Skip to main content

Advertisement

SpringerLink
Log in

Autoimmune-mediated encephalitis

  • Continuing Education
  • Published:
Neuroradiology Aims and scope Submit manuscript

Abstract

Autoimmune-mediated encephalitis may occur as a paraneoplastic or as a non-paraneoplastic condition. The role of neuroimaging in autoimmune-mediated encephalitis has changed in the last decade partly due to improvements in sequence optimisation and higher field strength and partly due to the discovery of an increasing number of antibodies to neuronal cell and cell membrane antigens. Imaging is important since it can support the clinical diagnosis particularly in the absence of antibodies. Structural imaging findings can be subtle and are usually best seen on FLAIR images. A progressive as well as a relapsing–remitting course can be observed. Autoimmune-mediated encephalitis is classically linked to involvement of the hippocampus and amygdala, but extensive changes in the temporal cortex, basal ganglia, hypothalamus, brain stem, frontal and parietal cortex are not unusual. This report is based on a review of the literature (except the literature in Japanese) and own findings in patients with autoimmune-mediated encephalitis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11
Fig. 12
Fig. 13
Fig. 14
Fig. 15
Fig. 16

References

  1. Corsellis JA, Goldberg GJ, Norton AR (1968) ‘Limbic encephalitis’ and its association with carcinoma. Brain 91:481–496

    Article  PubMed  CAS  Google Scholar 

  2. Graus F, Saiz A, Dalmau J (2010) Antibodies and neuronal autoimmune disorders of the CNS. J Neurol 257:509–517

    Article  PubMed  CAS  Google Scholar 

  3. Dalmau J, Bataller L (2006) Clinical and immunological diversity of limbic encephalitis: a model for paraneoplastic neurologic disorders. Hematol Oncol Clin North Am 20:1319–1335

    Article  PubMed  Google Scholar 

  4. Mathew RM, Vandenberghe R, Garcia-Merino A, Yamamoto T, Landolfi JC, Rosenfeld MR, Rossi JE, Thiessen B, Dropcho EJ, Dalmau J (2007) Orchiectomy for suspected tumor in patients with anti-Ma2-associated encephalitis. Neurology 20:900–905

    Article  Google Scholar 

  5. Bien CG, Urbach H, Schramm J, Soeder BM, Becker AJ, Voltz R, Vincent A, Elger CE (2007) Limbic encephalitis as a precipitating event in adult-onset temporal lobe epilepsy. Neurology 69:1236–1244

    Article  PubMed  CAS  Google Scholar 

  6. Mata S, Muscas GC, Naldi I, Rosati E, Paladini S, Cruciatti B, Bisulli F, Paganini M, Mazzi G, Sorbi S, Tinuper P (2008) Non-paraneoplastic limbic encephalitis associated with anti-glutamic acid decarboxylase antibodies. J Neuroimmunol 199:155–159

    Article  PubMed  CAS  Google Scholar 

  7. Malter MP, Helmstaedter C, Urbach H, Vincent A, Bien CG (2010) Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis. Ann Neurol 67:470–478

    Article  PubMed  Google Scholar 

  8. Vernino S, Lennon VA (2004) Autoantibody profiles and neurological correlations of thymoma. Clin Cancer Res 10:7270–7275

    Article  PubMed  CAS  Google Scholar 

  9. McHugh JC, Murray B, Renganathan R, Connolly S, Lynch T (2007) GAD antibody positive paraneoplastic stiff person synsdrome in a patient with renal cell carcinoma. Mov Disord 22:1343–1346

    Article  PubMed  Google Scholar 

  10. Thieben MJ, Lennon VA, Boeve BF, Aksamit AJ, Keegan M, Vernino S (2004) Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody. Neurology 62:1177–1182

    PubMed  CAS  Google Scholar 

  11. Vitaliani R, Mason W, Ances B, Zwerdling T, Jiang Z, Dalmau J (2005) Paraneoplastic encephalitis, psychiatric symptoms and ovarian teratoma. Ann Neurol 58:594–604

    Article  PubMed  Google Scholar 

  12. Ances BM, Vitaliani R, Taylor RA, Liebeskind DS, Voloschin A, Houghton DJ, Galetta SL, Dichter M, Alavi A, Rosenfeld MR, Dalmau J (2005) Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates. Brain 128:1764–1777

    Article  PubMed  Google Scholar 

  13. Sillevis Smitt P, Kinoshita A, De Leeuw B, Moll W, Coesmans M, Jaarsma D, Henzen-Logmans S, Vecht C, De Zeeuw C, Sekiyama N, Nakanishi S, Shigemoto R (2000) Paraneoplastic cerebellar ataxia due to autoantibodies against a glutamate receptor. N Engl J Med 342:21–27

    Article  PubMed  CAS  Google Scholar 

  14. Pittock SJ, Parisi JE, McKeon A, Roemer SF, Lucchinetti CF, Tan KM, Keegan BM, Hunter SF, Duncan PR, Baehring JM, Matsumoto JY, Lennon VA (2010) Paraneoplastic jaw dystonia and laryngospasm with antineuronal nuclear autoantibody type 2 (anti-Ri). Arch Neurol 67:1109–1115

    Article  PubMed  Google Scholar 

  15. Shams’ili S, Grefkens J, de Leeuw B, van den Bent M, Hooijkaas H, van der Holt B, Vecht C, Sillevis Smitt P (2003) Paraneoplastic cerebellar degeneration associated with antineuronal antibodies: analysis of 50 patients. Brain 126:1409–1418

    Article  PubMed  Google Scholar 

  16. Watson R, Jiang Y, Bermudez I (2004) Absence of antibodies to glutamata receptor type 3 (GluR3) in Rasmussen encephalitis. Neurology 63:43–50

    PubMed  CAS  Google Scholar 

  17. Anderson NE, Barber PA (2008) Limbic encephalitis—a review. J Clin Neurosci 15:961–971

    Article  PubMed  CAS  Google Scholar 

  18. Foster AR, Caplan JP (2009) Paraneoplastic limbic encephalitis. Psychosomatics 50:108–113

    Article  PubMed  Google Scholar 

  19. Kayser MS, Kohler CG, Dalmau J (2010) Psychiatric manifestations of paraneoplastic disorders. Am J Psychiatry 167:1039–1050

    Article  PubMed  Google Scholar 

  20. Vedeler CA, Antoine JC, Giometto B, Graus F, Grisold W, Hart IK, Honnorat P, Sillevis Smitt PAE, Verschuuren JJGM, Voltz R, Paraneoplastic Neurological Syndrome Euronetwork (2008) Management of paraneoplastic neurological syndromes: report of an EFNS Task Force Europa. Eur J Neurol 13:682–690

    Article  Google Scholar 

  21. Graus F, Delattre JY, Antoine JC, Antoine JC, Dalmau J, Giometto B, Grisold W, Honnorat J, Smitt PS, Vedeler Ch, Verschuuren JJ, Vincent A, Voltz R (2004) Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 75:1135–1140

    Article  PubMed  CAS  Google Scholar 

  22. Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J (2000) Paraneoplastic limbic encephalitis: neurological symprtoms, immunological findings and tumour association in 50 patients. Brain 123:1481–1494

    Article  PubMed  Google Scholar 

  23. Lawn ND, Westmoreland BF, Kiely MJ, Lennon VA, Vernino S (2003) Clinical, magnetic resonance imaging, and electroencephalographic findings in paraneoplastic limbic encephalitis. Mayo Clin Proc 78:1363–1368

    Article  PubMed  Google Scholar 

  24. Urbach H, Soeder BM, Jeub M, Klockgether T, Meyer B, Bien CG (2006) Serial MRI of limbic encephalitis. Neuroradiology 48:380–386

    Article  PubMed  Google Scholar 

  25. Thuerl C, Müller K, Laubenberger J, Volk B, Langer M (2003) MR imaging of autopsy-proved paraneoplastic limbic encephalitis in non-Hodgkin lymphoma. Am J Neuroradiol 24:507–511

    PubMed  Google Scholar 

  26. Sener RN (2002) MRI and diffusion MRI in nonparaneoplastic limbic encephalitis. Comput Med Imaging Graph 26:339–342

    Article  PubMed  CAS  Google Scholar 

  27. Launay M, Bozzolo E, Venissac N, Delmont E, Fredenrich A, Thomas P (2008) Paraneoplastic limbic encephalitis with positive anti-Ri antibodies and mediastinal seminoma. Rev Neurol 164:612–619

    Article  PubMed  CAS  Google Scholar 

  28. McKeon A, Ahlskog E, Britton JA, Lennon VA, Pittock SJ (2009) Reversible extralimbic paraneoplastic encephalopathies with large abnormalities on magnetic resonance images. Arch Neurol 66:268–271

    Article  PubMed  Google Scholar 

  29. Fauser S, Talazko J, Wagner K, Ziyeh S, Jarius S, Vincent A, Schulze-Bonhage A (2005) FDG-PET and MRI in potassium channel antibody-associated non-paraneoplastic limbic encephalitis with clinical course and neuropsychology. Acta Neurol Scand 111:338–343

    Article  PubMed  CAS  Google Scholar 

  30. Basu S, Alavi A (2008) Role of FDG-PET in the clinical management of paraneoplastic neurological syndrome: detection of the underlying malignancy and the brain PET-MRI correlates. Mol Imaging Biol 10:131–137

    Article  PubMed  Google Scholar 

  31. Scheid R, Lincke T, Voltz R, von Cramon DY, Sabri O (2004) Serial 18F-fluoro-2-deoxy-D-glucose positron emission tomography and magnetic resonance imaging of paraneoplastic limbic encephalitis. Arch Neurol 61:1785–1789

    Article  PubMed  Google Scholar 

  32. Provenzale JM, Barboriak DP, Coleman RE (1998) Limbic encephalitis: comparison of FDG-PET and MRI findings. Am J Roentgenol 18:1659–1660

    Google Scholar 

  33. Rubello D, Vitaliani R, Rigoni MT, Rampin L, Giometto B, Casara D, Zonzin GC, Zavagno G, Capirci C, Shapiro B, Muzzio PC (2005) A rare case of paraneoplastic cerebellar degeneration discovered by whole body F-18 FDG PET. Clin Nucl Med 30:704–706

    Article  PubMed  CAS  Google Scholar 

  34. Younes-Mhenni S, Janier MF, Cinotti L, Antoine JC, Tronc F, Cottin V, Ternamian PJ, Trouillas P, Honnorat J (2004) FDG-PET improves tumour detection in patients with paraneoplastic neurological syndromes. Brain 127:2331–2338

    Article  PubMed  CAS  Google Scholar 

  35. Bien CG, Elger CE (2007) Limbic encephalitis: a cause of temporal lobe epilepsy with onset in adult life. Epilepsy Behav 10:529–538

    Article  PubMed  Google Scholar 

  36. Titulaer MJ, Soffietti R, Dalmau J, Gilhus NE, Giometto B, Graus F, Grisold W, Honnorat J, Sillevis Smitt PA, Tanasescu R, Vedeler CA, Voltz R, Verschuuren JJ (2011) Screening for tumours in paraneoplastic syndromes: report of an EFNS task force. Eur J Neurol 18(1):19-e3. doi:10.1111/j.1468-1331.2010.03220.x

    Article  PubMed  Google Scholar 

  37. Cianci V, Labate A, Lanza P, Vincent A, Gambardella A, Branca D, Arcudi L, Aguglia U (2010) Non-paraneoplastic limbic encephalitis characterized by mesio-temporal seizures and extratemporal lesions: a case report. Seizure 19:446–449

    Article  PubMed  Google Scholar 

  38. Rosenfeld MR, Eichen JG, Wade DF, Posner JB, Dalmau J (2001) Molecular and clinical diversity in paraneoplastic immunity to Ma proteins. Ann Neurol 50:339–348

    Article  PubMed  CAS  Google Scholar 

  39. Vincent A, Buckley C, Schott JM, Baker I, Dewar B-K, Detert N, Clover L, Parkinson A, Bien CG, Omer S, Lang B, Rossor MN, Palace J (2004) Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain 127:701–712

    Article  PubMed  Google Scholar 

  40. Dalmau J, Tüzün E, Wu HY (2007) Paraneoplastic anti-N-methyl-d-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 61:25–36

    Article  PubMed  CAS  Google Scholar 

  41. Watanabe Y, Shimizu Y, Ooi S, Tanaka K, Inuzuka T, Nakashima K (2003) Steroid-responsive limbic encephalitis. Intern Med 42:428–432

    Article  PubMed  Google Scholar 

  42. Asaoka K, Shoji H, Nishizaka S, Ayabe M, Abe T, Ohori N, Ichiyama T, Eizura Y (2004) Non-herpetic acute limbic encephalitis: cerebrospinal fluid cytokines and magnetic resonance imaging findings. Intern Med 43:42–48

    Article  PubMed  Google Scholar 

Download references

Conflict of interest

We declare that we have no conflict of interest.

Author information

Authors and Affiliations

  1. Department of Radiology, University Hospitals K.U.Leuven, Herestraat 49, 3000, Leuven, Belgium

    Philippe Demaerel & Wim Van Dessel

  2. Department of Neurology, University Hospitals K.U.Leuven, Herestraat 49, 3000, Leuven, Belgium

    Wim Van Paesschen & Rik Vandenberghe

  3. Department of Nuclear Medicine, University Hospitals K.U.Leuven, Herestraat 49, 3000, Leuven, Belgium

    Koen Van Laere

  4. Department of Neuroradiology, University Hospital München, Munich, Germany

    Jennifer Linn

Authors
  1. Philippe Demaerel
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Wim Van Dessel
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Wim Van Paesschen
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Rik Vandenberghe
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Koen Van Laere
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Jennifer Linn
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Philippe Demaerel.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Demaerel, P., Van Dessel, W., Van Paesschen, W. et al. Autoimmune-mediated encephalitis. Neuroradiology 53, 837–851 (2011). https://doi.org/10.1007/s00234-010-0832-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00234-010-0832-0

Keywords

Search

Navigation