Limbic Encephalitis (Paraneoplastic Limbic Encephalitis)

A paraneoplastic syndrome marked by degeneration of neurons in the medial temporal lobe. Clinical features include behavioral changes, HALLUCINATIONS, loss of short term memory, anosmia, AGEUSIA, and DEMENTIA. Circulating anti-neuronal antibodies (anti-Hu; also called ANNA 1) and small cell lung carcinomas are frequently associated with this condition. (From Pathologe 1997 Sep;18(5):406-10; J Int Neuropsychol Soc 1996 Sep;2(5):460-6; Brain 1997 Jun;120(Pt 6):923-8)

Also Known As:

Networked: 426 relevant articles (15 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Small Cell Lung Carcinoma (Small Cell Lung Cancer)
2.	Neoplasms (Cancer)
3.	Seizures (Seizure)
4.	Encephalitis (Encephalitis, Rasmussen)
5.	Stiff-Person Syndrome (Stiff Man Syndrome)

Experts

1.	Vincent, Angela: 34 articles (04/2015 - 06/2003)
2.	Takahashi, Yukitoshi: 25 articles (01/2015 - 09/2005)
3.	Dalmau, Josep: 17 articles (03/2015 - 08/2005)
4.	Lang, Bethan: 15 articles (04/2015 - 06/2003)
5.	Graus, Francesc: 15 articles (07/2014 - 05/2003)
6.	Vincent, A: 12 articles (02/2015 - 07/2001)
7.	Dalmau, J: 11 articles (06/2015 - 07/2000)
8.	Bien, Christian G: 8 articles (02/2015 - 03/2004)
9.	Honnorat, J: 8 articles (05/2014 - 07/2006)
10.	Graus, F: 7 articles (06/2015 - 01/2005)

Drugs and Biologics

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

1.	AntibodiesIBA 01/01/2005 - "On the contrary, immunosuppressant treatment is not usually effective in limbic encephalitis associated to anti-Hu antibodies." 06/01/2012 - "This report is of a patient with type 1 diabetes and a rare case of non-paraneoplastic limbic encephalitis mediated by anti-GAD65 antibodies that improved with the use of immunosuppressive drugs." 12/01/2015 - "Future studies should clarify the prevalence and pathogenic relevance of anti-synapsin antibodies in limbic encephalitis." 08/01/2013 - "This study provides evidence for a potential association between antibodies and limbic encephalitis. " 09/01/2012 - "A neuropathological study following amygdalohippocampectomy revealed limbic encephalitis and LGI1 antibodies were identified in the serum. "
2.	SteroidsIBA 01/01/2014 - "The outcome of anti-VGKCrelated limbic encephalitis is improved with early treatment employing steroids or immunotherapy. " 01/01/2008 - "Based on the clinical picture, the patient's history of lung cancer, the brain magnetic resonance imaging findings and the results of the brain biopsy, we concluded that our patient had a 'definite' diagnosis of paraneoplastic limbic encephalitis and he was subsequently treated with a combination of chemotherapy and oral steroids, resulting in stabilization of his neurological status. " 02/01/2002 - "According to previous studies, clinical symptoms, magnetic resonance imaging (MRI) findings, and the responsiveness to steroids are similar among steroid-reactive encephalopathy, non-herpetic limbic encephalitis, and Hashimoto's encephalopathy. " 01/01/2010 - "Anti-epileptic drugs for seizures, chemotherapy for small-cell lung cancer, and intravenous immunoglobulin and steroids for paraneoplastic limbic encephalitis led to a resolution of her seizures and improved her mental status. "
3.	Potassium Channels (Potassium Channel)IBA 12/01/2013 - "There is a high level of diagnostic certainty for patients who have classic limbic encephalitis and have a neural antibody detected in serum or CSF (such as potassium channel complex antibody). " 05/01/2005 - "We report a patient with potassium channel antibody-associated non-paraneoplastic limbic encephalitis (NPLE) in whom repeated fluorodeoxyglucose-positron emission tomography (FDG-PET) and magnetic resonance imaging (MRI) are correlated with epileptic activity and memory performance during the course of disease. " 05/01/2005 - "FDG-PET and MRI in potassium channel antibody-associated non-paraneoplastic limbic encephalitis: correlation with clinical course and neuropsychology." 03/01/2004 - "Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis." 01/01/2006 - "Rapid eye movement sleep behavior disorder and potassium channel antibody-associated limbic encephalitis."
4.	DNA (Deoxyribonucleic Acid)IBA 01/01/2006 - "Among patients who had positive results for HHV-6 DNA, the HHV-6 DNA load was higher in plasma from those who developed limbic encephalitis (n = 4) (P < .0001). " 11/01/2012 - "All patients diagnosed with HHV-6-PALE had HHV-6 DNA in cerebrospinal fluid (CSF) specimens in addition to symptoms and studies indicating limbic encephalitis. " 08/01/2010 - "Detection of DNA of six human herpesviruses in the cerebrospinal fluid of immunocompetent non-herpetic acute limbic encephalitis patients." 01/01/2006 - "Development of limbic encephalitis is associated with high HHV-6 DNA load." 01/01/2001 - "When brain imaging is suggestive of limbic encephalitis in an immunocompromised patient, PCR of CSF for VZV DNA should be performed, as early antiviral treatment may improve the outcome."
5.	Voltage-Gated Potassium Channels (Voltage-Gated Potassium Channel)IBA 12/01/2015 - "Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis." 01/01/2015 - "The patient was diagnosed with voltage-gated potassium channel antibody-related limbic encephalitis based on clinical presentation, imaging findings, and antibody testing. " 01/01/2015 - "A case study of voltage-gated potassium channel antibody-related limbic encephalitis with PET/MRI findings." 07/01/2014 - "Voltage-gated potassium channel antibody-associated limbic encephalitis." 01/01/2014 - "We report two cases of anti-voltage gated potassium channel (VGKC) antibody-related limbic encephalitis highlighting the variable presentation of this condition. "
6.	Glutamate Decarboxylase (Decarboxylase, Glutamate)IBA 06/01/2012 - "Limbic encephalitis and type 1 diabetes with glutamic acid decarboxylase 65 (GAD65) autoimmunity: improvement with high-dose intravenous immunoglobulin therapy." 01/01/2015 - "These findings led to the diagnosis of glutamic acid decarboxylase antibody related limbic encephalitis. " 01/01/2015 - "Loss of Autonoetic Awareness of Recent Autobiographical Episodes and Accelerated Long-Term Forgetting in a Patient with Previously Unrecognized Glutamic Acid Decarboxylase Antibody Related Limbic Encephalitis." 08/15/2014 - "Glutamic acid decarboxylase (GAD) has been recently identified as a target of humoral autoimmunity in a small subgroup of patients with non-paraneoplastic limbic encephalitis (NPLE). " 05/01/2014 - "Anti-glutamic Acid decarboxylase antibody associated limbic encephalitis in a child: expanding the spectrum of pediatric inflammatory brain diseases."
7.	Opsoclonus myoclonusIBA 01/01/2014 - "This is a case of paraneoplastic overlap of limbic encephalitis and opsoclonus myoclonus in a patient with non-small squamous cell lung carcinoma. " 03/01/2012 - "Opsoclonus-myoclonus and anti-Hu positive limbic encephalitis in a patient with neuroblastoma." 01/01/2005 - "A 48-year-old man with autoimmune Addison disease developed the following paraneoplastic neurologic syndromes (PNNS): limbic encephalitis, opsoclonus/myoclonus, and sensorimotor and autonomic neuropathies. " 04/01/1995 - "Five of the patients responded to the therapy, with complete and durable responses in three patients with opsoclonus-myoclonus, objective, though transient, improvement in one patient with paraneoplastic brainstem encephalitis associated with a Merkel cell tumor, and stabilization and partial improvement in one patient with paraneoplastic limbic encephalitis. " 07/01/2006 - "paraneoplastic limbic encephalitis, subacute sensory neuronopathy, paraneoplastic cerebellar degeneration, paraneoplastic opsoclonus-myoclonus, Lambert-Eaton myasthenic syndrome and paraneoplastic peripheral nerve hyperexcitability is given. "
8.	Prednisolone (Predate)FDA LinkGeneric 01/01/2012 - "The clinical diagnosis was made as acute limbic encephalitis associated with SLE, and subsequent administration of prednisolone improved her conditions. " 10/01/2013 - "Faciobrachial dystonic seizures have recently been reported as immunotherapy-responsive, brief, frequent events that often predate the cognitive impairment associated with this limbic encephalitis. " 01/01/2001 - "A case of limbic encephalitis in a patient who had undergone prolonged immunosuppressive treatment with i.v. cyclophosphamide and oral prednisolone for a microscopic polyangeitis is reported. "
9.	MethylprednisoloneFDA LinkGeneric 10/01/2007 - "The tentative clinical diagnosis was non-herpetic acute limbic encephalitis (NHALE), and administration of methylprednisolone improved her conditions. " 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. " 05/01/2007 - "This case report indicates that high-dose IV methylprednisolone therapy may be an affective treatment for non-herpetic acute limbic encephalitis, possibly associated with autoimmune mechanisms."
10.	Immunoglobulins (Immunoglobulin)IBA 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). " 01/01/2010 - "Anti-epileptic drugs for seizures, chemotherapy for small-cell lung cancer, and intravenous immunoglobulin and steroids for paraneoplastic limbic encephalitis led to a resolution of her seizures and improved her mental status. " 02/26/2015 - "We aimed to investigate the migration and expansions of peripheral and central B cells to the production of LGI1-specific IgG. We performed PCR amplification and next generation deep immune repertoire sequencing of immunoglobulin (Ig) heavy chain variable regions (VH) from CSF and subsorted peripheral blood B-cell populations from two patients with limbic encephalitis and faciobrachial dystonic seizures associated with LGI1 antibodies. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 10/01/1993 - "This observation may indicate that chemotherapy has modified that part of the immunological system responsible for induction of limbic encephalitis." 01/01/2013 - "Female, 59 FINAL DIAGNOSIS: Paraneoplastic limbic encephalitis Symptoms: Seizure • memory changes • decreased concentration Chemotherapy Clinical Procedure: Cerebral images Specialty: Hematology • Oncology. " 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."
2.	Immunotherapy 01/15/2016 - "Immunotherapy had beneficial effects in 57.1% of patients, and the patients with limbic encephalitis responded especially well to immunotherapy. " 06/01/2014 - "In cases of unexplained adult-onset MTLE, limbic encephalitis associated with anti-VGKC-Ab, which responds well to immunotherapy, should be considered in the differential diagnosis. " 06/01/2014 - "By contrast high-positive (>400 pM; >0.4 nM) levels were considered definitely (38%) or possibly (49%) clinically relevant, but not all patients had a 'classical' limbic encephalitis and some did not receive immunotherapies." 10/01/2010 - "An effective immunotherapy regimen for VGKC antibody-positive limbic encephalitis." 04/01/2010 - "Early diagnosis and treatment with immunotherapy, tumor resection or both are important for improving or stabilizing the condition of limbic encephalitis."
3.	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."
4.	Radiotherapy 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. " 11/01/2008 - "Seizures may also appear associated with paraneoplastic syndromes, such as limbic encephalitis and metabolic and infectious complications of chemotherapy and radiotherapy. "
5.	Transplants (Transplant) 07/01/2012 - "Human herpesvirus 6 (HHV6) is the major cause of posttransplant acute limbic encephalitis (PALE) in immunosuppressed patients following hematopoietic stem cell transplant. " 03/01/2011 - "Thus, HHV-6 is considered to be etiological agent for post-transplant acute limbic encephalitis. " 02/05/2008 - "Post-transplant acute limbic encephalitis: clinical features and relationship to HHV6." 07/10/2007 - "Post-transplant acute limbic encephalitis: clinical features and relationship to HHV6." 07/10/2007 - "Patients undergoing allogeneic hematopoietic stem cell transplantation are at risk for post-transplant acute limbic encephalitis (PALE), a distinct neurologic syndrome. "