Current approaches to the treatment of paraneoplastic encephalitis

Therapeutic Advances in Neurological Disorders

Volumn 4, Issue 4, 2011, Pages 237-248

  Grisold, Wolfgang ^a   Giometto, Bruno ^b   Vitaliani, Roberta ^b   Oberndorfer, Stefan ^c  

^a MEDICAL UNIVERSITY OF VIENNA   (Austria)

^b Ludwig Boltzmann Institute for Experimental and Clinical Traumatology ^*  (Italy)

^c LUDWIG BOLTZMANN INSTITUTE FOR EXPERIMENTAL AND CLINICAL TRAUMATOLOGY   (Austria)

Author keywords

cancer; ion channel antibodies; limbic encephalitis; NMDA; onconeuronal antibodies; paraneoplastic disease; paraneoplastic encephalitis; surface antibodies

Indexed keywords

4 AMINOBUTYRIC ACID; AMPA RECEPTOR; AMPHIPHYSIN ANTIBODY; AUTOANTIBODY; BOTULINUM TOXIN; COLLAPSIN RESPONSE MEDIATOR PROTEIN; CV2 ANTIBODY; CYCLOPHOSPHAMIDE; GLUTAMATE DECARBOXYLASE; HU ANTIBODY; IMMUNOGLOBULIN; ION CHANNEL; MA2 ANTIBODY; MEMBRANE ANTIGEN; N METHYL DEXTRO ASPARTIC ACID RECEPTOR; RI ANTIBODY; STEROID; UNCLASSIFIED DRUG; VOLTAGE GATED POTASSIUM CHANNEL;

AMNESIA; BREAST CARCINOMA; BREAST TUMOR; CANCER IMMUNOTHERAPY; CANCER THERAPY; CEREBELLUM DEGENERATION; COGNITIVE DEFECT; CONFUSION; DELUSION; DISEASE ASSOCIATION; ELECTROENCEPHALOGRAM; ENCEPHALITIS; ENCEPHALOMYELITIS; EXTRAPYRAMIDAL SYMPTOM; GASTROINTESTINAL TUMOR; HERPES SIMPLEX ENCEPHALITIS; HUMAN; HYPOVENTILATION; IMMUNOMODULATION; IMMUNOSUPPRESSIVE TREATMENT; LUNG SMALL CELL CANCER; MYOCLONUS; NEUROBLASTOMA; NEUROPATHOLOGY; NUCLEAR MAGNETIC RESONANCE IMAGING; OCCUPATIONAL THERAPY; OPHELIA SYNDROME; OVARY TUMOR; PARANEOPLASTIC NEUROPATHY; PHYSIOTHERAPY; PLASMAPHERESIS; POSITRON EMISSION TOMOGRAPHY; PRIORITY JOURNAL; RELAPSE; REVIEW; RIGIDITY; SEIZURE; SEMINOMA; SPEECH THERAPY; TEMPORAL LOBE; TESTIS TUMOR; THYMOMA; TREATMENT OUTCOME;

EID: 79959330219     PISSN: 17562856     EISSN: 17562864     Source Type: Journal    
DOI: 10.1177/1756285611405395     Document Type: Review

References (89)

1. Akman C.I. Patterson M.C. Rubinstein A. Herzog R. (2009) Encephalitis associated with anti-GAD antibody and common variable immune deficiency. Dev Med Child Neurol 51: 563–567.
2. Ances, B.M., Vitaliani, R., Taylor, R.A., Liebeskind, D.S., Voloschin, A., Houghton, D.J. et al. (2005) Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates. Brain 128: 1764–1777.
3. Antoine J. Honnorat J. Koeing F. Aguera M. Berlin M.F. Michel D. (1993) Posterior uveitis and paraneoplastic encephalomyelitis with auto-antibodies reacting against cytoplasmic protein of brain and retina. J Neurol Sci 163: 159–162.
4. Barnett M. Prosser J. Sutton I. Halmagyi G.M. Davies L. Harper C. et al (2001) Paraneoplastic brain stem encephalitis in a woman with anti-Ma2 antibody. J Neurol Neurosurg Psychiatry 70: 222–225.
5. Bataller L. Galiano R. García-Escrig M. Martínez B. Sevilla T. Blasco R. et al (2010) Reversible paraneoplastic limbic encephalitis associated with antibodies to the AMPA receptor. Neurology 74: 265–267.
6. Bayreuther C. Bourg V. Dellamonica J. Borg M. Bernardin G. Thomas P. (2009) Complex partial status epilepticus revealing anti-NMDA receptor encephalitis. Epileptic Disord 11: 261–265.
7. Brierley J.B. Corsellys J.A.N. Hierons R. Nevin S. (1960) Subacute encephalitis of later adult life mainly affecting the limbic areas. Brain 83: 357–368.
8. Carr I. (1982) The Ophelia syndrome: memory loss in Hodgkin's disease. Lancet 1: 844–845.
9. Corsellis J.A.N. Goldberg G.J. Norton A.R. (1968) ‘Limbic encephalitis’ and it's association with carcinoma. Brain 91: 481–495.
10. Dale R.C. Irani S.R. Brilot F. Pillai S. Webster R. Gill D. et al (2009) NMDA receptor antibodies in pediatric dyskinetic encephalitis lethargica. Ann Neurol 66: 704–709.
11. Dalmau J. Gleichman A.J. Hughes E. Rossi J.E. Peng X. Lai M. et al (2008) Anti-NMDA- receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 7: 1091–1098.
12. Dalmau J. Graus J. Rosenblum M.K. Posner J. (1992) Anti-Hu associated paraneoplastic encephalomyelitis / sensory neuronopathy. A clinical study of 71 patients. Medicine 71: 59–72.
13. Dalmau J. Gultekin S.H. Voltz R. (1999) A novel neuron-testis specific protein, is recognized by the serum of patients with paraneoplastic neurological disorders. Brain 122: 27–39.
14. Dalmau J. Posner J.B. (1997) Paraneoplastic syndromes affecting the nervous system. Semin Oncol 24: 318–328.
15. Dalmau J. Tuzun E. Wu H.Y. Masjuan J. Rossi J.E. Voloschin A. et al (2007) Paraneoplastic anti-Nmethyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 61: 25–36.
16. Deramecourt V. Bombois S. Debette S. Delbeuck X. Ramirez C. Reyns N. et al (2009) Bilateral temporal glioma presenting as a paraneoplastic limbic encephalitis with pure cognitive impairment. Neurologist 15: 208–211.
17. Dirr L.Y. Elster A.D. Donofrio P.D. Smith M. (1990) Evolution of brain MRI abnormalities in limbic encephalitis. Neurology 40: 1304–1306.
18. Dorresteijn L.D. Kappaelle A.C. Reiner W.O. Gijtenbek J.M. (2002) Anti-amphiphysin associated limbic encephalitis: a paraneoplastic presentation of small cell carcinoma. J Neurol 249: 1307–1308.
19. Dropcho E.J. (1996) Antiamphiphysin antibodies with small cell lung carcinoma and paraneoplastic encephalomielitis. Ann Neurol 39: 659–667.
20. Fadul C.E. Stommel E.W. Dragnev K.H. Dalmau J.O. (2005) Focal paraneoplastic limbic encephalitis presenting as orgasmic epilepsy. J Neuro-Oncol 72: 195–198.
21. Florance N.R. Davis R.L. Lam C. Szperka C. Zhou L. Ahmad S. et al (2009) Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol 66: 11–18.
22. Gable M.S. Gavali S. Radner A. Tilley D.H. Lee B. Dyner L. et al (2009) Anti-NMDA receptor encephalitis: report of ten cases and comparison with viral encephalitis. Eur J Clin Microbiol Infect Dis 28: 1421–1429.
23. Giometto B. Grisold W. Vitaliani R. Graus F. Honnorat J. Bertolini G. et al (2010) Paraneoplastic neurologic syndrome in the PNS Euronetwork database. Arch Neurol 67: 330–335.
24. Graus F. Delattre J.Y. Antoine J.C. Dalmau J. Giometto B. Grisold W. et al (2004) Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 75: 1135–1140.
25. Graus F. Saiz A. Dalmau J. (2010) Antibodies and neuronal autoimmune disorders of the CNS. J Neurol 257: 509–517.
26. Graus F. Vincent A. Pozo-Rosich P. Sabater L. Saiz A. Lang B. et al (2005) Anti-glial nuclear antibody marker of lung cancer-related paraneoplastic neurologica syndromes. J Neuroimmunol 165: 166–171.
27. Greenlee J.E. (2010) Treatment of paraneoplastic neurologic disorders. Curr Treat Options Neurol 12: 212–230.
28. Hama K. Ishiguchi H. Tuji T. Miwa H. Kondo T. (2008) Neurosyphilis with mesiotemporal magnetic resonance imaging abnormalities. Intern Med 47: 1813–1817.
29. Harloff A. Hummer S. Kleinschidt M. Rauer S. (2005) Anti-Ri antibodies and limbic encephalitis in a patient with carcinoid tumor of the lung. J Neurol 252: 1404–1405.
30. Hart I.K. Waters C. Vincent A. Newland C. Beeson D. Pongs O. (1997) Autoantibodies detected to expressed K+ channels are implicated in neuromyotonia. Ann Neurol 41: 238–246.
31. Hoffmann L.A. Jarius S. Pellkofer H.L. Schueller M. Krumbholz M. Koenig F. (2008) Anti-Ma and anti-Ta associated paraneoplastic neurological syndromes: 22 newly diagnosed patients and review of previous cases. J Neurol Neurosurg Psychiatry 79: 767–773.
32. Irani S.R. Alexander S. Waters P. Kleopa K.A. Pettingill P. Zuliani L. et al (2010 b) Antibodies to Kv 1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan's syndrome and acquired neuromyotonia. Brain 133: 2734–2748.
33. Irani S.R. Bera K. Waters P. Zuliani L. Maxwell S. Zandi M. et al (2010 a) N-methyl-D-aspartate antibody encephalitis: temporal progression of clinical and paraclinical observations in a predominantly non-paraneoplastic disorder of both sexes. Brain 133: 1655–1667.
34. Ito Y. Abe T. Tomioka R. Komori T. Araki N. (2010) Anti-NMDA receptor encephalitis during pregnancy (in Japanese). Rinsho Shinkeigaku 50: 103–107.
35. Kayser C.G. Kohler C.G. Dalmau J. (2010) Psychiatric manifestations of paraneoplastic disorders. Am J Psychiatry 167: 1039–1050.
36. Kellinghaus C. Kraus J. Blaes F. Nabavi D.G. Schabitz W.R. (2007) CRMP-5 autoantibodies in testicular cancer associated with limbic encephalitis and choreiform dyskinesia. Eur Neurol 57: 241–243.
37. Kirkpatrick M.P. Clarke C.D. Sonmezturk H.H. Abou-Khalil B. (2011) Rhythmic delta activity represents a form of nonconvulsive status epilepticus in anti- NMDA receptor antibody encephalitis. Epilepsy Behav 20 (2): 392–394. [Epub 28 December 2010].
38. Kleinig T.J. Thompson P.D. Matar W. Duggins A. Kimber T.E. Morris J.G. et al (2008) The distinctive movement disorder of ovarian teratoma-associated encephalitis. Mov Disord 23: 1256–1261.
39. Kleopa K.A. Elman L.B. Lang B. Vincent A. Scherer S.S. (2006) Neuromyotonia and limbic encephalitis sera target mature shaker-type K+ channels: subunit specificity correlates with clinical manifestations. Brain 129: 1570–1584.
40. Knudsen A. Bredolt A. Storstein A. Oteldal L. Dvanger S. Krossness B. et al (2007) Antibodies to CRMP3-4 associated with limbic encephalitis and thymoma. Clin Exp Immunol 147: 16–22.
41. Kobayakawa Y. Tateishi T. Kawamura N. Doi H. Ohyagi Y. Kira J. (2010) A case of immune-mediated encephalopathy showing refractory epilepsy and extensive brain MRI lesions associated with anti-glutamic acid decarboxylase antibody. Rinsho Shinkeigaku 50: 92–97.
42. Kohler, J., Hufschmidt, A., Hermle, L., Volk, B., Lücking, C.H. (1988). J Neuroimmunol 20: 177–178.
43. Lai M. Hughes E.G. Peng X. Zhou L. Gleichman A.J. Shu H. et al (2009) AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol 65: 424–434.
44. Lancaster E. Lai M. Peng X. Hughes E. Constantinescu R. Raizer J. et al (2010) Antibodies to the GABA(B) receptor in limbic encephalitis with seizures: case series and characterisation of the antigen. Lancet Neurol 9: 67–76.
45. Launary M. Bozzolo E. Vennisacc N. Delemont E. Fredenrich A. Thomas P. (2008) Paraneoplastic limbic encephalitis with positive anti-Ri antibodies and mediastinal seminoma. Rev Neurol (Paris) 164: 612–619.
46. Liguori R. Vincent A. Clover L. Avoni P. Plazzi G. Cortelli P. et al (2001) Morvan's syndrome: peripheral and central nervous system and cardiac involvement with antibodies to voltage-gated potassium channels. Brain 124: 2417–2426.
47. Luchinetti C.F. Kimmel D.W. Vanda A.L. (1998) Paraneoplastic and oncologic profiles of patients seropositive for Type 1 antineuronal nuclear autoantibodies. Neurology 50: 652–657.
48. Malter M.P. Helmstaedter C. Urbach H. Vincent A. Bien C.G. (2010) Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis. Ann Neurol 67: 470–478.
49. Marchiori G.C. Vaglia A. Vianello M. Bardin P.G. Giometto B. (2001) Encephalitis associated with glutamic acid decarboxylase autoantibodies. Neurology 56: 814–815.
50. Matà S. Muscas G.C. Cincotta M. Bartolozzi M.L. Ambrosini S. Sorbi S. (2010) GAD antibodies associated neurological disorders: incidence and phenotype distribution among neurological inflammatory diseases. J Neuroimmunol 227: 175–177.
51. Mathew R.M. Vandenberghe R. Garcia-Merino A. Yamamoto T. Landolfi J.C. Rosenfeld M.R. et al (2007) Orchiectomy for suspected microscopic tumor in patients with anti-Ma2-associated encephalitis. Neurology 68: 900–905.
52. Meinck H.M. Thompson P.D. (2002) Stiff man syndrome and related conditions. Mov Disord 17: 853–886.
53. Montiel P. Sellal F. Clerc C. Ricard P. Bataillard M. (2008) Limbic encephalitis with severe sleep disorder associated with voltage gated potassium channels (VGKC) antibodies (in French). Rev Neurol (Paris) 164: 181–184.
54. Pittock S.H. Parisi J.E. McKeon A. Roemer S.F. Lucchinetti C.F. Tan K.M. et al (2010) Paraneoplastic jaw dystonia and laryngospasm with antineuronal nuclear autoantibody type 2 (anti-Ri). Arch Neurol 67: 1109–1115.
55. Pittock S.J. Luchinetti C.F. Parisi J.E. Benearroch E.E. Mokri B. Stephan C.L. et al (2005) Amphiphisin autoimmunity: paraneoplastic accompaniments. Ann Neurol 58: 96–107.
56. Plotkin S.A. (2000) Rabies. Clin Infect Dis 30: 4–12.
57. Pozo-Rosich P. Clover L. Saiz A. Vincent A. Graus F. (2003) Voltage-gated potassium channel antibodies in limbic encephalitis. Ann Neurol 54: 530–533.
58. Reid J.M. Foley P. Willison H.J. (2009) Voltage-gated potassium channel-associated limbic encephalitis in the West of Scotland: case reports and literature review. Scott Med J 54: 27–31.
59. Rosenfeld M.R. Eichen J.G. Wade D.F. Posner J.B. Dalmau J. (2001) Molecular and clinical diversity in paraneoplastic immunity to Ma proteins. Ann Neurol 50: 339–348.
60. Sabater L. Gomez-Choco M. Saiz A. Graus F. (2005) BR serine / threonine kinase 2: a new autoantigen in paraneoplastic limbic encephalitis. J Neuroimmunol 170: 186–190.
61. Sabater L. Titulaer M. Saiz A. Verschuuren J. Gure A.O. Graus F. (2008) Sox 1 antibodies are a markers of paraneoplastic Lambert–Eaton myastenic syndrome. Neurology 70: 928–940.
62. Saidha, S., Murphy, S., Ronayne, A., McCarthy, P., Hennessy, MJ., Counihan, T. (2010) Treatment of anti-glutamic acid decarboxylase antibody-associated limbic encephalitis with mycophenolate mofetil. J Neurol 257 (6): 1035–1038.
63. Saiz A. Bruna J. Stourac P. Vigliani M.C. Giometto B. Grisold W. et al (2009) Anti-Hu- associated brainstem encephalitis. J Neurol Neurosurg Psychiatry 80: 404–407.
64. Schimmel M. Bien C.G. Vincent A. Schenk W. Penzien J. (2009) Successful treatment of anti-N-methyl D-aspartate receptor encephalitis presenting with catatonia. Arch Dis Child 94: 314–316.
65. Seeley W.W. Marty F.M. Holmes T.M. Upchurch K. Soiffer R.J. Antin J.H. et al (2007) Post-transplant acute limbic encephalitis. Clinical features and relationship to HHV6. Neurology 69: 156–165.
66. Shindo A. Kagawa K. Li Y. Sasaki R. Kokubo Y. Kuzuhara S. (2009) Anti-N- methyl-D-aspartate receptor-related grave but reversible encephalitis with ovarian teratoma in 2 Japanese women presenting with excellent recovery without tumor resection. Eur Neurol 61: 50–51.
67. Shinohara T. Kojima H. Nakamura N. Ogata A. Betsuyaku T. Suzuki A. et al (2005) Pathology of pure hippocampal sclerosis in a patient with dementia and Hodgkin's disease: the Ophelia syndrome. Neuropathology 25: 353–360.
68. Simo M. Dalmau J. (2009) Gliomatosis cerebri simulando una encefalitis limbica. Neurologia 24: 500–501.
69. Störring G.E. Hauss K. Ule G. (1962) Zur topischen Diagnostik des amnestischen Symptomenkomplexes. Psychiat Neurol (Basel) 143: 161–177.
70. Sutton I. Winer J. Rowlands D. Dalmau J. (2000) Limbic encephalitis and antibodies to Ma2: A paraneoplastic presentation of breast cancer. Neurol Neurosurg Psychiatr 69: 266–268.
71. Thieben M.J. Lennon V.A. Boeve B.F. Aksamit A.J. Keegan M. et al (2004) Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody. Neurology 62: 1177–1182.
72. Titulaer M.J. Soffietti R. Dalmau J. Gilhus N.E. Giometto B. Graus F. et al (2010) Screening for tumours in paraneoplastic syndromes: report of an EFNS Task Force. Eur J Neurol, in press.
73. Tüzün E. Rossi J.E. Karner S.F. Centurion A.F. Dalmau J. (2007) Adenylate kinase 5 autoimmunity in treatment refractory limbic encephalitis. J Neuroimmunol 186: 177–180.
74. Ulrich J. Spiess H. Huber R. (1967) Neurologische Syndrome als Fernwirkung maligner Tumore (Ammonshornsklerose bei Bronchuskarzinom). Schw Arch Neurol Neurochir Psychiat 99: 83–100.
75. Vedeler C.A. Antoine J.C. Giometto B. Graus F. Grisold W. Hart I.K. et al (2006) Management of paraneoplastic neurological syndromes: report of an EFNS Task Force. Eur J Neurol 13: 682–690.
76. Vedeler C.A. Storstein A. (2009) Autoimmune limbic encephalitis. Acta Neurol Scand Suppl 189: 63–67.
77. Vernino S. Tuite P. Adler C.H. Meschia J.F. Boeve B.F. Boasberg P. et al (2002) Paraneoplastic chorea associated with CRMP-5 neuronal antibody and lung carcinoma. Ann Neurol 51: 625–630.
78. Vieria A. Matias S. Saraiva P. Goulao A. (2005) Differential diagnosis of mesiotemporal lesions; case report of neurosyphillis. Neuroradiology 47: 774–777.
79. Vincent A. (2010) Guest lecture. J Neurol Neurosurg Psychiatry 81: e10–e11.
80. Vincent A. Buckley C. Schott J.M. Baker I. Dewar B.K. Detert N. et al (2004) Potassium channel antibody associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain 127: 701–712.
81. Vincent A. Irani S.R. Lang B. (2010) The growing recognition of immunotherapy responsive seizure disorders with autoantibodies to specific neuronal proteins. Curr Opin Neurol 23: 144–150.
82. Vitaliani R. Mason B. Ances B. Zwerdling T. Jang Z. Dalmau J. (2005) Paraneoplastic encephalitis, psychiatric symptoms and hypoventilation in ovarian teratoma. Ann Neurol 58: 594–604.
83. Vitaliani R. Zoccarato M. Vianello M. Giometto B. (2008) Clinical, immunological and therapeutic aspects of autoimmune encephalitis. Recent Patents on CNS Drug Discovery 3: 16–22.
84. Voltz R. Gultekin S.H. Rosenfeld M.R. Gerstner E. Eichen J. Posner J.B. et al (1999) A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer. N Engl J Med 340: 1788–1795.
85. White D. Beringer T. (2010) Paraneoplastic limbic encephalitis in an elderly patient with small cell lung carcinoma. Ulster Med J 79: 22–24.
86. Wong-Kisiel L.C. Ji T. Renaud D.I. Kotagal S. Patterson M.C. Dalmau J. et al (2010) Response to immunotherapy in a 20-month old boy with anti NMDA receptor encephalitis. Neurology 74: 1550–1551.
87. Zandi M.S. Irani S.R. Follows G. Moody A.M. Molyneux P. Vincent A. (2009) Limbic encephalitis associated with antibodies to the NMDA receptor in Hodgkin lymphoma. Neurology 73: 2039–2040.
88. Zandi M.S. Irani S.R. Lang B. Waters P. Jones P.B. McKenna P. et al (2011) Disease-relevant autoantibodies in first episode schizophrenia. J Neurol 258 (4): 686–688. [Epub 26 October 2010].
89. Zuliani L. Tavolato B. Giometto B. Vincent A. Graus F. (2007) Paraneoplastic limbic encephalitis associated with potassium channel antibodies: value of anti-glial nuclear antibodies in indentifying the tumor. J Neurol Neurosurg Psychiatry 78: 381–385.