limbic encephalitis

Summary

Summary: 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.

Top Publications

  1. Ances B, Vitaliani R, Taylor R, Liebeskind D, Voloschin A, Houghton D, et al. Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates. Brain. 2005;128:1764-77 pubmed
    We report seven patients, six from a single institution, who developed subacute limbic encephalitis initially considered of uncertain aetiology. Four patients presented with symptoms of hippocampal dysfunction (i.e...
  2. Wainwright M, Martin P, Morse R, Lacaze M, Provenzale J, Coleman R, et al. Human herpesvirus 6 limbic encephalitis after stem cell transplantation. Ann Neurol. 2001;50:612-9 pubmed
    ..Because of its subtle clinical presentation, this syndrome may be underrecognized, but can be diagnosed with appropriate magnetic resonance imaging techniques, EEG monitoring, and cerebrospinal fluid viral studies...
  3. Ohkawa T, Fukata Y, Yamasaki M, Miyazaki T, Yokoi N, Takashima H, et al. Autoantibodies to epilepsy-related LGI1 in limbic encephalitis neutralize LGI1-ADAM22 interaction and reduce synaptic AMPA receptors. J Neurosci. 2013;33:18161-74 pubmed publisher
    ..Here, we focused on a mode of action of LGI1 autoantibodies associated with limbic encephalitis (LE), which is one of acquired epileptic disorders characterized by subacute onset of amnesia and seizures...
  4. Tuzun E, Rossi J, Karner S, Centurion A, Dalmau J. Adenylate kinase 5 autoimmunity in treatment refractory limbic encephalitis. J Neuroimmunol. 2007;186:177-80 pubmed
    We report two men with limbic encephalitis (LE) refractory to corticosteroids, IVIg and plasma exchange. Both patients had serum/CSF antibodies that reacted with the cytoplasm of neurons...
  5. Yoo J, Hirsch L. Limbic encephalitis associated with anti-voltage-gated potassium channel complex antibodies mimicking Creutzfeldt-Jakob disease. JAMA Neurol. 2014;71:79-82 pubmed publisher
    b>Limbic encephalitis that is associated with anti-voltage-gated potassium channel complex (VGKCC) antibodies (VGKCC syndrome) is an autoimmune, usually nonparaneoplastic form of encephalitis that is responsive to immunotherapy...
  6. Frisch C, Malter M, Elger C, Helmstaedter C. Neuropsychological course of voltage-gated potassium channel and glutamic acid decarboxylase antibody related limbic encephalitis. Eur J Neurol. 2013;20:1297-304 pubmed publisher
    Autoantibodies (abs) to glutamic acid decarboxylase (GAD) and to voltage-gated potassium channels (VGKC) induce distinct courses of limbic encephalitis, related to MRI findings, seizure outcome and cognition.
  7. Kawano H, Hamaguchi E, Kawahito S, Tsutsumi Y, Tanaka K, Kitahata H, et al. Anaesthesia for a patient with paraneoplastic limbic encephalitis with ovarian teratoma: relationship to anti-N-methyl-D-aspartate receptor antibodies. Anaesthesia. 2011;66:515-8 pubmed publisher
    Paraneoplastic limbic encephalitis associated with ovarian teratoma has recently been related to the development of antibodies to specific heteromers of the N-methyl-d-aspartate receptor and exhibits various manifestations including ..
  8. Pearce J. Paraneoplastic limbic encephalitis. Eur Neurol. 2005;53:106-8 pubmed
  9. Munshi S, Thanvi B, Chin S, Hubbard I, Fletcher A, Vallance T. Paraneoplastic limbic encephalitis--case report and review of literature. Age Ageing. 2005;34:190-3 pubmed
    ..for his illness could be detected in his lifetime, but at post mortem he was found to have paraneoplastic limbic encephalitis and a bronchogenic tumour...

More Information

Publications62

  1. Dalmau J, Graus F, Villarejo A, Posner J, Blumenthal D, Thiessen B, et al. Clinical analysis of anti-Ma2-associated encephalitis. Brain. 2004;127:1831-44 pubmed
    ..Only 26% had classical limbic encephalitis. Excessive daytime sleepiness affected 32% of the patients, sometimes with narcolepsy-cataplexy and low CSF ..
  2. Sener R. MRI and diffusion MRI in nonparaneoplastic limbic encephalitis. Comput Med Imaging Graph. 2002;26:339-42 pubmed
    This paper reports an 65-year-old woman with nonparaneoplastic limbic encephalitis. On MRI and diffusion MRI, diffuse and symmetrical, bilateral high-signal lesions were evident in the medial temporal lobes...
  3. Graus F, Saiz A, Lai M, Bruna J, Lopez F, Sabater L, et al. Neuronal surface antigen antibodies in limbic encephalitis: clinical-immunologic associations. Neurology. 2008;71:930-6 pubmed publisher
    To report the frequency and type of antibodies against neuronal surface antigens (NSA-ab) in limbic encephalitis (LE).
  4. Pozo Rosich P, Clover L, Saiz A, Vincent A, Graus F. Voltage-gated potassium channel antibodies in limbic encephalitis. Ann Neurol. 2003;54:530-3 pubmed
    We found voltage-gated potassium channel (VGKC) antibodies in 4 of 15 patients with limbic encephalitis (LE)...
  5. Wong S, Saunders M, Larner A, Das K, Hart I. An effective immunotherapy regimen for VGKC antibody-positive limbic encephalitis. J Neurol Neurosurg Psychiatry. 2010;81:1167-9 pubmed publisher
    Voltage-gated potassium channel antibody-positive limbic encephalitis (VGKC+LE) frequently improves with immunotherapy, although the optimum regimen is unknown...
  6. Demaerel P, Van Dessel W, Van Paesschen W, Vandenberghe R, Van Laere K, Linn J. Autoimmune-mediated encephalitis. Neuroradiology. 2011;53:837-51 pubmed publisher
    ..This report is based on a review of the literature (except the literature in Japanese) and own findings in patients with autoimmune-mediated encephalitis...
  7. Kleopa K, Elman L, Lang B, Vincent A, Scherer S. Neuromyotonia and limbic encephalitis sera target mature Shaker-type K+ channels: subunit specificity correlates with clinical manifestations. Brain. 2006;129:1570-84 pubmed
    ..is called Morvan's syndrome (MoS); and CNS manifestations without peripheral involvement is called limbic encephalitis (LE)...
  8. Koide R, Shimizu T, Koike K, Dalmau J. EFA6A-like antibodies in paraneoplastic encephalitis associated with immature ovarian teratoma: a case report. J Neurooncol. 2007;81:71-4 pubmed
    ..Antibodies that colocalize with EFA6A are a valuable marker for early diagnosis of a potentially reversible paraneoplastic encephalitis associated with OT...
  9. Lee A, Ou Y, Lee W, Wong Y. Paraneoplastic limbic encephalitis masquerading as chronic behavioural disturbance in an adolescent girl. Acta Paediatr. 2003;92:506-9 pubmed
    ..To describe an unusual but treatable cause of behavioural disturbance in adolescence...
  10. Sakuma H, Sugai K, Sasaki M. Acute nonparaneoplastic limbic encephalitis in childhood: a case series in Japan. Pediatr Neurol. 2010;43:167-72 pubmed publisher
    b>Limbic encephalitis not associated with malignancy was investigated in Japanese children, with particular focus on clinical features distinct from adult cases...
  11. Zuliani L, Saiz A, Tavolato B, Giometto B, Vincent A, Graus F. Paraneoplastic limbic encephalitis associated with potassium channel antibodies: value of anti-glial nuclear antibodies in identifying the tumour. J Neurol Neurosurg Psychiatry. 2007;78:204-5 pubmed
  12. Vincent A, Buckley C, Schott J, Baker I, Dewar B, Detert N, et al. Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain. 2004;127:701-12 pubmed
    ..Amongst the differential diagnoses, herpes simplex encephalitis, Korsakoff's syndrome and limbic encephalitis should be considered...
  13. Bak T, Antoun N, Balan K, Hodges J. Memory lost, memory regained: neuropsychological findings and neuroimaging in two cases of paraneoplastic limbic encephalitis with radically different outcomes. J Neurol Neurosurg Psychiatry. 2001;71:40-7 pubmed
    To report two cases of paraneoplastic limbic encephalitis (PNLE) with similar clinical presentation, but dramatically different outcome and to highlight the role of neuropsychological and radiological evaluation in PNLE.
  14. Braik T, Evans A, Telfer M, McDunn S. Paraneoplastic neurological syndromes: unusual presentations of cancer. A practical review. Am J Med Sci. 2010;340:301-8 pubmed publisher
    ..Paraneoplastic neurological syndromes (PNS) are uncommon and imperfectly understood and, therefore, are frequently underdiagnosed...
  15. Bien C, Urbach H, Schramm J, Soeder B, Becker A, Voltz R, et al. Limbic encephalitis as a precipitating event in adult-onset temporal lobe epilepsy. Neurology. 2007;69:1236-44 pubmed
    ..There have been few studies of adult-onset disease. We recognized that some adult individuals have evidence of limbic encephalitis (LE), an autoimmune condition of adult life, which we proposed might lead directly to this syndrome.
  16. Modoni A, Masciullo M, Spinelli P, Marra C, Tartaglione T, Andreetta F, et al. Successful treatment of acute autoimmune limbic encephalitis with negative VGKC and NMDAR antibodies. Cogn Behav Neurol. 2009;22:63-6 pubmed publisher
    To describe a case of acute nonherpetic limbic encephalitis (LE) with negative testing for antibodies directed against onconeuronal and cell membrane antigens, including voltage-gated potassium channels and N-methyl-D-aspartate receptor, ..
  17. Lancaster E, Martinez Hernandez E, Titulaer M, Boulos M, Weaver S, Antoine J, et al. Antibodies to metabotropic glutamate receptor 5 in the Ophelia syndrome. Neurology. 2011;77:1698-701 pubmed publisher
    ..To report the metabotropic glutamate receptor 5 (mGluR5) as the autoantigen of antibodies from 2 patients with Hodgkin lymphoma (HL) and limbic encephalopathy (Ophelia syndrome)...
  18. Baumgartner A, Rauer S, Mader I, Meyer P. Cerebral FDG-PET and MRI findings in autoimmune limbic encephalitis: correlation with autoantibody types. J Neurol. 2013;260:2744-53 pubmed publisher
    In parallel to the detection of new neuronal autoantibodies, the diagnosis of non-infectious limbic encephalitis has risen...
  19. Haberlandt E, Bast T, Ebner A, Holthausen H, Kluger G, Kravljanac R, et al. Limbic encephalitis in children and adolescents. Arch Dis Child. 2011;96:186-91 pubmed publisher
    b>Limbic encephalitis is rare in people <18 years of age and rarely given a formal diagnosis.
  20. Bataller L, Galiano R, Garcia Escrig M, Martinez B, Sevilla T, Blasco R, et al. Reversible paraneoplastic limbic encephalitis associated with antibodies to the AMPA receptor. Neurology. 2010;74:265-7 pubmed publisher
  21. Ohta Y, Nagano I, Niiya D, Fujioka H, Kishimoto T, Shoji M, et al. Nonparaneoplastic limbic encephalitis with relapsing polychondritis. J Neurol Sci. 2004;220:85-8 pubmed
    ..This is thought to be the first case of RP presenting amnesic syndrome and mental disorder associated with nonparaneoplastic limbic encephalitis involving bilateral hippocampi and amygdaloidal bodies detected by MRI.
  22. Asaoka K, Shoji H, Nishizaka S, Ayabe M, Abe T, Ohori N, et al. Non-herpetic acute limbic encephalitis: cerebrospinal fluid cytokines and magnetic resonance imaging findings. Intern Med. 2004;43:42-8 pubmed
    Non-herpetic acute limbic encephalitis (non-herpetic ALE) is regarded as a new subgroup of limbic encephalitis...
  23. Sobas M, Galiano Leis M, De la Fuente Cid R, Pereiro Zabala I. [Paraneoplastic limbic encephalitis and epidermoid carcinoma of the pyriform]. An Med Interna. 2006;23:331-4 pubmed
    Paraneoplastic limbic encephalitis is a syndrome characterised by behaviour changes, short-term memory loss and seizures...
  24. Knudsen A, Bredholt G, Storstein A, Oltedal L, Davanger S, Krossnes B, et al. Antibodies to CRMP3-4 associated with limbic encephalitis and thymoma. Clin Exp Immunol. 2007;149:16-22 pubmed
    We present a case with subacute limbic encephalitis (LE) and thymoma...
  25. Bien C, Elger C. Limbic encephalitis: a cause of temporal lobe epilepsy with onset in adult life. Epilepsy Behav. 2007;10:529-38 pubmed
    b>Limbic encephalitis (LE) was described in the 1960s as a clinical-pathological syndrome in adults. Initially, the paraneoplastic form was the center of interest...
  26. Jacob S, Irani S, Rajabally Y, Grubneac A, Walters R, Yazaki M, et al. Hypothermia in VGKC antibody-associated limbic encephalitis. J Neurol Neurosurg Psychiatry. 2008;79:202-4 pubmed publisher
    Voltage-gated potassium channel antibody (VGKC-Ab)-associated limbic encephalitis (LE) is a recently described syndrome that broadens the spectrum of immunotherapy-responsive central nervous system disorders...
  27. Irani S, Michell A, Lang B, Pettingill P, Waters P, Johnson M, et al. Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Ann Neurol. 2011;69:892-900 pubmed publisher
    To describe a distinctive seizure semiology that closely associates with voltage-gated potassium channel (VGKC)-complex/Lgi1 antibodies and commonly precedes the onset of limbic encephalitis (LE).
  28. Mazzi G, Roia D, Cruciatti B, Matà S, Catapano R. Plasma exchange for anti GAD associated non paraneoplastic limbic encephalitis. Transfus Apher Sci. 2008;39:229-33 pubmed publisher
    b>Limbic encephalitis (LE) is a neurological syndrome usually presenting in a paraneoplastic form...
  29. Lee A, Glick D, Dinwiddie S. Electroconvulsive therapy in a pediatric patient with malignant catatonia and paraneoplastic limbic encephalitis. J ECT. 2006;22:267-70 pubmed
    Paraneoplastic limbic encephalitis is a rare disorder that can cause memory loss, confusion, personality change, cognitive dysfunction, and psychosis...
  30. Jarius S, Hoffmann L, Clover L, Vincent A, Voltz R. CSF findings in patients with voltage gated potassium channel antibody associated limbic encephalitis. J Neurol Sci. 2008;268:74-7 pubmed
    Recently, a new subtype of limbic encephalitis (LE) has been described, serologically characterized by the presence of antibodies against voltage gated potassium channels (VGKC, to be called VGKC-LE)...
  31. Sprengelmeyer R, Atkinson A, Sprengelmeyer A, Mair Walther J, Jacobi C, Wildemann B, et al. Disgust and fear recognition in paraneoplastic limbic encephalitis. Cortex. 2010;46:650-7 pubmed publisher
    Paraneoplastic limbic encephalitis (PNLE) affects limbic portions of the brain associated with recognition of social signals of emotions. Yet it is not known whether this perceptual ability is impaired in individuals with PNLE...
  32. Asztely F, Kumlien E. The diagnosis and treatment of limbic encephalitis. Acta Neurol Scand. 2012;126:365-75 pubmed publisher
    The term limbic encephalitis (LE) was first introduced in 1968...
  33. Harrower T, Foltynie T, Kartsounis L, de Silva R, Hodges J. A case of voltage-gated potassium channel antibody-related limbic encephalitis. Nat Clin Pract Neurol. 2006;2:339-43; quiz following 343 pubmed
    ..He had become apathetic and uninterested in his family. He had no relevant past medical or family history. General and physical neurological examinations were unremarkable, as was bedside cognitive testing...
  34. Collison K, Rees J. Asymmetric cerebellar ataxia and limbic encephalitis as a presenting feature of primary Sjögren's syndrome. J Neurol. 2007;254:1609-11 pubmed
  35. Barajas R, Collins D, Cha S, Geschwind M. Adult-onset drug-refractory seizure disorder associated with anti-voltage-gated potassium-channel antibody. Epilepsia. 2010;51:473-7 pubmed publisher
    ..Once the diagnosis has been established the initiation of immunotherapy should be undertaken without delay...
  36. Lai M, Hughes E, Peng X, Zhou L, Gleichman A, Shu H, et al. AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol. 2009;65:424-34 pubmed publisher
    To report the clinical and immunological features of a novel autoantigen related to limbic encephalitis (LE) and the effect of patients' antibodies on neuronal cultures.
  37. Monstad S, Nøstbakken J, Vedeler C. CRMP5 antibodies found in a patient with limbic encephalitis and myasthenia gravis. J Neurol Neurosurg Psychiatry. 2009;80:241-2 pubmed publisher
    ..antibodies are often associated with thymoma or small cell lung cancer and paraneoplastic syndromes such as limbic encephalitis (LE)...
  38. Weimer T, Boling W, Pryputniewicz D, Palade A. Temporal lobectomy for refractory status epilepticus in a case of limbic encephalitis. J Neurosurg. 2008;109:742-5 pubmed publisher
    The authors report a case of status epilepticus secondary to limbic encephalitis that was successfully treated with temporal lobectomy...
  39. Urbach H, Soeder B, Jeub M, Klockgether T, Meyer B, Bien C. Serial MRI of limbic encephalitis. Neuroradiology. 2006;48:380-6 pubmed
    The aim of the study was to analyze serial magnetic resonance imaging (MRI) scans in patients with various forms of limbic encephalitis (LE) in order to evaluate whether, and at what time point, MRI findings support the diagnosis of LE.
  40. Kartsounis L, de Silva R. Unusual amnesia in a patient with VGKC-Ab limbic encephalitis: a case study. Cortex. 2011;47:451-9 pubmed publisher
    ..We argue that the reported case broadens the phenomenology of VGKC-Ab limbic encephalitis and raises important theoretical questions about the aetiology of this patient's most unusual memory ..
  41. Nakaoku Y, Maki T, Kanazawa K, Matsumoto R, Fukuyama H, Takahashi R, et al. [A case of smoldering anti-leucine-rich glioma-inactivated 1 (LGI1) antibody-associated limbic encephalitis with faciobrachial dystonic seizure]. Rinsho Shinkeigaku. 2013;53:706-11 pubmed
    ..59-year-old right-handed woman with smoldering leucine-rich glioma-inactivated 1 (LGI1) antibody-associated limbic encephalitis (LE) following faciobrachial dystonic seizures...
  42. Takahashi Y, Mogami Y, Takayama R, Ikeda H, Imai K. [Antibodies to glutamate receptor in limbic encephalitis]. Brain Nerve. 2010;62:827-37 pubmed
    ..In non-paraneoplastic, non-herpetic acute limbic encephalitis (NHALE), serum antibodies to GluRepsilon2 (NR2B) were detected in approximately 60% of the patients from ..
  43. Maguire E, Nannery R, Spiers H. Navigation around London by a taxi driver with bilateral hippocampal lesions. Brain. 2006;129:2894-907 pubmed
  44. Sabin T, Jednacz J, Staats P. Case records of the Massachusetts General Hospital. Case 26-2008. A 26-year-old woman with headache and behavioral changes. N Engl J Med. 2008;359:842-53 pubmed publisher
  45. Bataller L, Kleopa K, Wu G, Rossi J, Rosenfeld M, Dalmau J. Autoimmune limbic encephalitis in 39 patients: immunophenotypes and outcomes. J Neurol Neurosurg Psychiatry. 2007;78:381-5 pubmed
    About 40% of patients with limbic encephalitis do not have detectable CNS antibodies. Some of these patients have immune-mediated limbic encephalitis, but their frequency is unknown.
  46. Kamaleshwaran K, Iyer R, Antony J, Radhakrishnan E, Shinto A. 18F-FDG PET/CT findings in voltage-gated potassium channel limbic encephalitis. Clin Nucl Med. 2013;38:392-4 pubmed publisher
    b>Limbic encephalitis (LE) can be associated with cancer, viral infection, or be idiopathic. One such rare but treatable form is associated with voltage-gated potassium channel (VGKC) antibodies...
  47. Stein Wexler R, Wootton Gorges S, Greco C, Brunberg J. Paraneoplastic limbic encephalitis in a teenage girl with an immature ovarian teratoma. Pediatr Radiol. 2005;35:694-7 pubmed
    Paraneoplastic limbic encephalitis (PLE) is an unusual disorder that is characterized by the association of clinical limbic system abnormalities with neoplasia, usually malignancy...
  48. Yoshikawa H, Yamazaki S. [A child with non-herpetic acute limbic encephalitis]. No To Hattatsu. 2003;35:429-31 pubmed
  49. Gast H, Schindler K, Z Graggen W, Hess C. Improvement of non-paraneoplastic voltage-gated potassium channel antibody-associated limbic encephalitis without immunosuppressive therapy. Epilepsy Behav. 2010;17:555-7 pubmed publisher
    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...
  50. Khadem G, Heble S, Kumar R, White C. Anti-N-methyl-D-aspartate receptor antibody limbic encephalitis. Intern Med J. 2009;39:54-6 pubmed publisher
    ..Most patients improve with immunosuppressive treatment. Raising awareness of this rare but increasingly reported condition is important, as it is responsive to treatment and potentially reversible...
  51. Shindo A, Ii Y, Sasaki R, Takahashi Y, Yoneda M, Kuzuhara S. [Non-herpetic acute limbic encephalitis-like manifestation in a case of Hashimoto's encephalopathy with positive autoantibodies against ionotropic glutamate receptor epsilon2]. Rinsho Shinkeigaku. 2007;47:629-34 pubmed
    ..The tentative clinical diagnosis was non-herpetic acute limbic encephalitis (NHALE), and administration of methylprednisolone improved her conditions...
  52. Kerling F, Blumcke I, Stefan H. Pitfalls in diagnosing limbic encephalitis - a case report. Acta Neurol Scand. 2008;118:339-42 pubmed publisher
    The syndrome of limbic encephalitis (LE) is characterized by subacute onset of temporal lobe epilepsy, loss of short-term memory, cognitive confusion and psychiatric symptoms.
  53. Boronat A, Sabater L, Saiz A, Dalmau J, Graus F. GABA(B) receptor antibodies in limbic encephalitis and anti-GAD-associated neurologic disorders. Neurology. 2011;76:795-800 pubmed publisher
    Aminobutyric acid-B receptor antibodies (GABA(B)R-ab) were recently described in 15 patients with limbic encephalitis (LE), associated with small-cell lung cancer (SCLC) or with concurrent glutamic acid decarboxylase (GAD) antibodies...