characterized by diffuse brain injury and respond well to corticosteroids or immunosuppressive agents, including Hashimoto's encephalopathy (HE), limbic encephalitis (LE), systemic lupus erythematosus encephalopathy (SLEE), antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitis encephalopathy (AASV), viral encephalitis (VE), and primary central nervous system lymphoma (PCNSL). "
10.	Leucine (L-Leucine)FDA Link 01/01/2016 - "This study aims to retrospectively study clinical characteristics, diagnosis, treatment and follow-up visits in patients with anti-leucine-rich glioma inactivated-1 (LGI1) limbic encephalitis (LE) for prompting the early diagnosis and efficacious treatment of this disease. " 01/21/2023 - "Anti-leucine-rich glioma-inactivated 1 limbic encephalitis (LGI1-LE) is a rare autoimmune condition that affects the structural integrity and functioning of the brain's limbic system. " 12/01/2022 - "The best documented example is leucine-rich glioma-inactivated 1 (LGI1)-antibodies in domestic cats with limbic encephalitis (LE). " 09/15/2021 - "Anti-leucine-rich glioma-inactivated 1 (LGI1) antibody is associated with limbic encephalitis. " 09/01/2021 - "Anti-leucine-rich-glioma-inactivated 1 Limbic Encephalitis: An Underrecognised Disease."

Therapies and Procedures

1.	Therapeutics 09/01/2002 - "Immuno modulatory therapy should, nevertheless, be initiated as early as possible and seems especially helpful for peripheral syndromes and limbic encephalitis. " 03/01/2023 - "Two Cases of Limbic Encephalitis Successfully Treated With Electroconvulsive Therapy After Standard Immunomodulating Therapy Was Unsuccessful." 12/07/2021 - "One with definite limbic encephalitis was immune therapy responsive but had residual mood and memory problems. " 03/24/2010 - "Early recognition of paraneoplastic limbic encephalitis and prompt intervention with immune therapies at the onset of presentation will probably translate into more favorable neurological outcomes." 11/01/2009 - "The neural basis of effective memory therapy in a patient with limbic encephalitis."
2.	Immunotherapy 02/01/2022 - "Cerebrospinal fluid GAD65Ab was only checked and positive in case 1. Case 2 was diagnosed with limbic encephalitis, treated with immunotherapy, and showed a remarkable clinical improvement. " 01/01/2022 - "Nonparaneoplastic Anti-GAD Limbic Encephalitis: Seizure Outcome and Long-term Neuropsychological Follow-up After Immunotherapy." 10/22/2021 - "These findings suggest that the clinical manifestations of anti-AK5 limbic encephalitis result from a distinct T cell-mediated pathogenesis, with major cytotoxicity-induced apoptosis leading to a prompt and aggressive neuronal loss, likely explaining the poor prognosis and response to immunotherapy." 12/01/2017 - "Herein, we report the first case of neuro-Behçet's disease presenting with limbic encephalitis who received early stage immunotherapy." 03/23/2016 - "However, there has been a paucity in reports of limbic encephalitis associated with these immunotherapies. "
3.	Drug Therapy (Chemotherapy) 01/01/2013 - "Female, 59 FINAL DIAGNOSIS: Paraneoplastic limbic encephalitis Symptoms: Seizure • memory changes • decreased concentration Chemotherapy Clinical Procedure: Cerebral images Specialty: Hematology • Oncology. " 10/01/1993 - "This observation may indicate that chemotherapy has modified that part of the immunological system responsible for induction of limbic encephalitis." 08/01/2011 - "We hereby report a rare case of small-cell lung cancer associated with paraneoplastic limbic encephalitis during chemotherapy." 08/01/2011 - "[A case of small-cell lung cancer associated with paraneoplastic limbic encephalitis during chemotherapy]." 11/01/2004 - "Paraneoplastic limbic encephalitis is a rare complication of ovarian tumors that is potentially reversible with prompt surgical management of the primary tumor, plasmapheresis, and chemotherapy."
4.	Plasma Exchange 12/01/2008 - "Plasma exchange for anti GAD associated non paraneoplastic limbic encephalitis." 01/01/2019 - "A growing body of research suggests that, in the clinical application of steroid-responsive encephalopathy, such as for Hashimoto's encephalopathy, limbic encephalitis, systemic lupus erythematosus encephalopathy, ANCA-associated vasculitis encephalopathy, and acute disseminated encephalomyelitis, plasma exchange is a safe, and effective option when steroids or other immunosuppressive therapies are ineffective in the short term or when contraindications are present. " 05/01/2007 - "We report two men with limbic encephalitis (LE) refractory to corticosteroids, IVIg and plasma exchange. " 04/01/2020 - "Immune checkpoint inhibitor induced anti-glutamic acid decarboxylase 65 (Anti-GAD 65) limbic encephalitis responsive to intravenous immunoglobulin and plasma exchange." 02/01/2016 - "Therapeutic plasma exchange as a steroid-sparing therapy in a patient with limbic encephalitis due to antibodies to voltage-gated potassium channels."
5.	Thymectomy 01/01/2011 - "Unusual association of seronegative, nonparaneoplastic limbic encephalitis and relapsing polychondritis in a patient with history of thymectomy for myasthenia: a case study." 06/01/1995 - "Two patients with clinical and radiological evidence of limbic encephalitis associated with an invasive lymphoepithelial thymoma who improved after thymectomy and radiotherapy are reported. " 06/15/2012 - "We report an unusual patient with paraneoplastic limbic encephalitis diagnosed a few months after total thymectomy for asymptomatic thymoma, followed 18 years later by the onset of symptomatic MG without evidence of tumor recurrence." 11/13/2018 - "We report a young adult with thymoma-associated myasthenia gravis (MG) who, following thymectomy, developed paraneoplastic limbic encephalitis (LE) and systemic lupus erythematosus (SLE). " 11/13/2018 - "Thymoma-associated Myasthenia Gravis in a Young Adult with Development of Paraneoplastic Limbic Encephalitis and Systemic Lupus Erythematosus Post-thymectomy: A Case Report."