癲癇性腦病：對一個重要臨床概念的使用和濫用_Journal of Epilepsy

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HowellKB , HarveyAS , ArcherJS , 張樂 , 慕潔

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Keywords：

癲癇性腦病; Lennox-Gastaut綜合征

DOI：

10.7507/2096-0247.20170088

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癲癇性腦病(Epileptic encephalopathy, EE)是指癲癇活動對腦功能的不良影響超過其潛在的病因。對其潛在的機制知之甚少，但最近的研究表明癲癇發作和發作間期癲癇樣放電可以破壞暫時和永久支撐認知功能的神經網絡分布。EE只是癲癇中影響發育的多種因素之一。EE所致的認知損害通常難以與潛在的病因或是抗癲癇藥物的不良反應區分。這種困難導致越來越多地使用EE來概括“嚴重的”癲癇綜合征或是與嚴重的癲癇以及智力障礙相關的病因，而不管癲癇影響認知功能的證據。文獻中不但使用EE這個術語來描述癲癇活動導致認知損害的過程，也將其歸為一類嚴重的癲癇綜合征，這導致了混淆。提議EE這一術語應該嚴格用于普遍的癲癇過程影響發育這個中心概念，而應該避免使用EE作為分類方式。需要另一個術語來概括廣泛且異質的有嚴重癲癇和智力障礙的患者，其機制可能是未知的，但是通常與潛在的遺傳性、代謝性或結構性病因密切相關。提高對EE致病機制的認識至關重要，可能有利于明確生物標志物用于早期診斷和治療。

Citation： HowellKB, HarveyAS, ArcherJS, 張樂, 慕潔. 癲癇性腦病：對一個重要臨床概念的使用和濫用. 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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