癫痫性脑病：对一个重要临床概念的使用和滥用_Journal of Epilepsy

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HowellKB , HarveyAS , ArcherJS , 张乐 , 慕洁

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Keywords：

癫痫性脑病; Lennox-Gastaut综合征

DOI：

10.7507/2096-0247.20170088

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癫痫性脑病(Epileptic encephalopathy, EE)是指癫痫活动对脑功能的不良影响超过其潜在的病因。对其潜在的机制知之甚少，但最近的研究表明癫痫发作和发作间期癫痫样放电可以破坏暂时和永久支撑认知功能的神经网络分布。EE只是癫痫中影响发育的多种因素之一。EE所致的认知损害通常难以与潜在的病因或是抗癫痫药物的不良反应区分。这种困难导致越来越多地使用EE来概括“严重的”癫痫综合征或是与严重的癫痫以及智力障碍相关的病因，而不管癫痫影响认知功能的证据。文献中不但使用EE这个术语来描述癫痫活动导致认知损害的过程，也将其归为一类严重的癫痫综合征，这导致了混淆。提议EE这一术语应该严格用于普遍的癫痫过程影响发育这个中心概念，而应该避免使用EE作为分类方式。需要另一个术语来概括广泛且异质的有严重癫痫和智力障碍的患者，其机制可能是未知的，但是通常与潜在的遗传性、代谢性或结构性病因密切相关。提高对EE致病机制的认识至关重要，可能有利于明确生物标志物用于早期诊断和治疗。

Citation： HowellKB, HarveyAS, ArcherJS, 张乐, 慕洁. 癫痫性脑病：对一个重要临床概念的使用和滥用. Journal of Epilepsy, 2017, 3(6): 542-545. doi: 10.7507/2096-0247.20170088 Copy

1.	Berg AT, Berkovic SF, Brodie MJ, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia, 2010, 51(4): 676-685.
2.	Engel J Jr. a proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology. Epilepsia, 2001, 42(6): 796-803.
3.	Engel J Jr. Report of the ILAE classification core group. Epilepsia, 2006, 47(9): 1558-1568.
4.	Berg AT, Zelko FA, Levy SR, et al. Age at onset of epilepsy, pharmacoresistance, and cognitive outcomes: a prospective cohort study. Neurology, 2012, 79(13): 1384-1391.
5.	Sanchez FI, Loddenkemper T, Galanopoulou AS, et al. Should epileptiform discharges be treated. Epilepsia, 2015, 56(10): 1492-1504.
6.	Chapman KE, Specchio N, Shinnar S, et al. Seizing control of epileptic activity can improve outcome. Epilepsia, 2015, 56(10): 1482-1485.
7.	Jehi L, Wyllie E, Devinsky O. Epileptic encephalopathies: optimizing seizure control and developmental outcome. Epilepsia, 2015, 56(10): 1486-1489.
8.	Devlin AM, Cross JH, Harkness W, et al. Clinical outcomes of hemispherectomy for epilepsy in childhood and adolescence. Brain, 2003, 126(3): 556-566.
9.	Lettori D, Battaglia D, Sacco A, et al. Early hemispherectomy in catastrophic epilepsy: a neuro-cognitive and epileptic long-term follow-up. Seizure, 2008, 17(1): 49-63.
10.	Korff CM, Brunklaus A, Zuberi SM. Epileptic activity is a surrogate for an underlying etiology and stopping the activity has a limited impact on developmental outcome. Epilepsia, 2015, 56(10): 1477-1481.
11.	Brunklaus A, Ellis R, Reavey E, et al. Prognostic, clinical and demographic features in SCN1A mutation-positive dravet syndrome. Brain, 2012, 135(Pt 8): 2329-2336.
12.	Bureau M, Dalla Bernardina B. Electroencephalographic characteristics of dravet syndrome. Epilepsia, 2011, 52(Suppl 2): 13-23.
13.	Brunklaus A, Zuberi SM. Dravet syndrome-from epileptic encephalopathy to channelopathy. Epilepsia, 2014, 55(7): 979-984.
14.	Catarino CB, Liu JY, Liagkouras I, et al. Dravet syndrome as epileptic encephalopathy: evidence from long-term course and neuropathology. Brain, 2011, 134(Pt 10): 2982-3010.
15.	Matsumoto A, Watanabe K, Negoro T, et al. Long-term prognosis after infantile spasms: a statistical study of prognostic factors in 200 cases. Dev Med Child Neurol, 1981, 23(1): 51-65.
16.	Kivity S, Lerman P, Ariel R, et al. Long-term cognitive outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone. Epilepsia, 2004, 45(3): 255-262.
17.	O'Callaghan FJ, Lux AL, Darke K, et al. The effect of lead time to treatment and of age of onset on developmental outcome at 4 years in infantile spasms: evidence from the United Kingdom Infantile Spasms Study. Epilepsia, 2011, 52(7): 1359-1364.
18.	Berg AT, Loddenkemper T, Baca CB. Diagnostic delays in children with early onset epilepsy: impact, reasons, and opportunities to improve care. Epilepsia, 2014, 55(1): 123-132.
19.	Eisermann MM, De LaRaillere A, Dellatolas G, et al. Infantile spasms in down syndrome-effects of delayed anticonvulsive treatment. Epilepsy Res, 2003, 55(1-2): 21-27.
20.	Humphrey A, MacLean C, Ploubidis GB, et al. Intellectual development before and after the onset of infantile spasms: a controlled prospective longitudinal study in tuberous sclerosis. Epilepsia, 2014, 55(1): 108-116.
21.	Jozwiak S, Kotulska K, Domanska-Pakiela D, et al. Antiepileptic treatment before the onset of seizures reduces epilepsy severity and risk of mental retardation in infants with tuberous sclerosis complex. Eur J Paediatr Neurol, 2011, 15(5): 424-431.
22.	Berg AT, Smith SN, Frobish D, et al. Longitudinal assessment of adaptive behavior in infants and young children with newly diagnosed epilepsy: influences of etiology, syndrome, and seizure control. Pediatrics, 2004, 114(3): 645-650.
23.	Jonas R, Nguyen S, Hu B, et al. Cerebral hemispherectomy: hospital course, seizure, developmental, language, and motor outcomes. Neurology, 2004, 62(10): 1712-1721.
24.	Delalande O, Bulteau C, Dellatolas G, et al. Vertical parasagittal hemispherotomy: surgical procedures and clinical long-term outcomes in a population of 83 children. Neurosurgery, 2007, 60(2 Suppl 1): ONS19-32; discussion ONS32.
25.	Freeman J, Harvey A, Rosenfeld J, et al. Generalized epilepsy in hypothalamic hamartoma evolution and postoperative resolution. Neurology, 2003, 60(5): 762-767.
26.	Wethe JV, Prigatano GP, Gray J, et al. Cognitive functioning before and after surgical resection for hypothalamic hamartoma and epilepsy. Neurology, 2013, 81(12): 1044-1050.
27.	Loddenkemper T, Holland KD, Stanford LD, et al. Developmental outcome after epilepsy surgery in infancy. Pediatrics, 2007, 119(5): 930-935.
28.	Kleen JK, Scott RC, Lenck-Santini PP, et al. Cognitive and Behavioral Co-Morbidities of Epilepsy. In: Noebels JL, Avoli M, Rogawski MA, et al. (Eds) Jasper's Basic Mechanisms of the Epilepsies. 4th ed. Bethesda, MD: National Center for Biotechnology Information, 2012.
29.	Holmes GL. Cognitive impairment in epilepsy: the role of network abnormalities. Epileptic Disord, 2015, 17(2): 101-116.
30.	Archer JS, Warren AE, Stagnitti MR, et al. Lennox-Gastaut syndrome and phenotype: secondary network epilepsies. Epilepsia, 2014, 55(8): 1245-1254.
31.	Pillay N, Archer JS, Badawy RA, et al. Networks underlying paroxysmal fast activity and slow spike and wave in Lennox-Gastaut syndrome. Neurology, 2013, 81(7): 665-673.
32.	Archer JS, Warren AE, Jackson GD, et al. Conceptualizing Lennox-Gastaut syndrome as a secondary network epilepsy. Front Neurol, 2014, 5: 225.
33.	Ibrahim GM, Morgan BR, Lee W, et al. Impaired development of intrinsic connectivity networks in children with medically intractable localization-related epilepsy. Hum Brain Mapp, 2014, 35(11): 5686-5700.
34.	Pedersen M, Curwood EK, Archer JS, et al. Brain regions with abnormal network properties in severe epilepsy of Lennox-Gastaut phenotype: multivariate analysis of task-free fMRI. Epilepsia, 2015, 56(11): 1767-1773.
35.	Reid CA, Leaw B, Richards KL, et al. Reduced dendritic arborization and hyperexcitability of pyramidal neurons in a scn1b-based model of dravet syndrome. Brain, 2014, 137(Pt 6): 1701-1715.
36.	Pearson TS, Akman C, Hinton VJ, et al. Phenotypic spectrum of glucose transporter type 1 deficiency syndrome (Glut1 DS). Curr Neurol Neurosci Rep, 2013, 13(4): 342.
37.	Alter AS, Engelstad K, Hinton VJ, et al. Long-term clinical course of glut1 deficiency syndrome. J Child Neurol, 2015, 30(2): 160-169.
38.	Engel J Jr. International classification: implications for neocortical epilepsies. Adv Neurol, 2000, 84: 119-124.

1. Berg AT, Berkovic SF, Brodie MJ, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia, 2010, 51(4): 676-685.
2. Engel J Jr. a proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology. Epilepsia, 2001, 42(6): 796-803.
3. Engel J Jr. Report of the ILAE classification core group. Epilepsia, 2006, 47(9): 1558-1568.
4. Berg AT, Zelko FA, Levy SR, et al. Age at onset of epilepsy, pharmacoresistance, and cognitive outcomes: a prospective cohort study. Neurology, 2012, 79(13): 1384-1391.
5. Sanchez FI, Loddenkemper T, Galanopoulou AS, et al. Should epileptiform discharges be treated. Epilepsia, 2015, 56(10): 1492-1504.
6. Chapman KE, Specchio N, Shinnar S, et al. Seizing control of epileptic activity can improve outcome. Epilepsia, 2015, 56(10): 1482-1485.
7. Jehi L, Wyllie E, Devinsky O. Epileptic encephalopathies: optimizing seizure control and developmental outcome. Epilepsia, 2015, 56(10): 1486-1489.
8. Devlin AM, Cross JH, Harkness W, et al. Clinical outcomes of hemispherectomy for epilepsy in childhood and adolescence. Brain, 2003, 126(3): 556-566.
9. Lettori D, Battaglia D, Sacco A, et al. Early hemispherectomy in catastrophic epilepsy: a neuro-cognitive and epileptic long-term follow-up. Seizure, 2008, 17(1): 49-63.
10. Korff CM, Brunklaus A, Zuberi SM. Epileptic activity is a surrogate for an underlying etiology and stopping the activity has a limited impact on developmental outcome. Epilepsia, 2015, 56(10): 1477-1481.
11. Brunklaus A, Ellis R, Reavey E, et al. Prognostic, clinical and demographic features in SCN1A mutation-positive dravet syndrome. Brain, 2012, 135(Pt 8): 2329-2336.
12. Bureau M, Dalla Bernardina B. Electroencephalographic characteristics of dravet syndrome. Epilepsia, 2011, 52(Suppl 2): 13-23.
13. Brunklaus A, Zuberi SM. Dravet syndrome-from epileptic encephalopathy to channelopathy. Epilepsia, 2014, 55(7): 979-984.
14. Catarino CB, Liu JY, Liagkouras I, et al. Dravet syndrome as epileptic encephalopathy: evidence from long-term course and neuropathology. Brain, 2011, 134(Pt 10): 2982-3010.
15. Matsumoto A, Watanabe K, Negoro T, et al. Long-term prognosis after infantile spasms: a statistical study of prognostic factors in 200 cases. Dev Med Child Neurol, 1981, 23(1): 51-65.
16. Kivity S, Lerman P, Ariel R, et al. Long-term cognitive outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone. Epilepsia, 2004, 45(3): 255-262.
17. O'Callaghan FJ, Lux AL, Darke K, et al. The effect of lead time to treatment and of age of onset on developmental outcome at 4 years in infantile spasms: evidence from the United Kingdom Infantile Spasms Study. Epilepsia, 2011, 52(7): 1359-1364.
18. Berg AT, Loddenkemper T, Baca CB. Diagnostic delays in children with early onset epilepsy: impact, reasons, and opportunities to improve care. Epilepsia, 2014, 55(1): 123-132.
19. Eisermann MM, De LaRaillere A, Dellatolas G, et al. Infantile spasms in down syndrome-effects of delayed anticonvulsive treatment. Epilepsy Res, 2003, 55(1-2): 21-27.
20. Humphrey A, MacLean C, Ploubidis GB, et al. Intellectual development before and after the onset of infantile spasms: a controlled prospective longitudinal study in tuberous sclerosis. Epilepsia, 2014, 55(1): 108-116.
21. Jozwiak S, Kotulska K, Domanska-Pakiela D, et al. Antiepileptic treatment before the onset of seizures reduces epilepsy severity and risk of mental retardation in infants with tuberous sclerosis complex. Eur J Paediatr Neurol, 2011, 15(5): 424-431.
22. Berg AT, Smith SN, Frobish D, et al. Longitudinal assessment of adaptive behavior in infants and young children with newly diagnosed epilepsy: influences of etiology, syndrome, and seizure control. Pediatrics, 2004, 114(3): 645-650.
23. Jonas R, Nguyen S, Hu B, et al. Cerebral hemispherectomy: hospital course, seizure, developmental, language, and motor outcomes. Neurology, 2004, 62(10): 1712-1721.
24. Delalande O, Bulteau C, Dellatolas G, et al. Vertical parasagittal hemispherotomy: surgical procedures and clinical long-term outcomes in a population of 83 children. Neurosurgery, 2007, 60(2 Suppl 1): ONS19-32; discussion ONS32.
25. Freeman J, Harvey A, Rosenfeld J, et al. Generalized epilepsy in hypothalamic hamartoma evolution and postoperative resolution. Neurology, 2003, 60(5): 762-767.
26. Wethe JV, Prigatano GP, Gray J, et al. Cognitive functioning before and after surgical resection for hypothalamic hamartoma and epilepsy. Neurology, 2013, 81(12): 1044-1050.
27. Loddenkemper T, Holland KD, Stanford LD, et al. Developmental outcome after epilepsy surgery in infancy. Pediatrics, 2007, 119(5): 930-935.
28. Kleen JK, Scott RC, Lenck-Santini PP, et al. Cognitive and Behavioral Co-Morbidities of Epilepsy. In: Noebels JL, Avoli M, Rogawski MA, et al. (Eds) Jasper's Basic Mechanisms of the Epilepsies. 4th ed. Bethesda, MD: National Center for Biotechnology Information, 2012.
29. Holmes GL. Cognitive impairment in epilepsy: the role of network abnormalities. Epileptic Disord, 2015, 17(2): 101-116.
30. Archer JS, Warren AE, Stagnitti MR, et al. Lennox-Gastaut syndrome and phenotype: secondary network epilepsies. Epilepsia, 2014, 55(8): 1245-1254.
31. Pillay N, Archer JS, Badawy RA, et al. Networks underlying paroxysmal fast activity and slow spike and wave in Lennox-Gastaut syndrome. Neurology, 2013, 81(7): 665-673.
32. Archer JS, Warren AE, Jackson GD, et al. Conceptualizing Lennox-Gastaut syndrome as a secondary network epilepsy. Front Neurol, 2014, 5: 225.
33. Ibrahim GM, Morgan BR, Lee W, et al. Impaired development of intrinsic connectivity networks in children with medically intractable localization-related epilepsy. Hum Brain Mapp, 2014, 35(11): 5686-5700.
34. Pedersen M, Curwood EK, Archer JS, et al. Brain regions with abnormal network properties in severe epilepsy of Lennox-Gastaut phenotype: multivariate analysis of task-free fMRI. Epilepsia, 2015, 56(11): 1767-1773.
35. Reid CA, Leaw B, Richards KL, et al. Reduced dendritic arborization and hyperexcitability of pyramidal neurons in a scn1b-based model of dravet syndrome. Brain, 2014, 137(Pt 6): 1701-1715.
36. Pearson TS, Akman C, Hinton VJ, et al. Phenotypic spectrum of glucose transporter type 1 deficiency syndrome (Glut1 DS). Curr Neurol Neurosci Rep, 2013, 13(4): 342.
37. Alter AS, Engelstad K, Hinton VJ, et al. Long-term clinical course of glut1 deficiency syndrome. J Child Neurol, 2015, 30(2): 160-169.
38. Engel J Jr. International classification: implications for neocortical epilepsies. Adv Neurol, 2000, 84: 119-124.

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