25
Views
3
CrossRef citations to date
0
Altmetric
Review

Rasmussen’s encephalitis: update on pathogenesis and treatment

, , , &
Pages 835-843 | Published online: 10 Jan 2014

References

  • Aarli JA. Immunological aspects of epilepsy. Brain Dev. 15,41–50 (1993).
  • Barker CF, Billingham RE. Immunologically privileged sites. Adv. Immunol 25,1–54 (1977).
  • Wekerle H, Linington C, Lassmann H, Meyermann R. Cellular immune reactivity within the CNS. Trends Neurosci. 9, 271–277 (1986).
  • Seabrook TJ, Johnston M, Hay JB. Cerebral spinal fluid lymphocytes are part of the normal recirculating lymphocyte pool. J. Neuroimmunol 91,100–107 (1998).
  • Brabb T, von Dassow P, Ordonez N, Schnabel B, Duke B, Goverman J. In situ tolerance within the central nervous system as a mechanism for preventing autoimmunity. J. Exp. Med. 192,871–880 (2000).
  • Hart Y, Andermann F. Rasmussen syndrome. In: Intractable Focal Epilepsy Oxbury JM, Polkey CE, Duchowny M (Eds). WB Saunders, London, UK, 233–248 (2000).
  • Hart YM, Andermann F, Fish DR et al Chronic encephalitis and epilepsy in adults and adolescents: a variant of Rasmussen's syndrome? Neurology 48, 418–424 (1997).
  • Leach JP, Chadwick DW, Miles JB, Hart IK. Improvement in adult onset Rasmussen's encephalitis with long-term immunomodulatory therapy. Neurology52, 738–742 (1999).
  • Villani F, SpreafIco R, Farina L eta! Positive response to immunomodulatory therapy in an adult patient with Rasmussen's encephalitis. Neurology 56, 248–250 (2001).
  • Vadlamudi L, Galion CJ, Jeavons SJ et al. Rasmussen's syndrome in a 54 year old female: more support for an adult variant. J. Clin. Neurosci. 7,154-156 (2000).
  • Granata T, Spreafico R, Gobbi G eta! Rasmussen's encephalitis: early characteristics allow diagnosis. Neurology 60,422-425 (2003). Useful report on the clinical features allowing early diagnosis of Rasmussen's encephalitis (RE).
  • Bien CG, Widman G, Urbach H eta! The natural history of Rasmussen's encephalitis. Brain 125,1751-1759 (2002). First paper covering the clinical natural history of RE with particular reference to the time course of brain destruction and for the evaluation of prognosis in disease.
  • Oguni H, Andermann F, Rasmussen TB. The natural history of the syndrome of chronic encephalitis and epilepsy: a study of the MNI series of forty-eight cases. In: Chronic Encephalitis and Epilepsy: Rasmussen's Syndrome. Andermann F (Ed.). Butterworth-Heinemann, Boston, MA, USA, 7–35 (1991).
  • Tampieri D, Melanson D, Ethier R. Imaging of chronic encephalitis. In Chronic Encephalitis and Epilepsy: Rasmussen Syndrome. Andermann F (Ed.). Butterworth-Heinemann, Boston, MA, USA, 47–60 (1991).
  • Chiapparini L, Granata T, Farina L eta! Diagnostic imaging in 13 cases of Rasmussen's encephalitis: can early MRI suggest the diagnosis? Neurology 45, 171–183 (2003).
  • Bien CG, Urbach H, Deckert Metal Diagnosis and staging of Rasmusseris encephalitis by serial MRI and histopathology. Ainrology58, 250–257 (2002).
  • Rasmussen T, Olszewski J, Lloyd-Smith D. Focal seizures due to chronic localized encephalitis. Neurology8, 435–445 (1958).
  • Farrell MA, Droogan O, Secor DL, Poukens V, Quinn B, Vinters HV. Chronic encephalitis associated with epilepsy: immunohistochemical and ultrastructural studies. Acta Neuropathol 89,313–321 (1995).
  • Robitaille Y. Neuropathologic aspects of chronic encephalitis. In: Chronic Encephalitis and Epilepsy: Rasmussen Syndrome. Andermann F (Ed.). Butterworth-Heinemann, Boston, MA, USA, 89–110 (1991).
  • Andermann F, Hart Y. Rasmussen's syndrome. In: Textbook of Neumlogy Neurobase. International League Against Epilepsy (Ed.). Arbor Press, MI, USA (2003).
  • Friedman H, Chien L, Parham D. Virus in brain of child with hemiplegia, hemiconvulsions and epilepsy. Lancet 2, 666 (1977).
  • Walter GF, Renella RR. Epstein-Barr virus in brain and Rasmussen's encephalitis. Lancet 1,279–280 (1989).
  • Power C, Poland SD, Blume WT, Girvin JP, Rice GP Cytomegalovirus and Rasmussen's encephalitis. Lancet 336, 1282–1284 (1990).
  • McLachlan RS, Girvin JP, Blume WT, Reichman H. Rasmussen's chronic encephalitis in adults. Azrh. Neural. 50, 269–274 (1993).
  • Jay V, Becker LE, Otsubo H eta! Chronic encephalitis and epilepsy (Rasmussen's encephalitis): detection of cytomegalovirus and herpes simplex virus 1 by the polymerase chain reaction and in situ hybridization. Neurology45,108–117 (1995).
  • Rasmussen T Further observations on the syndrome of chronic encephalitis and epilepsy. Appl. Neumphysiol 41,1–12 (1978).
  • Mizuno Y, Chou SM, Estes ML, Erenberg G, Cruse RE Rothner AD. Chronic localized encephalitis (Rasmussen's) with focal cerebral seizures revisited. J. Neuropathol Exp. Neural. 44, 351 (1985).
  • Asher DM, Gajdusek DC. Virologic studies in chronic encephalitis. In: Chronic Encephalitis and Epilepsy' Rasmussen's Syndrome. Andermann F (Ed.). Butterworth-Heinemann, Boston, MA, USA, 147–158 (1991).
  • Paas Y. The macro- and microarchitectures of the ligand-binding domain of glutamate receptors. Trends Neumsci. 21,117–125 (1998).
  • Rogers SW, Andrews PI, Gahring LC eta! Autoantibodies to glutamate receptor G1uR3 in Rasmussen's encephalitis. Science 265,648–651 (1994).
  • LuR3 is a key pathogenetic mechanism in the disease.
  • Antozzi C, Granata T, Aurisano N eta! Long-term selective IgG immunoadsorption improves Rasmussen's encephalitis. Neurology51,302–305 (1998).
  • •The use of protein A imrnunoadsorption allowed us to identify, in the imrnunoglobulin G fraction, a critical compartment the removal of which induced a significant reduction of seizures frequency and neurological deficits, thus reinforcing the concept of immune-mediated mechanism in RE.
  • Krauss GL, Campbell ML, Roche KW, Huganir RL, Niedermeyer E. Chronic steroid-responsive encephalitis without autoantibodies to glutamate receptor G1uR3. Neumlogy46,247–249 (1996).
  • Mantegazza R, Bemasconi P, Baggi F eta]. Antibodies against G1uR3 peptides are not specific for Rasmussen's encephalitis but are also present in epilepsy patients with severe, early onset disease and intractable seizures. Neumimmunal 131,179–185 (2002).
  • antibody presence in a large cohort of epileptic patients indicating that these antibodies are not specific for RE and identified another subgroup of epileptic patients, characterized by a catastrophic clinical evolution, that is highly positive for antiGluR3 antibodies.
  • Wiendl H, Bien CG, Bemasconi P eta]. G1uR3 antibodies: prevalence in focal epilepsy but no specificity for Rasmussen's encephalitis. Neumlogy57,1511–1514 (2001).
  • Frassoni C, Spreafico R, Franceschetti S eta! Labeling of rat neurons by antiGluR3 IgG from patients with Rasmussen's encephalitis. Neumlogy57, 324–327 (2001).
  • Bemasconi P, Cipelletti B, Passerini L eta! Similar binding to glutamate receptors by Rasmussen and partial epilepsy patients' sera. Neurology59, 1998–2001 (2002).
  • Baranzini SE, Laxer K, Saketkhoo R et al Analysis of antibody gene rearrangement, usage and specificity in chronic focal encephalitis. Neumlogy58,709–716 (2002).
  • Twyman RE, Gahring LC, Spless C, Rogers SW Glutamate receptor antibodies activate a subset of receptors and reveal an agonist binding site. Neuron 14,755–762 (1995).
  • He XP, Patel M, Whitney KD, Janumpalli S, Tenner A, McNamara JO. Glutamate receptor G1uR3 antibodies and death of cortical cells. Neuron 20,153–163 (1998).
  • Levite M, Fleidervish IA, Schwarz A eta! Autoantibodies to the glutamate receptor kill neurons via activation of receptor ion channel. Autoimmun. 13,61–72 (1999).
  • Carlson NG, Gahring LC, Twyman RE, Rogers SW Identification of amino acids in the glutamate receptor, G1uR3, important for antibody-binding and receptor-specific activation. J. Biol. Chem. 272, 11295–11301 (1997).
  • Whitney KD, Andrews PI, McNamara JO. Immunoglobulin G and complement immunoreactivity in the cerebral cortex of patients with Rasmussen's encephalitis. Neurology 53, 699–708 (1999).
  • Whitney KD, McNamara JO. G1uR3 autoantibodies destroy neural cells in a complement-dependent manner modulated by complement regulatory proteins. I Neurosci. 20,7307–7316 (2000).
  • Xiong Z-Q, Oian W, Suzuki K, McNamara JO. Formation of complement membrane attack complex in mammalian cerebral cortex evokes seizures and neurodegeneration. j Neurosci. 23, 955–960 (2003).
  • Yang R, Puranam RS, Butler LS et al. Autoimmunity to Munc-18 in Rasmussen's encephalitis. Neuron 28, 375–383 (2000).
  • Hata Y, Slaughter CA, Sudhof TC. Synaptic vesicle fusion complex contains unc-18 homologue bound to syntaxin. Nature 366,347–351 (1993).
  • Li Y, Uccelli A, Laxer KD et al. Local- clonal expansion of infiltrating T-lymphocytes in chronic encephalitis of Rasmussen. J. Immunal 158, 1428–1437 (1997).
  • Bien CG, Bauer J, Deckwerth TL eta! Destruction of neurons by cytotoxic T-cells: a new pathogenic mechanism in Rasmussen's encephalitis. Ann. Neural. 51, 311–318 (2002).
  • ••Paper is of extreme interest because itdemonstrates that in RE the first step in neurone degeneration is mediated by cytotoxic T-lymphocytes and that this event precedes the autoantibody attack.
  • Lieberman J. The ABCs of granule- mediated cytotoxicity: new weapons in the arsenal. Nat. Rev Immunal 3,361–370 (2003)
  • Gahring LC, Carlson NG, Meyer EL, Rogers SW Granzyme B proteolysis of a neuronal glutamate receptor generates an autoantigen and is modulated by glycosylation. j Immunal 166,1433–1438 (2001).
  • Baranzini SE, Laxer K, Bollen A, Oksenberg JR. Gene expression analysis reveals altered brain transcription of glutamate receptors and inflammatory genes in a patient with chronic focal (Rasmussen's) encephalitis. Neumiminunal 128,9-15 (2002). A new molecular approach for the analysis of altered brain tissues from RE patients, showing upregulation of inflammation-related genes and downregulation of several GluRs.
  • Villemure J-G. Hemispherectomy techniques: a critical review. In: Paediatric Epilepsy Syndromes and their Surgical Treatment. Tuxhorn I, Holthausen H, Boenigk H (Eds). John Libbey, London, UK, 729–738 (1997).
  • Villemure J-G, Mascott CR. Pen-insular hemispherotomy: surgical principles and anatomy. Neurosurgery37, 975–980 (1995).
  • Devlin AM, Cross JH, Harkness W et al Clinical outcomes of hemispherectomy for epilepsy in childhood and adolescence. Brain 126,556–566 (2003).
  • Schramm J, Kral T, Clusmann H. Transsylvian keyhole functional hemispherectomy. Neurosurgery49, 891–900 (2001).
  • Delalande 0, Pinard JM, Basevant C, Gauthe M, Plouin P, Dulac O. Hemispherotomy: A new procedure for central disconnection. Epilepsia 33 (Suppl. 3), 99–100 (1992).
  • Chinchilla D, Dulac 0, Robain 0 et al Reappraisal of Rasmussen's syndrome with special emphasis on treatment with high doses of steroids. J. Neural. Neurosurg. Psychiatry 57,1325–1333 (1994).
  • Hart YM, Cortez M, Andermann F eta]. Medical treatment of Rasmussen's syndrome (chronic encephalitis and epilepsy): effect of high-dose steroids or immunoglobulins in 19 patients. Neumlogy 44,1030–1036 (1994).
  • •A large experience on RE patients treated by corticosteroids and intravenous imrnunoglobulins.
  • Andrews PI, Dichter MA, Berkovic SF, Newton MR, McNamara JO. Plasmapheresis in Rasmussen's encephalitis. Neurology46,242–246 (1996).
  • Walsh PJ. Treatment of Rasmusseris syndrome with intravenous gammaglobulin. In: Chronic Encephalitis anc101ilepsy: Rasmusseds SyncharrE. Andermann F (Ed.). Butterworth-Heinemann, Boston, MA, USA, 201–204 (1991).
  • Wise MS, Rutledge SL, Kuzniecky RI. Rasmussen's syndrome and long-term response to gamma globulin. Pal Neural. 14,149–152 (1996).
  • Granata T, Fusco L, Gobbi G eta]. Experience with immunomodulatory treatments in Rasmussen's encephalitis. Neurology (2003) (In Press).

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.