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Autoimmune and Inflammatory Encephalopathies

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Clinical Electroencephalography

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Abstract

Autoimmune encephalitides and inflammatory encephalopathies are frequently characterized by seizures refractory to antiepileptic drugs associated with neurological, cognitive, and behavioral manifestations suggestive of a multifocal or diffuse cortical-subcortical dysfunction. Despite the increasing number of autoantibodies directed against neuronal antigens identified in recent years, conventional neurological evaluation, cerebrospinal fluid analysis, electroencephalography, and magnetic resonance imaging are still essential means to entertain a correct diagnosis. Indeed, electroencephalographic features, such as extreme delta brush, as well as electro-clinical phenomena, such as faciobrachial dystonic seizures and epilepsia partialis continua, have been recognized to be associated with specific forms of non-infective immune-mediated encephalitis. A prompt diagnosis of these neurological disorders, facilitated by a knowledgeable use of electroencephalography, is highly desirable in order to begin an early appropriate treatment and achieve a better outcome.

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References

  1. Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15:391–404.

    PubMed  PubMed Central  Google Scholar 

  2. Venkatesan A, Tunkel AR, Bloch KC, Lauring AS, Sejvar J, Bitnun A, et al. Case definitions, diagnostic algorithms, and priorities in encephalitis: consensus statement of the international encephalitis consortium. Clin Infect Dis. 2013;57:1114–28.

    CAS  PubMed  PubMed Central  Google Scholar 

  3. Britton PN, Eastwood K, Paterson B, Durrheim DN, Dale RC, Cheng AC, et al. Consensus guidelines for the investigation and management of encephalitis in adults and children in Australia and New Zealand. Intern Med J. 2015;45:563–76.

    CAS  PubMed  Google Scholar 

  4. Leypoldt F, Armangue T, Dalmau J. Autoimmune encephalopathies. Ann N Y Acad Sci. 2015;1338:94–114.

    CAS  PubMed  Google Scholar 

  5. Schmitt SE, Pargeon K, Frechette ES, Hirsch LJ, Dalmau J, Friedman D. Extreme delta brush: a unique EEG pattern in adults with anti-NMDA receptor encephalitis. Neurology. 2012;79:1094–100.

    PubMed  PubMed Central  Google Scholar 

  6. Granerod J, Ambrose HE, Davies NW, Clewley JP, Walsh AL, Morgan D, et al. Causes of encephalitis and differences in their clinical presentations in England: a multicentre, population-based prospective study. Lancet Infect Dis. 2010;10:835–44.

    PubMed  Google Scholar 

  7. Lancaster E, Dalmau J. Neuronal autoantigens-pathogenesis, associated disorders and antibody testing. Nat Rev Neurol. 2012;8:380–90.

    CAS  PubMed  PubMed Central  Google Scholar 

  8. Toledano M, Pittock SJ. Autoimmune Epilepsy. Semin Neurol. 2015;35:245–58.

    PubMed  Google Scholar 

  9. Graus F, Delattre JY, Antoine JC, Dalmau J, Giometto B, Grisold W, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. 2004;75:1135–40.

    CAS  PubMed  PubMed Central  Google Scholar 

  10. Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 2000;123:1481–94.

    PubMed  Google Scholar 

  11. Lawn ND, Westmoreland BF, Kiely MJ, Lennon VA, Vernino S. Clinical, magnetic resonance imaging, and electroencephalographic findings in paraneoplastic limbic encephalitis. Mayo Clin Proc. 2003;78:1363–8.

    PubMed  Google Scholar 

  12. Kaplan PW, Sutter R. Electroencephalography of autoimmune limbic encephalopathy. J Clin Neurophysiol. 2013;30:490–504.

    PubMed  Google Scholar 

  13. Sutter R, Fuhr P, Grize L, Marsch S, Rüegg S. Continuous video-EEG monitoring increases detection rate of nonconvulsive status epilepticus in the ICU. Epilepsia. 2011;52:453–7.

    PubMed  Google Scholar 

  14. Hamer HM, Najm I, Mohamed A, Wyllie E. Interictal epileptiform discharges in temporal lobe epilepsy due to hippocampal sclerosis versus medial temporal lobe tumors. Epilepsia. 1999;40:1261–8.

    CAS  PubMed  Google Scholar 

  15. Kaplan PW, Rossetti AO, Kaplan EH, Wieser HG. Proposition: limbic encephalitis may represent limbic status epilepticus. A review of clinical and EEG characteristics. Epilepsy Behav. 2012;24:1–6.

    PubMed  Google Scholar 

  16. Dalmau J. NMDA receptor encephalitis and other antibody-mediated disorders of the synapse: the 2016 Cotzias lecture. Neurology. 2016;87:2471–82.

    CAS  PubMed  PubMed Central  Google Scholar 

  17. Dalmau J, Tüzün E, Wu HY, Masjuan J, Rossi JE, Voloschin A, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61:25–36.

    CAS  PubMed  PubMed Central  Google Scholar 

  18. Sabater L, Gaig C, Gelpi E, Bataller L, Lewerenz J, Torres-Vega E, et al. A novel non-rapid-eye movement and rapid-eye-movement parasomnia with sleep breathing disorder associated with antibodies to IgLON5: a case series, characterisation of the antigen, and post-mortem study. Lancet Neurol. 2014;13:575–86.

    CAS  PubMed  PubMed Central  Google Scholar 

  19. Gresa-Arribas N, Planagumà J, Petit-Pedrol M, Kawachi I, Katada S, Glaser CA, et al. Human neurexin-3α antibodies associate with encephalitis and alter synapse development. Neurology. 2016;86:2235–42.

    CAS  PubMed  PubMed Central  Google Scholar 

  20. Irani SR, Bera K, Waters P, Zuliani L, Maxwell S, Zandi MS, et al. N-methyl-D-aspartate antibody encephalitis: temporal progression of clinical and paraclinical observations in a predominantly non-paraneoplastic disorder of both sexes. Brain. 2010;133:1655–67.

    PubMed  PubMed Central  Google Scholar 

  21. Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. 2013;12:157–65.

    CAS  PubMed  PubMed Central  Google Scholar 

  22. Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol. 2011;10:63–74.

    CAS  PubMed  PubMed Central  Google Scholar 

  23. Vincent A, Bien CG, Irani SR, Waters P. Autoantibodies associated with diseases of the CNS: new developments and future challenges. Lancet Neurol. 2011;10:759–72.

    CAS  PubMed  Google Scholar 

  24. Viaccoz A, Desestret V, Ducray F, Picard G, Cavillon G, Rogemond V, et al. Clinical specificities of adult male patients with NMDA receptor antibodies encephalitis. Neurology. 2014;82:556–63.

    CAS  PubMed  Google Scholar 

  25. Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008;7:1091–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  26. Iizuka T, Sakai F, Mochizuki H. Update on anti-NMDA receptor encephalitis. Brain Nerve. 2010;62:331–8.

    PubMed  Google Scholar 

  27. Shimazaki H, Ando Y, Nakano I, Dalmau J. Reversible limbic encephalitis with antibodies against the membranes of neurones of the hippocampus. BMJ Case Rep. 2009;2009:bcr07.2008.0509.

    PubMed  PubMed Central  Google Scholar 

  28. Sansing LH, Tüzün E, Ko MW, Baccon J, Lynch DR, Dalmau J. A patient with encephalitis associated with NMDA receptor antibodies. Nat Clin Pract Neurol. 2007;3:291–6.

    PubMed  PubMed Central  Google Scholar 

  29. Veciana M, Becerra JL, Fossas P, Muriana D, Sansa G, Santamarina E, et al. EEG extreme delta brush: an ictal pattern in patients with anti-NMDA receptor encephalitis. Epilepsy Behav. 2015;49:280–5.

    CAS  PubMed  Google Scholar 

  30. Zhang Y, Liu G, Di Jiang M, Li LP, Su YY. Analysis of electroencephalogram characteristics of anti-NMDA receptor encephalitis patients in China. Clin Neurophysiol. 2017;128:1227–33.

    PubMed  Google Scholar 

  31. Ikeda A, Matsui M, Hase Y, Hitomi T, Takahashi Y, Shibasaki H, et al. “Burst and slow complexes” in nonconvulsive epileptic status. Epileptic Disord. 2006;8:61–4.

    PubMed  Google Scholar 

  32. Chong DJ, Hirsch LJ. Which EEG patterns warrant treatment in the critically ill? Reviewing the evidence for treatment of periodic epileptiform discharges and related patterns. J Clin Neurophysiol. 2005;22:79–91.

    PubMed  Google Scholar 

  33. da Silva-Júnior FP, Castro LH, Andrade JQ, Bastos CG, Moreira CH, Valério RM, et al. Serial and prolonged EEG monitoring in anti-N-methyl-d-aspartate receptor encephalitis. Clin Neurophysiol. 2014;125:1541–4.

    PubMed  Google Scholar 

  34. VanHaerents S, Stillman A, Inoa V, Searls DE, Herman ST. Early and persistent “extreme delta brush” in a patient with anti-NMDA receptor encephalitis. Epilepsy Behav Case Rep. 2014;2:67–70.

    PubMed  PubMed Central  Google Scholar 

  35. Yoo JY, Rampal N, Petroff OA, Hirsch LJ, Gaspard N. Brief potentially ictal rhythmic discharges in critically ill adults. JAMA Neurol. 2014;71:454–62.

    PubMed  Google Scholar 

  36. Abbas A, Garg A, Jain R, Mazibrada G, Jacob S. Extreme delta brushes and BIRDs in the EEG of anti-NMDA-receptor encephalitis. Pract Neurol. 2016;16:326–7.

    PubMed  Google Scholar 

  37. Gitiaux C, Simonnet H, Eisermann M, Leunen D, Dulac O, Nabbout R, et al. Early electro-clinical features may contribute to diagnosis of the anti-NMDA receptor encephalitis in children. Clin Neurophysiol. 2013;124:2354–61.

    PubMed  Google Scholar 

  38. Kirkpatrick MP, Clarke CD, Sonmezturk HH, Abou-Khalil B. Rhythmic delta activity represents a form of nonconvulsive status epilepticus in anti-NMDA receptor antibody encephalitis. Epilepsy Behav. 2011;20:392–4.

    PubMed  Google Scholar 

  39. Buckley C, Oger J, Clover L, Tüzün E, Carpenter K, Jackson M, et al. Potassium channel antibodies in two patients with reversible limbic encephalitis. Ann Neurol. 2001;50:73–8.

    CAS  PubMed  Google Scholar 

  40. Thieben MJ, Lennon VA, Boeve BF, Aksamit AJ, Keegan M, Vernino S. Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody. Neurology. 2004;62:1177–82.

    CAS  PubMed  Google Scholar 

  41. Lai M, Huijbers MG, Lancaster E, Graus F, Bataller L, Balice-Gordon R, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol. 2010;9:776–85.

    CAS  PubMed  PubMed Central  Google Scholar 

  42. Irani SR, Alexander S, Waters P, Kleopa KA, Pettingill P, Zuliani L, et al. Antibodies to Kv1 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. 2010;133:2734–48.

    PubMed  PubMed Central  Google Scholar 

  43. Klein CJ, Lennon VA, Aston PA, McKeon A, O’Toole O, Quek A, et al. Insights from LGI1 and CASPR2 potassium channel complex autoantibody subtyping. JAMA Neurol. 2013;70:229–34.

    PubMed  PubMed Central  Google Scholar 

  44. van Sonderen A, Thijs RD, Coenders EC, Jiskoot LC, Sanchez E, de Bruijn MA, et al. Anti-LGI1 encephalitis: clinical syndrome and long-term follow-up. Neurology. 2016;87:1449–56.

    PubMed  Google Scholar 

  45. Navarro V, Kas A, Apartis E, Chami L, Rogemond V, Levy P, et al. Motor cortex and hippocampus are the two main cortical targets in LGI1-antibody encephalitis. Brain. 2016;139:1079–93.

    PubMed  Google Scholar 

  46. Irani SR, Michell AW, Lang B, Pettingill P, Waters P, Johnson MR, et al. Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Ann Neurol. 2011;69:892–900.

    PubMed  Google Scholar 

  47. Wennberg R, Steriade C, Chen R, Andrade D. Frontal infraslow activity marks the motor spasms of anti-LGI1 encephalitis. Clin Neurophysiol. 2018;129:59–68.

    PubMed  Google Scholar 

  48. Rocamora R, Becerra JL, Fossas P, Gomez M, Vivanco-Hidalgo RM, Mauri JA, et al. Pilomotor seizures: an autonomic semiology of limbic encephalitis? Seizure. 2014;23:670–3.

    PubMed  Google Scholar 

  49. Irani SR, Stagg CJ, Schott JM, Rosenthal CR, Schneider SA, Pettingill P, et al. Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype. Brain. 2013;136:3151–62.

    PubMed  Google Scholar 

  50. Andrade DM, Tai P, Dalmau J, Wennberg R. Tonic seizures: a diagnostic clue of anti-LGI1 encephalitis? Neurology. 2011;76:1355–7.

    PubMed  PubMed Central  Google Scholar 

  51. Irani SR, Schott JM, Vincent A, Smith SJ. Tonic Seizures: a diagnostic clue of anti-LGI1 encephalitis? Neurology. 2011;77:2140–1.. author reply 2141–3

    PubMed  Google Scholar 

  52. Steriade C, Mirsattari SM, Murray BJ, Wennberg R. Subclinical temporal EEG seizure pattern in LGI1-antibody–mediated encephalitis. Epilepsia. 2016;57:e155–60.

    CAS  PubMed  Google Scholar 

  53. van Sonderen A, Ariño H, Petit-Pedrol M, Leypoldt F, Körtvélyessy P, Lancaster E, et al. The clinical spectrum of Caspr2 antibody-associated disease. Neurology. 2016;87:521–8.

    PubMed  PubMed Central  Google Scholar 

  54. Lai M, Hughes EG, Peng X, Zhou L, Gleichman AJ, Shu H, et al. AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol. 2009;65:424–34.

    CAS  PubMed  PubMed Central  Google Scholar 

  55. Höftberger R, van Sonderen A, Leypoldt F, Houghton D, Geschwind M, Gelfand J, et al. Encephalitis and AMPA receptor antibodies: novel findings in a case series of 22 patients. Neurology. 2015;84:2403–12.

    PubMed  PubMed Central  Google Scholar 

  56. Lancaster E, Lai M, Peng X, Hughes E, Constantinescu R, Raizer J, et al. Antibodies to the GABA(B) receptor in limbic encephalitis with seizures: case series and characterisation of the antigen. Lancet Neurol. 2010;9:67–76.

    CAS  PubMed  Google Scholar 

  57. Höftberger R, Titulaer MJ, Sabater L, Dome B, Rózsás A, Hegedus B, et al. Encephalitis and GABAB receptor antibodies: novel findings in a new case series of 20 patients. Neurology. 2013;81:1500–6.

    PubMed  PubMed Central  Google Scholar 

  58. Petit-Pedrol M, Armangue T, Peng X, Bataller L, Cellucci T, Davis R, et al. Encephalitis with refractory seizures, status epilepticus, and antibodies to the GABAA receptor: a case series, characterisation of the antigen, and analysis of the effects of antibodies. Lancet Neurol. 2014;13:276–86.

    CAS  PubMed  PubMed Central  Google Scholar 

  59. Chong JY, Rowland LP, Utiger RD. Hashimoto encephalopathy: syndrome or myth? Arch Neurol. 2003;60:164–71.

    PubMed  Google Scholar 

  60. Castillo P, Woodruff B, Caselli R, Vernino S, Lucchinetti C, Swanson J, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol. 2006;63:197–202.

    PubMed  Google Scholar 

  61. Hollowell JG, Staehling NW, Flanders WD, Hannon WH, Gunter EW, Spencer CA, et al. Serum TSH, T(4), and thyroid antibodies in the United States population (1988 to 1994): National Health and nutrition examination survey (NHANES III). J Clin Endocrinol Metab. 2002;87:489–99.

    CAS  PubMed  Google Scholar 

  62. Hilberath JM, Schmidt H, Wolf GK. Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): case report of reversible coma and status epilepticus in an adolescent patient and review of the literature. Eur J Pediatr. 2014;173:1263–73.

    PubMed  Google Scholar 

  63. Montagna G, Imperiali M, Agazzi P, D’Aurizio F, Tozzoli R, Feldt-Rasmussen U, et al. Hashimoto’s encephalopathy: a rare proteiform disorder. Autoimmun Rev. 2016;15:466–76.

    PubMed  Google Scholar 

  64. Fatourechi V. Hashimoto’s encephalopathy: myth or reality? An endocrinologist’s perspective. Best Pract Res Clin Endocrinol Metab. 2005;19:53–66.

    CAS  PubMed  Google Scholar 

  65. Forchetti CM, Katsamakis G, Garron DC. Autoimmune thyroiditis and a rapidly progressive dementia: global hypoperfusion on SPECT scanning suggests a possible mechanism. Neurology. 1997;49:623–6.

    CAS  PubMed  Google Scholar 

  66. Lee SW, Donlon S, Caplan JP. Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) or Hashimoto’s encephalopathy: a case and review. Psychosomatics. 2011;52:99–108.

    PubMed  Google Scholar 

  67. Schäuble B, Castillo PR, Boeve BF, Westmoreland BF. EEG findings in steroid-responsive encephalopathy associated with autoimmune thyroiditis. Clin Neurophysiol. 2003;114:32–7.

    PubMed  Google Scholar 

  68. Rodriguez AJ, Jicha GA, Steeves TD, Benarroch EE, Westmoreland BF. EEG changes in a patient with steroid-responsive encephalopathy associated with antibodies to thyroperoxidase (SREAT, Hashimoto’s encephalopathy). J Clin Neurophysiol. 2006;23:371–3.

    PubMed  Google Scholar 

  69. Pedroso JL, Knobel M, Barsottini OG, Caboclo LO, Knobel E. The relevance of electroencephalogram as a follow-up test in Hashimoto encephalopathy course after corticosteroids therapy. Acta Neurol Belg. 2012;112:409–11.

    PubMed  Google Scholar 

  70. Varadkar S, Bien CG, Kruse CA, Jensen FE, Bauer J, Pardo CA, et al. Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurol. 2014;13:195–205.

    PubMed  PubMed Central  Google Scholar 

  71. Bien CG, Tiemeier H, Sassen R, Kuczaty S, Urbach H, von Lehe M, et al. Rasmussen encephalitis: incidence and course under randomized therapy with tacrolimus or intravenous immunoglobulins. Epilepsia. 2013;54:543–50.

    CAS  PubMed  Google Scholar 

  72. Thomas JE, Reagan TJ, Klass DW. Epilepsia partialis continua. A review of 32 cases. Arch Neurol. 1977;34:266–75.

    CAS  PubMed  Google Scholar 

  73. Obeso JA, Rothwell JC, Marsden CD. The spectrum of cortical myoclonus. From focal reflex jerks to spontaneous motor epilepsy. Brain. 1985;108:193–224.

    PubMed  Google Scholar 

  74. Longaretti F, Dunkley C, Varadkar S, Vargha-Khadem F, Boyd SG, Cross JH. Evolution of the EEG in children with Rasmussen’s syndrome. Epilepsia. 2012;53:1539–45.

    PubMed  Google Scholar 

  75. 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: Andermann F, editor. Chronic encephalitis and epilepsy. Rasmussen’s syndrome. Boston: Butterworth-Heinemann; 1991. p. 7–35.

    Google Scholar 

  76. Bien CG, Granata T, Antozzi C, Cross JH, Dulac O, Kurthen M, et al. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. Brain. 2005;128:454–71.

    CAS  PubMed  Google Scholar 

  77. Derry C, Dale RC, Thom M, Miller DH, Giovannoni G. Unihemispheric cerebral vasculitis mimicking Rasmussen’s encephalitis. Neurology. 2002;58:327–8.

    CAS  PubMed  Google Scholar 

  78. Oguni H, Andermann F, Rasmussen TB. The syndrome of chronic encephalitis and epilepsy. A study based on the MNI series of 48 cases. Adv Neurol. 1992;57:419–33.

    CAS  PubMed  Google Scholar 

  79. Bien CG, Widman G, Urbach H, Sassen R, Kuczaty S, Wiestler OD, et al. The natural history of Rasmussen’s encephalitis. Brain. 2002;125:1751–9.

    PubMed  Google Scholar 

  80. Granata T, Gobbi G, Spreafico R, Vigevano F, Capovilla G, Ragona F, et al. Rasmussen’s encephalitis: early characteristics allow diagnosis. Neurology. 2003;60:422–5.

    CAS  PubMed  Google Scholar 

  81. Juul-Jensen P, Denny-Brown D. Epilepsia Partialis continua. Arch Neurol. 1966;15:563–78.

    CAS  PubMed  Google Scholar 

  82. Bancaud J, Bonis A, Talairach J, Bordas-Ferrer M, Buser P. Kojewnikow syndrome and somato-motor attacks (clinical, E.E.G., E.M.G. and S.E.E.G. study). Encéphale. 1970;59:391–438.

    CAS  PubMed  Google Scholar 

  83. Wieser HG, Graf HP, Bernoulli C, Siegfried J. Quantitative analysis of intracerebral recordings in epilepsia partialis continua. Electroencephalogr Clin Neurophysiol. 1978;44:14–22.

    CAS  PubMed  Google Scholar 

  84. Cockerell OC, Rothwell J, Thompson PD, Marsden CD, Shorvon SD. Clinical and physiological features of epilepsia partialis continua. Cases ascertained in the UK. Brain. 1996;119:393–407.

    PubMed  Google Scholar 

  85. Mameniškienė R, Wolf P. Epilepsia partialis continua: a review. Seizure. 2017;44:74–80.

    PubMed  Google Scholar 

  86. So NK, Gloor P. Electroencephalographic and electrocorticographic findings in chronic encephalitis of the Rasmussen type. In: Andermann F, editor. Chronic encephalitis and epilepsy. Rasmussen’s syndrome. Boston: Butterworth-Heinemann; 1991. p. 37–45.

    Google Scholar 

  87. Barkley GL, Baumgartner C. MEG and EEG in epilepsy. J Clin Neurophysiol. 2003;20:163–78.

    PubMed  Google Scholar 

  88. Rossi Sebastiano D, Visani E, Duran D, Freri E, Panzica F, Chiapparini L, et al. Epileptic spikes in Rasmussen’s encephalitis: migratory pattern and short-term evolution. A MEG study. Clin Neurophysiol. 2017;128:1898–905.

    PubMed  Google Scholar 

  89. Marrie RA, Reider N, Cohen J, Trojano M, Sorensen PS, Cutter G, et al. A systematic review of the incidence and prevalence of sleep disorders and seizure disorders in multiple sclerosis. Mult Scler. 2015;21:342–9.

    PubMed  PubMed Central  Google Scholar 

  90. Martínez-Juárez IE, López-Meza E, González-Aragón Mdel CF, Ramírez-Bermúdez J, Corona T. Epilepsy and multiple sclerosis: increased risk among progressive forms. Epilepsy Res. 2009;84:250–3.

    PubMed  Google Scholar 

  91. Catenoix H, Marignier R, Ritleng C, Dufour M, Mauguière F, Confavreux C, et al. Multiple sclerosis and epileptic seizures. Mult Scler. 2011;17:96–102.

    CAS  PubMed  Google Scholar 

  92. Burman J, Zelano J. Epilepsy in multiple sclerosis: a nationwide population-based register study. Neurology. 2017;89:2462–8.

    PubMed  Google Scholar 

  93. Gasparini S, Ferlazzo E, Ascoli M, Sueri C, Cianci V, Russo C, et al. Risk factors for unprovoked epileptic seizures in multiple sclerosis: a systematic review and meta-analysis. Neurol Sci. 2017;38:399–406.

    PubMed  Google Scholar 

  94. Koch M, Uyttenboogaart M, Polman S, De Keyser J. Seizures in multiple sclerosis. Epilepsia. 2008;49:948–53.

    PubMed  Google Scholar 

  95. Spatt J, Chaix R, Mamoli B. Epileptic and non-epileptic seizures in multiple sclerosis. J Neurol. 2001;248:2–9.

    CAS  PubMed  Google Scholar 

  96. Kinnunen E, Wikström J. Prevalence and prognosis of epilepsy in patients with multiple sclerosis. Epilepsia. 1986;27:729–33.

    CAS  PubMed  Google Scholar 

  97. Poser CM, Brinar VV. Epilepsy and multiple sclerosis. Epilepsy Behav. 2003;4:6–12.

    PubMed  Google Scholar 

  98. Thompson AJ, Kermode AG, Moseley IF, MacManus DG, McDonald WI. Seizures due to multiple sclerosis: seven patients with MRI correlations. J Neurol Neurosurg Psychiatry. 1993;56:1317–20.

    CAS  PubMed  PubMed Central  Google Scholar 

  99. Hess DC, Sethi KD. Epilepsia partialis continua in multiple sclerosis. Int J Neurosci. 1990;50:109–11.

    CAS  PubMed  Google Scholar 

  100. Spatt J, Goldenberg G, Mamoli B. Epilepsia partialis continua in multiple sclerosis. Lancet. 1995;345:658–9.

    CAS  PubMed  Google Scholar 

  101. Nyquist PA, Cascino GD, Rodriguez M. Seizures in patients with multiple sclerosis seen at Mayo Clinic, Rochester, Minn, 1990–1998. Mayo Clin Proc. 2001;76:983–6.

    CAS  PubMed  Google Scholar 

  102. Striano P, Striano S, Carrieri PB, Boccella P. Epilepsia partialis continua as a first symptom of multiple sclerosis: electrophysiological study of one case. Mult Scler. 2003;9:199–203.

    CAS  PubMed  Google Scholar 

  103. Maingueneau F, Honnorat J, Isnard J, Tommasi-Davenas C, Derex L, Nighoghossian N, et al. Partial non-convulsive status epilepsy in multiple sclerosis. Neurophysiol Clin. 1999;29:463–72.

    CAS  PubMed  Google Scholar 

  104. Chabolla DR, Moore JL, Westmoreland BF. Periodic lateralized epileptiform discharges in multiple sclerosis. Electroencephalogr Clin Neurophysiol. 1996;98:5–8.

    CAS  PubMed  Google Scholar 

  105. Iragui-Madoz V. Electrophysiology of multiple sclerosis. In: Daly DD, Pedley TA, editors. Current practice of clinical electroencephalography. 2nd ed. New York: Raven Press; 1990. p. 708–38.

    Google Scholar 

  106. Grabow JD. Optimal recordings techniques and activation procedures: children and adults. In: Wada JA, Ellingson RJ, editors. Clinical neurophysiology of epilepsy. Amsterdam: Elsevier Science; 1990. p. 39–77.

    Google Scholar 

  107. Calabrese LH, Mallek JA. Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore). 1988;67:20–39.

    CAS  Google Scholar 

  108. Salvarani C, Brown RD Jr, Hunder GG. Adult primary central nervous system vasculitis. Lancet. 2012;380:767–77.

    PubMed  Google Scholar 

  109. Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller DV, et al. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol. 2007;62:442–51.

    PubMed  Google Scholar 

  110. Jennekens FG, Kater L. The central nervous system in systemic lupus erythematosus. Part 1. Clinical syndromes: a literature investigation. Rheumatology (Oxford). 2002;41:605–18.

    CAS  Google Scholar 

  111. Herranz MT, Rivier G, Khamashta MA, Blaser KU, Hughes GR. Association between antiphospholipid antibodies and epilepsy in patients with systemic lupus erythematosus. Arthritis Rheum. 1994;37:568–71.

    CAS  PubMed  Google Scholar 

  112. Mackworth-Young CG, Hughes GR. Epilepsy: an early symptom of systemic lupus erythematosus. J Neurol Neurosurg Psychiatry. 1985;48:185.

    CAS  PubMed  PubMed Central  Google Scholar 

  113. Appenzeller S, Cendes F, Costallat LT. Epileptic seizures in systemic lupus erythematosus. Neurology. 2004;63:1808–12.

    PubMed  Google Scholar 

  114. González-Duarte A, Cantú-Brito CG, Ruano-Calderón L, García-Ramos G. Clinical description of seizures in patients with systemic lupus erythematosus. Eur Neurol. 2008;59:320–3.

    PubMed  Google Scholar 

  115. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet’s disease. N Engl J Med. 1999;341:1284–91.

    CAS  PubMed  Google Scholar 

  116. Verity DH, Wallace GR, Vaughan RW, Stanford MR. Behçet’s disease: from Hippocrates to the third millennium. Br J Ophthalmol. 2003;87:1175–83.

    CAS  PubMed  PubMed Central  Google Scholar 

  117. Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V, et al. The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003;82:60–76.

    Google Scholar 

  118. Akman-Demir G, Serdaroglu P, Tasçi B. Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. The Neuro-Behçet Study Group. Brain. 1999;122:2171–82.

    PubMed  Google Scholar 

  119. Al-Fahad SA, Al-Araji AH. Neuro-Behcet’s disease in Iraq: a study of 40 patients. J Neurol Sci. 1999;170:105–11.

    CAS  PubMed  Google Scholar 

  120. Siva A, Kantarci OH, Saip S, Altintas A, Hamuryudan V, Islak C, et al. Behçet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol. 2001;248:95–103.

    CAS  PubMed  Google Scholar 

  121. Aykutlu E, Baykan B, Serdaroglu P, Gökyigit A, Akman-Demir G. Epileptic seizures in Behçet disease. Epilepsia. 2002;43:832–5.

    PubMed  Google Scholar 

  122. Mead S, Kidd D, Good C, Plant G. Behcet’s syndrome may present with partial seizures. J Neurol Neurosurg Psychiatry. 2000;68:392–3.

    CAS  PubMed  PubMed Central  Google Scholar 

  123. Aktekin B, Doǧan EA, Oǧuz Y, Karaali K. Epilepsia partialis continua in a patient with Behçet’s disease. Clin Neurol Neurosurg. 2006;108:392–5.

    PubMed  Google Scholar 

  124. Al-Araji A, Kidd DP. Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol. 2009;8:192–204.

    PubMed  Google Scholar 

  125. Stern BJ, Krumholz A, Johns C, Scott P, Nissim J. Sarcoidosis and its neurological manifestations. Arch Neurol. 1985;42:909–17.

    CAS  PubMed  Google Scholar 

  126. James DG, Sharma OP. Neurosarcoidosis. Proc R Soc Med. 1967;60:1169–70.

    CAS  PubMed  PubMed Central  Google Scholar 

  127. Lynch JP 3rd. Neurosarcoidosis: how good are the diagnostic tests? J Neuroophthalmol. 2003;23:187–9.

    PubMed  Google Scholar 

  128. Moore FG, Andermann F, Richardson J, Tampieri D, Giaccone R. The role of MRI and nerve root biopsy in the diagnosis of neurosarcoidosis. Can J Neurol Sci. 2001;28:349–53.

    CAS  PubMed  Google Scholar 

  129. Krumholz A, Stern BJ, Stern EG. Clinical implications of seizures in neurosarcoidosis. Arch Neurol. 1991;48:842–4.

    CAS  PubMed  Google Scholar 

  130. Sponsler JL, Werz MA, Maciunas R, Cohen M. Neurosarcoidosis presenting with simple partial seizures and solitary enhancing mass: case reports and review of the literature. Epilepsy Behav. 2005;6:623–30.

    PubMed  Google Scholar 

  131. Besur S, Bishnoi R, Talluri SK. Neurosarcoidosis: rare initial presentation with seizures and delirium. QJM. 2011;104:801–3.

    CAS  PubMed  Google Scholar 

  132. Gullapalli D, Phillips LH 2nd. Neurologic manifestations of sarcoidosis. Neurol Clin. 2002;20:59–83.

    PubMed  Google Scholar 

  133. Delaney P. Seizures in sarcoidosis: a poor prognosis. Ann Neurol. 1980;7:494.

    CAS  PubMed  Google Scholar 

  134. Delaney P. Neurologic manifestations in sarcoidosis: review of the literature, with a report of 23 cases. Ann Intern Med. 1977;87:336–45.

    CAS  PubMed  Google Scholar 

  135. Brophy GM, Bell R, Claassen J, Alldredge B, Bleck TP, Glauser T, et al. Guidelines for the evaluation and management of status epilepticus. Neurocrit Care. 2012;17:3–23.

    PubMed  Google Scholar 

  136. Tan RY, Neligan A, Shorvon SD. The uncommon causes of status epilepticus: a systematic review. Epilepsy Res. 2010;91:111–22.

    CAS  PubMed  Google Scholar 

  137. Watemberg N, Segal G. A suggested approach to the etiologic evaluation of status epilepticus in children: what to seek after the usual causes have been ruled out. J Child Neurol. 2010;25:203–11.

    PubMed  Google Scholar 

  138. Hirsch LJ, Gaspard N. Status Epilepticus. Continuum (Minneap Minn). 2013;19:767–94.

    Google Scholar 

  139. Jayalakshmi S, Vooturi S, Sahu S, Yada PK, Mohandas S. Causes and outcomes of new onset status epilepticus and predictors of refractoriness to therapy. J Clin Neurosci. 2016;26:89–94.

    PubMed  Google Scholar 

  140. Wilder-Smith EP, Lim EC, Teoh HL, Sharma VK, Tan JJ, Chan BP, et al. The NORSE (new-onset refractory status epilepticus) syndrome: defining a disease entity. Ann Acad Med Singap. 2005;34:417–20.

    CAS  PubMed  Google Scholar 

  141. Costello DJ, Kilbride RD, Cole AJ. Cryptogenic new onset refractory status epilepticus (NORSE) in adults-infectious or not? J Neurol Sci. 2009;277:26–31.

    PubMed  Google Scholar 

  142. Gaspard N, Foreman BP, Alvarez V, Cabrera Kang C, Probasco JC, Jongeling AC, et al. New-onset refractory status epilepticus: etiology, clinical features, and outcome. Neurology. 2015;85:1604–13.

    PubMed  PubMed Central  Google Scholar 

  143. Baxter P, Clarke A, Cross H, Harding B, Hicks E, Livingston J, et al. Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status. Seizure. 2003;12:379–87.

    PubMed  Google Scholar 

  144. Kramer U, Chi CS, Lin KL, Specchio N, Sahin M, Olson H, et al. Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children. Epilepsia. 2011;52:1956–65.

    PubMed  Google Scholar 

  145. van Baalen A, Häusler M, Boor R, Rohr A, Sperner J, Kurlemann G, et al. Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood. Epilepsia. 2010;51:1323–8.

    PubMed  Google Scholar 

  146. Sakuma H. Acute encephalitis with refractory, repetitive partial seizures. Brain and Development. 2009;31:510–4.

    PubMed  Google Scholar 

  147. Mikaeloff Y, Jambaqué I, Hertz-Pannier L, Zamfirescu A, Adamsbaum C, Plouin P, et al. Devastating epileptic encephalopathy in school-aged children (DESC): a pseudo encephalitis. Epilepsy Res. 2006;69:67–79.

    CAS  PubMed  Google Scholar 

  148. Hirsch LJ, Gaspard N, van Baalen A, Nabbout R, Demeret S, Loddenkemper T, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia. 2018;59:739–44.

    PubMed  Google Scholar 

  149. Gaspard N, Hirsch LJ, Sculier C, Loddenkemper T, van Baalen A, Lancrenon J, et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): state of the art and perspectives. Epilepsia. 2018;59:745–52.

    PubMed  Google Scholar 

  150. Meletti S, Slonkova J, Mareckova I, Monti G, Specchio N, Hon P, et al. Claustrum damage and refractory status epilepticus following febrile illness. Neurology. 2015;85:1224–32.

    PubMed  PubMed Central  Google Scholar 

  151. Farias-Moeller R, Bartolini L, Staso K, Schreiber JM, Carpenter JL. Early ictal and interictal patterns in FIRES: the sparks before the blaze. Epilepsia. 2017;58:1340–8.

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Clinical and Experimental Epileptology, Foundation IRCCS Neurological Institute “Carlo Besta”, Milan, Italy

    Andrea Stabile

  2. Department of Neurology, San Gerardo Hospital ASST Monza, University of Milano Bicocca, Monza, Italy

    Andrea Stabile

  3. Epilepsy Monitoring Unit “Paolo Zorzi”, Division of Clinical and Experimental Epileptology, Foundation IRCCS Neurological Institute “Carlo Besta”, Milan, Italy

    Flavio Villani

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  2. Flavio Villani
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  1. Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy

    Oriano Mecarelli

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Stabile, A., Villani, F. (2019). Autoimmune and Inflammatory Encephalopathies. In: Mecarelli, O. (eds) Clinical Electroencephalography. Springer, Cham. https://doi.org/10.1007/978-3-030-04573-9_40

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