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Paediatric Status Epilepticus

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Clinical Electroencephalography

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Abstract

Status epilepticus (SE) is one of the most common paediatric neurologic emergencies, requiring timely identification and emergency treatment to reduce morbidity and mortality. The evaluation and management of SE are similar in adults and children; however, specific conditions and syndromes with convulsive status epilepticus (CSE) and NCSE occur in children. In NCSE, a correct diagnosis usually needs an EEG. Clinical suspicion of NCSE arises in patients who have disturbance of consciousness with or without minor motor phenomena (e.g., perioral or periorbital low-amplitude jerking). In some cases, only EEG can reveal the epileptiform or rhythmic discharges that lead to diagnosis. The task force recommends describing the EEG correlate of status in a patient using the following descriptors, name of pattern, morphology, location, time-related features, modulation, and effect of intervention, and use the proposed terminology by the American Clinical Neurophysiology Society and the diagnostic “Salzburg EEG criteria for NCSE”. We will describe the electro-clinical features and EEG pattern of the peculiar forms of CSE/NCSE that might be seen in some paediatric epilepsies such as Dravet syndrome; Panayiotopoulos syndrome; Ring chromosome 20-related epilepsy; Angelman syndrome; Rett syndrome; Epilepsy with myoclonic-atonic seizures (Doose syndrome); myoclonic status in nonprogressive encephalopathies; mitochondrial diseases causing SE, absence status, and tonic status in Lennox-Gastaut syndrome; and encephalopathy with electrical status epilepticus in sleep (ESES).

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References

  1. 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 

  2. Nishiyama M, Nagase H, Tanaka T, Fujita K, Maruyama A, Toyoshima D, et al. Demographics and outcomes of patients with pediatric febrile convulsive status epilepticus. Pediatr Neurol. 2015;52:499–503.

    PubMed  Google Scholar 

  3. Jafarpour S, Stredny CM, Piantino J, Chapman KE. Baseline and outcome assessment in pediatric status epilepticus. Seizure. 2018. https://doi.org/10.1016/j.seizure.2018.04.019.

  4. Lv RJ, Wang Q, Cui T, Zhu F, Shao XQ. Status epilepticus-related etiology, incidence and mortality: a meta-analysis. Epilepsy Res. 2017;136:12–7.

    PubMed  Google Scholar 

  5. Barnard C, Wirrell E. Does status epilepticus in children cause developmental deterioration and exacerbation of epilepsy? J Child Neurol. 1999;14:787–94.

    CAS  PubMed  Google Scholar 

  6. Barry E, Hauser WA. Status epilepticus: the interaction of epilepsy and acute brain disease. Neurology. 1993;43:1473–8.

    CAS  PubMed  Google Scholar 

  7. Krumholz A, Sung GY, Fisher RS, Barry E, Bergey GK, Grattan LM. Complex partial status epilepticus accompanied by serious morbidity and mortality. Neurology. 1995;45:1499–504.

    CAS  PubMed  Google Scholar 

  8. Riviello JJ. Pediatric status epilepticus: initial management and the special syndromes of status epilepticus in children. In: Drislane FW, Kaplan PW, editors. Status epilepticus: a clinical perspective. Totowa, NJ: Humana Press; 2018. p. 333–52.

    Google Scholar 

  9. Trinka E, Cock H, Hesdorffer D, Rossetti AO, Scheffer IE, Shinnar S, et al. A definition and classification of status epilepticus - report of the ILAE task force on classification of status epilepticus. Epilepsia. 2015;56:1515–23.

    PubMed  Google Scholar 

  10. Neligan A, Shorvon SD. Prognostic factors, morbidity and mortality in tonic-clonic status epilepticus: a review. Epilepsy Res. 2011;93:1–10.

    CAS  PubMed  Google Scholar 

  11. Aicardi J, Chevrie JJ. Convulsive status epilepticus in infants and children: a study of 239 cases. Epilepsia. 1970;11:187–97.

    CAS  PubMed  Google Scholar 

  12. DeLorenzo RJ, Towne AR, Pellock JM, Ko D. Status epilepticus in children, adults, and the elderly. Epilepsia. 1992;33(Suppl 4):S15–25.

    PubMed  Google Scholar 

  13. Morton LD, Garnett L, Towne A, Waterhouse E, Brown A, Byers SF, et al. Mortality of status epilepticus in the first year of life. Epilepsia. 2001;42(Suppl 7):165.

    Google Scholar 

  14. Young GB, Jordan KG, Doig GS. An assessment of nonconvulsive seizures in the intensive care unit using continuous EEG monitoring: an investigation of variables associated with mortality. Neurology. 1996;47:83–9.

    CAS  PubMed  Google Scholar 

  15. Hirsch LJ, Laroche SM, Gaspard N, Gerard E, Svoronos A, Herman ST, et al. American clinical neurophysiology society’s standardized critical care EEG terminology: 2012 version. J Clin Neurophysiol. 2013;30:1–27.

    CAS  PubMed  Google Scholar 

  16. Beniczky S, Hirsch LJ, Kaplan PW, Pressler R, Bauer G, Aurlien H, et al. Unified EEG terminology and criteria for nonconvulsive status epilepticus. Epilepsia. 2013;54(Suppl 6):28–9.

    PubMed  Google Scholar 

  17. Leitinger M, Trinka E, Gardella E, Rohracher A, Kalss G, Qerama E, et al. Diagnostic accuracy of the Salzburg EEG criteria for non-convulsive status epilepticus: a retrospective study. Lancet Neurol. 2016;15:1054–62.

    PubMed  Google Scholar 

  18. Mameniškienė R, Bast T, Bentes C, Canevini MP, Dimova P, Granata T, et al. Clinical course and variability of non-Rasmussen, nonstroke motor and sensory epilepsia partialis continua: a European survey and analysis of 65 cases. Epilepsia. 2011;52:1168–76.

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  20. Chauvel P, Liegeois-Chauvel C, Marquis P, Bancaud J. Distinction between the myoclonus-related potential and the epileptic spike in epilepsia partialis continua. Electroencephalogr Clin Neurophysiol. 1986;64:304–7.

    CAS  PubMed  Google Scholar 

  21. Pandian JD, Thomas SV, Santoshkumar B, Radhakrishnan K, Sarma PS, Joseph S, et al. Epilepsia partialis continua - a clinical and electroencephalography study. Seizure. 2002;11:437–41.

    CAS  PubMed  Google Scholar 

  22. Dulac O, Dravet C, Plouin P, Bureau M, Ponsot G, Gerbaut L, et al. Nosological aspects of epilepsia partialis continua in children. Arch Fr Pediatr. 1983;40:689–95.. [article in French]

    CAS  PubMed  Google Scholar 

  23. 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 

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

    Google Scholar 

  25. Ian Andrews P, McNamara JO, Lewis DV. Clinical and electroencephalographic correlates in Rasmussen’s encephalitis. Epilepsia. 1997;38:189–94.

    Google Scholar 

  26. 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 

  27. Saitoh M, Shinohara M, Hoshino H, Kubota M, Amemiya K, Takanashi JL, et al. Mutations of the SCN1A gene in acute encephalopathy. Epilepsia. 2012;53:558–64.

    CAS  PubMed  Google Scholar 

  28. Dalla Bernardina B, Capovilla G, Chiamenti C, Trevisan E, Colamaria V, Fontana E. Myoclonic epilepsies, advances in neurology series. In: Wolf P, Dam MJ, editors. Advances in epileptology, vol. 16. New York: Raven Press; 1987. p. 175–9.

    Google Scholar 

  29. Oguni H, Hayashi K, Awaya Y, Fukuyama Y, Osawa M. Severe myoclonic epilepsy in infants – a review based on the Tokyo Women’s medical university series of 84 cases. Brain Dev. 2001;23:736–48.

    CAS  PubMed  Google Scholar 

  30. Bureau M, Bernardina BD. Electroencephalographic characteristics of Dravet syndrome. Epilepsia. 2011;52(Suppl. 2):13–23.

    PubMed  Google Scholar 

  31. Ohmori I, Ohtsuka Y, Murakami N, Asano T, Hattori J. Analysis of ictal EEG in severe myoclonic epilepsy in infancy. Epilepsia. 2001;42(Suppl 6):54.

    Google Scholar 

  32. Wakai S, Ikehata M, Nihira H, Ito N, Sueoka H, Kawamoto Y, et al. “Obtundation status (Dravet)” caused by complex partial status epilepticus in a patient with severe myoclonic epilepsy in infancy. Epilepsia. 1996;37:1020–2.

    CAS  PubMed  Google Scholar 

  33. Dravet C, Bureau M, Oguni H, Fukuyama Y, Cokar O. Severe myoclonic epilepsy in infancy (Dravet syndrome). In: Roger J, Bureau M, Dravet C, Genton P, Tassinari CA, Wolf W, editors. Epileptic syndromes in infancy, childhood and adolescence. 4th ed. Montrouge: John Libbey Eurotext; 2005. p. 89–113.

    Google Scholar 

  34. Tian X, Ye J, Zeng Q, Zhang J, Yang X, Liu A, et al. The clinical outcome and neuroimaging of acute encephalopathy after status epilepticus in Dravet syndrome. Dev Med Child Neurol. 2018;60:566–73.

    PubMed  Google Scholar 

  35. Panayiotopoulos CP. Vomiting as an ictal manifestation of epileptic seizures and syndromes. J Neurol Neurosurg Psychiatry. 1988;51:1448–51.

    CAS  PubMed  PubMed Central  Google Scholar 

  36. Ferrie CD, Caraballo R, Covanis A, Demirbilek V, Dervent A, Fejerman N, et al. Autonomic status epilepticus in panayiotopoulos syndrome and other childhood and adult epilepsies: a consensus view. Epilepsia. 2007;48:1165–72.

    PubMed  Google Scholar 

  37. Beaumanoir A. Occipital seizures and epilepsies in children. In: Andermann F, Beaumanoir A, Mira L, Roger J, Tassinari CA, editors. Occipital seizures and epilepsies in children. London: John Libbey and Company Ltd; 1993. p. 71–86.

    Google Scholar 

  38. Vigevano F, Lispi ML, Ricci S. Early onset benign occipital susceptibility syndrome: video-EEG documentation of an illustrative case. Clin Neurophysiol. 2000;111(Suppl 2):S81–6.

    PubMed  Google Scholar 

  39. Specchio N, Trivisano M, Claps D, Battaglia D, Fusco L, Vigevano F. Documentation of autonomic seizures and autonomic status epilepticus with ictal EEG in Panayiotopoulos syndrome. Epilepsy Behav. 2010;19:383–93.

    PubMed  Google Scholar 

  40. Inoue Y, Fujiwara T, Matsuda K, Kubota H, Tanaka M, Yagi K, et al. Ring chromosome 20 and nonconvulsive status epilepticus. A new epileptic syndrome. Brain. 1997;120:939–53.

    PubMed  Google Scholar 

  41. Galván-Manso M, Campistol J, Conill J, Sanmartí FX. Analysis of the characteristics of epilepsy in 37 patients with the molecular diagnosis of Angelman syndrome. Epileptic Disord. 2005;7:19–25.

    PubMed  Google Scholar 

  42. Pelc K, Boyd SG, Cheron G, Dan B. Epilepsy in Angelman syndrome. Seizure. 2008;17:211–7.

    PubMed  Google Scholar 

  43. Weber P. Levetiracetam in nonconvulsive status epilepticus in a child with angelman syndrome. J Child Neurol. 2010;25:393–6.

    PubMed  Google Scholar 

  44. Amir RE, Van den Veyver IB, Wan M, Tran CQ, Francke U, Zoghbi HY. Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2. Nat Genet. 1999;23:185–8.

    CAS  PubMed  Google Scholar 

  45. Nissenkorn A, Gak E, Vecsler M, Reznik H, Menascu S, Ben Zeev B. Epilepsy in Rett syndrome – the experience of a National Rett Center. Epilepsia. 2010;51:1252–8.

    PubMed  Google Scholar 

  46. Trivisano M, Specchio N, Cappelletti S, Di Ciommo V, Claps D, Specchio LM, et al. Myoclonic astatic epilepsy: an age-dependent epileptic syndrome with favorable seizure outcome but variable cognitive evolution. Epilepsy Res. 2011;97:133–41.

    PubMed  Google Scholar 

  47. Kelley SA, Kossoff EH. Doose syndrome (myoclonic-astatic epilepsy): 40 years of progress. Dev Med Child Neurol. 2010;52:988–93.

    PubMed  Google Scholar 

  48. Guerrini R, Aicardi J. Epileptic encephalopathies with myoclonic seizures in infants and children (severe myoclonic epilepsy and myoclonic-astatic epilepsy). J Clin Neurophysiol. 2003;20:449–61.

    PubMed  Google Scholar 

  49. Kobayashi K, Inoue T, Kikumoto K, Endoh F, Miya K, Oka M, et al. Relation of spasms and myoclonus to suppression-burst on EEG in epileptic encephalopathy in early infancy. Neuropediatrics. 2007;38:244–50.

    CAS  PubMed  Google Scholar 

  50. Dalla Bernardina B, Fontana E, Darra F. Myoclonic epilepsies, advances in neurology series. In: Delgado-Escueta AV, Guerrini R, Medina MT, Genton P, Bureau MDC, editors. Myoclonic epilepsies, advances in neurology series. Philadelphia: Lippincott Williams & Wilkins; 2004. p. 153–6.

    Google Scholar 

  51. Caraballo RH, Cersósimo RO, Espeche A, Arroyo HA, Fejerman N. Myoclonic status in nonprogressive encephalopathies: study of 29 cases. Epilepsia. 2007;48:107–13.

    PubMed  Google Scholar 

  52. Dalla Bernardina B, Fontana E, Darra F. Myoclonic status in non progressive encephalopathies. In: Bureau M, Genton P, Dravet C, Delgado-Escueta A, Tassinari CA, Thomas P, Wolf P, editors. Epileptic syndromes in infancy, childhood and adolescence. 5th ed. Montrouge: John Libbey Eurotext Ltd; 2012. p. 431–9.

    Google Scholar 

  53. Wiltshire E, Davidzon G, Dimauro S, Akman HO, Sadleir L, Haas L, et al. Juvenile Alpers disease. Arch Neurol. 2008;65:121–4.

    PubMed  Google Scholar 

  54. Bicknese AR, May W, Hickey WF, Dodson WE. Early childhood hepatocerebral degeneration misdiagnosed as valproate hepatotoxicity. Ann Neurol. 1992;32:767–75.

    CAS  PubMed  Google Scholar 

  55. Uusimaa J, Finnilä S, Vainionpää L, Kärppä M, Herva R, Rantala H, et al. A mutation in mitochondrial DNA-encoded cytochrome c oxidase II gene in a child with Alpers-Huttenlocher-like disease. Pediatrics. 2003;111:e262–8.

    PubMed  Google Scholar 

  56. Wolf NI, Rahman S, Schmitt B, Taanman JW, Duncan AJ, Harting I, et al. Status epilepticus in children with Alpers’ disease caused by POLG1 mutations: EEG and MRI features. Epilepsia. 2009;50:1596–607.

    PubMed  Google Scholar 

  57. Gastaut H, Roger J, Soulayrol R, Tassinari CA, Régis H, Dravet C, et al. Childhood epileptic encephalopathy with diffuse slow spike-waves (otherwise known as “petit mal variant”) or Lennox syndrome. Epilepsia. 1966;7:139–79.

    Google Scholar 

  58. Arzimanoglou A, Guerrini R, Aicardi J. Lennox-Gastaut syndrome. In: Aicardi’s epilepsy in children. Philadelphia: Lippincott Williams & Wilkins; 2004. p. 38–50.

    Google Scholar 

  59. Hoffmann-Riem M, Diener W, Benninger C, Rating D, Unnebrink K, Stephani U, et al. Nonconvulsive status epilepticus--a possible cause of mental retardation in patients with Lennox-Gastaut syndrome. Neuropediatrics. 2000;31:169–74.

    CAS  PubMed  Google Scholar 

  60. Livingston JH, Brown JK. Non-convulsive status epilepticus resistant to benzodiazepines. Arch Dis Child. 1987;62:41–4.

    CAS  PubMed  PubMed Central  Google Scholar 

  61. Ohtsuka Y, Sato M, Oka E. Nonconvulsive status epilepticus in childhood localization-related epilepsy. Epilepsia. 1999;40:1003–10.

    CAS  PubMed  Google Scholar 

  62. Bauer G, Trinka E. Nonconvulsive status epilepticus and coma. Epilepsia. 2010;51:177–90.

    PubMed  Google Scholar 

  63. Tassinari CA, Dravet C, Roger J, Cano JP, Gastaut H. Tonic status epilepticus precipitated by intravenous benzodiazepine in five patients with Lennox–Gastaut syndrome. Epilepsia. 1972;13:421–35.

    CAS  PubMed  Google Scholar 

  64. Patry G, Lyagoubi S, Tassinari CA. Subclinical “electrical status epilepticus” induced by sleep in children: a clinical and electroencephalographic study of six cases. Arch Neurol. 1971;24:242–52.

    CAS  PubMed  Google Scholar 

  65. McVicar KA, Ballaban-Gil K, Rapin I, Moshé SL, Shinnar S. Epileptiform EEG abnormalities in children with language regression. Neurology. 2005;65:129–31.

    CAS  PubMed  Google Scholar 

  66. Nickels K, Wirrell E. Electrical status epilepticus in sleep. Semin Pediatr Neurol. 2008;15:50–60.

    PubMed  Google Scholar 

  67. Rudolf G, Valenti MP, Hirsch E, Szepetowski P. From rolandic epilepsy to continuous spike-and-waves during sleep and Landau-Kleffner syndromes: insights into possible genetic factors. Epilepsia. 2009;50(Suppl 7):25–8.

    CAS  PubMed  Google Scholar 

  68. Tsai MH, Vears DF, Turner SJ, Smith RL, Berkovic SF, Sadleir LG, et al. Clinical genetic study of the epilepsy-aphasia spectrum. Epilepsia. 2013;54:280–7.

    PubMed  Google Scholar 

  69. Kessi M, Peng J, Yang L, Xiong J, Duan H, Pang N, et al. Genetic etiologies of the electrical status epilepticus during slow wave sleep: systematic review. BMC Genet. 2018;19:40.

    PubMed  PubMed Central  Google Scholar 

  70. Zanni G, Barresi S, Cohen R, Specchio N, Basel-Vanagaite L, Valente EM, et al. A novel mutation in the endosomal Na+/H+ exchanger NHE6 (SLC9A6) causes Christianson syndrome with electrical status epilepticus during slow-wave sleep (ESES). Epilepsy Res. 2014;108:811–5.

    CAS  PubMed  Google Scholar 

  71. Pacheva I, Panov G, Gillberg C, Neville B. A girl with tuberous sclerosis complex presenting with severe epilepsy and electrical status epilepticus during sleep, and with high-functioning autism and mutism. Cogn Behav Neurol. 2014;27:88–95.

    PubMed  Google Scholar 

  72. Carmi N, Lev D, Leshinsky-Silver E, Anikster Y, Blumkin L, Kivity S, et al. Atypical presentation of Costeff syndrome-severe psychomotor involvement and electrical status epilepticus during slow wave sleep. Eur J Paediatr Neurol. 2015;19:733–6.

    PubMed  Google Scholar 

  73. Lesca G, Rudolf G, Labalme A, Hirsch E, Arzimanoglou A, Genton P, et al. Epileptic encephalopathies of the Landau-Kleffner and continuous spike and waves during slow-wave sleep types: genomic dissection makes the link with autism. Epilepsia. 2012;53:1526–38.

    CAS  PubMed  Google Scholar 

  74. Nabbout R. Autoimmune and inflammatory epilepsies. Epilepsia. 2012;53:58–62.

    CAS  PubMed  Google Scholar 

  75. Minassian BA, Striano P, Avanzini G. Progressive myoclonus epilepsies: state-of-the-art. Epileptic Disord. 2016;18(Suppl 2):S1–158.

    Google Scholar 

  76. Miyahara A, Saito Y, Sugai K, Nakagawa E, Sakuma H, Komaki H, et al. Reassessment of phenytoin for treatment of late stage progressive myoclonus epilepsy complicated with status epilepticus. Epilepsy Res. 2009;84:801–9.

    Google Scholar 

  77. Fanella M, Egeo G, Fattouch J, Casciato S, Lapenta L, Morano A, et al. Oxcarbazepine-induced myoclonic status epilepticus in juvenile myoclonic epilepsy. Epileptic Disord. 2013;15:181–7.

    PubMed  Google Scholar 

  78. Gélisse P, Crespel A. Mixed myoclonic-absence status epilepticus in juvenile myoclonic epilepsy. Epileptic Disord. 2015;17:95–6.

    PubMed  Google Scholar 

  79. Knake S, Rosenow F, Vescovi M, Oertel WH, Mueller HH, Wirbatz A, et al. Incidence of status epilepticus in adults in Germany: a prospective, population-based study. Epilepsia. 2001;42:714–8.

    CAS  PubMed  Google Scholar 

  80. Cascino GD. Nonconvulsive status epilepticus in adults and children. Epilepsia. 1993;34(Suppl 1):S21–8.

    PubMed  Google Scholar 

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  1. Rare and Complex Epilepsy Unit, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children’s Hospital, Rome, Italy

    Nicola Specchio & Nicola Pietrafusa

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  1. Nicola Specchio
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  1. Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy

    Oriano Mecarelli

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Specchio, N., Pietrafusa, N. (2019). Paediatric Status Epilepticus. In: Mecarelli, O. (eds) Clinical Electroencephalography. Springer, Cham. https://doi.org/10.1007/978-3-030-04573-9_31

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