Lennox Gastaut综合征和表型——继发性脑网络癫痫_Journal of Epilepsy

Authors：

 ArcherJS , WarrenAE , StagnittiMR , MastertonRA , AbbottDF , JacksonGD , 迟潇洒 , 慕洁

Corresponding author：

ArcherJS, Email: jarcher@unimelb.edu.au

Keywords：

Lennox-Gastaut综合征; 继发性全面性癫痫; 脑电图-功能磁共振; 手术; 病灶

DOI：

10.7507/2096-0247.20160048

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Lennox-Gastaut综合征(Lennox-Gastautsyndrome, LGS)是一种严重的多病因且具有特征性电生理特征的癫痫表型。前驱研究发现常见的脑网络与特征性的发作间期放电:慢棘慢复合波(Slow spike-and-wave, SSW)和阵发性快活动(Paroxysmal fast activity, PFA)。一些患者有Lennox-Gastaut样表型和皮质病灶。现旨在探讨脑网络和病灶的相互作用。选取6例具有Lennox-Gastaut表型和结构病灶的患者为研究对象, 采用3T脑电图-功能磁共振(Electroencephalography-functional magnetic resonance imaging, EEG-fMRI)进行检测。SSW和PFA时间监测用于事件相关fMRI分析, 以分析关键区域的血流动力学时间进程。结果:①PFA发生时, 额叶和顶叶的联合皮层区、丘脑和脑桥的fMRI信号增强, 且注意和静息态脑网络均同时增强, 这是一个少见的模式; ②SSW发生时, 表现为混合性的fMRI信号增强和减弱, 联合皮质区和丘脑在放电发生前信号增强, 而在放电后显著减弱, 在初级皮层区fMRI信号减弱; ③在PFA和SSW放电期间, 病灶区表现为不同的fMRI信号增强。3例患者在病灶切除术后1年无痫性发作。研究认为Lennox-Gastaut表型是一种网络癫痫, 且主要的脑网络自发不稳。Lennox-Gastaut表型和LGS的癫痫样活动, 似乎通过联合皮质区放大和表达, 这可能是由于基本的脑网络, 注意和静息态网络广泛地相互作用。接受病灶切除术后癫痫患者无癫痫发作表明皮质损伤能建立并保持这种非正常的不稳定脑网络。LGS可能是继发性脑网络癫痫, 因为其统一的癫痫临床表现, 包括PFA和SSW, 反映出脑网络功能异常而非特定的触发过程

Citation： ArcherJS, WarrenAE, StagnittiMR, MastertonRA, AbbottDF, JacksonGD, 迟潇洒, 慕洁. Lennox Gastaut综合征和表型——继发性脑网络癫痫. Journal of Epilepsy, 2016, 2(3): 255-263. doi: 10.7507/2096-0247.20160048 Copy

1.	Gastaut H, Roger J, Soulayrol R, et al. Childhood epilepticencephalopathy with diffuse slow spike-waves (otherwise knownas "petit mal variant") or Lennox syndrome. Epilepsia, 1966, 7(2):139-179.
2.	Gastaut H, Gastaut JL, Goncalves e Silva GE, et al. Relative frequencyof different types of epilepsy:a study employing the classification of theInternational League Against Epilepsy. Epilepsia, 1975, 16(8):457-461.
3.	Crumrine PK. Lennox-Gastaut syndrome. J Child Neurol, 2002, 17(Suppl.1):70-75.
4.	Arzimanoglou A, French J, Blume WT, et al. Lennox-Gastautsyndrome:a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol, 2009, 8(2):82-93.
5.	Blume WT. Lennox-Gastaut syndrome:potential mechanisms ofcognitive regression. Ment Retard Dev Disabil Res Rev, 2004, 10(1):150-153.
6.	Li LM, Fish DR, Sisodiya SM, et al. High resolution magneticresonance imaging in adults with partial or secondary generalizedepilepsy attending a tertiary referral unit. J Neurol NeurosurgPsychiatry, 1995, 59(11):384-387.
7.	Goldsmith IL, Zupanc ML, Buchhalter JR. Long-term seizure outcomein 74 patients with Lennox-Gastaut syndrome:effects of incorporatingMRI head imaging in defining the cryptogenic subgroup. Epilepsia, 2000, 41(3):395-399.
8.	Bladin P. Adult Lennox Gastaut syndrome:patients with large focalstructural lesions. Clin Exp Neurol, 1985, 21(7):105-114.
9.	You S, Lee J, Ko T. Epilepsy surgery in a patient with Lennox-Gastautsyndrome and cortical dysplasia. Brain Dev, 2007, 29(5):167-170.
10.	Wyllie E, Lachhwani DK, Gupta A, et al. Successful surgery forepilepsy due to early brain lesions despite generalized EEG findings.Neurology, 2007, 69(12):389-397.
11.	Salek-Haddadi A, Diehl B, Hamandi K, et al. Hemodynamiccorrelates of epileptiform discharges:an EEG-fMRI study of 63patients with focal epilepsy. Brain Res, 2006, 1088(112):148-166.
12.	Gotman J, Pittau F. Combining EEG and fMRI in the study of epilepticdischarges. Epilepsia, 2011, 52(Suppl.4):38-42.
13.	Hamandi K, Salek-Haddadi A, Laufs H, et al. EEG-fMRI of idiopathicand secondarily generalized epilepsies. Neuroimage, 2006, 31(9):1700-1710.
14.	Siniatchkin M, Coropceanu D, Moeller F, et al. EEG-fMRI revealsactivation of brainstem and thalamus in patients with Lennox-Gastautsyndrome. Epilepsia, 2011, 52(10):766-774.
15.	Pillay N, Archer JS, Badawy RA, et al. Networks underlyingparoxysmal fast activity and slow spike and wave in Lennox-Gastautsyndrome. Neurology, 2013, 81(9):665-673.
16.	Carney PW, Masterton RA, Harvey AS, et al. The core network inabsence epilepsy. Differences in cortical and thalamic BOLDresponse. Neurology, 2010, 75(8):904-911.
17.	Masterton RA, Abbott DF, Fleming SW, et al. Measurement andreduction of motion and ballistocardiogram artefacts fromsimultaneous EEG and fMRI recordings. Neuroimage, 2007, 37(11):202-211.
18.	Flanagan D, Abbott DF, Jackson GD. How wrong can we be? Theeffect of inaccurate mark-up of EEG/fMRI studies in epilepsy. ClinNeurophysiol, 2009, 120(8):1637-1647.
19.	Abbott D, Jackson G. iBrain-software for analysis and visualizationof functional MRimages. Neuroimage, 2001, 13(Suppl 2):59.
20.	Abbott D, Masterton R, Waites A, et al. The iBrain(TM) analysistoolbox for SPM. The 17th Annual Meeting of the Organisationfor Human Brain Mapping. 364 WTh, Quebec City, Canada, 2011.
21.	Masterton RA, Harvey AS, Archer JS, et al. Focal epileptiform spikesdo not show a canonical BOLD response in patients with benignrolandic epilepsy (BECTS). Neuroimage, 2010, 51(8):252-260.
22.	Fox MD, Snyder AZ, Vincent JL, et al. The human brain isintrinsically organized into dynamic, anticorrelated functionalnetworks. Proc Natl Acad Sci USA, 2005, 102(7):9673-9678.
23.	Intusoma U, Abbott DF, Masterton RA, et al. Tonic seizures ofLennox-Gastaut syndrome:periictal single-photon emission computedtomography suggests a corticopontine network. Epilepsia, 2013, 54(12):2151-2157.
24.	Blumenfeld H, Westerveld M, Ostroff RB, et al. Selective frontal, parietal, and temporal networks in generalized seizures. Neuroimage, 2003, 19(7):1556-1566.
25.	Enev M, McNally KA, Varghese G, et al. Imaging onsetand propagation of ECT-induced seizures. Epilepsia, 2007, 48(10):238-244.
26.	Bai X, Vestal M, Berman R, et al. Dynamic time course of typicalchildhood absence seizures:EEG, behavior, and functional magneticresonance imaging. J Neurosci, 2010, 30(11):5884-5893.
27.	Moeller F, Siebner HR, Wolff S, et al. Changes in activity of striatothalamo-cortical network precede generalized spike wave discharges.Neuroimage, 2008, 39(12):1839-1849.
28.	Carney PW, Masterton RA, Flanagan D, et al. The frontal lobe inabsence epilepsy:EEG-fMRI findings. Neurology, 2012, 78(2):1157-1165.
29.	Velasco M, Velasco F, Alcala H, et al. Epileptiform EEG activity ofthe centromedian thalamic nuclei in children with intractablegeneralized seizures of the Lennox-Gastaut syndrome. Epilepsia, 1991, 32(4):310-321.
30.	Velasco M, Velasco F, Velasco AL. Temporo-spacial correlationsbetween cortical and subcortical EEG spike-wave complexes of theIdiopathic Lennox-Gastaut syndrome. Stereotact Funct Neurosurg, 1997, 69(10):216-220.
31.	Logothetis NK, Wandell BA. Interpreting the BOLD signal. Annu RevPhysiol, 2004, 66(8):735-769.

1. Gastaut H, Roger J, Soulayrol R, et al. Childhood epilepticencephalopathy with diffuse slow spike-waves (otherwise knownas "petit mal variant") or Lennox syndrome. Epilepsia, 1966, 7(2):139-179.
2. Gastaut H, Gastaut JL, Goncalves e Silva GE, et al. Relative frequencyof different types of epilepsy:a study employing the classification of theInternational League Against Epilepsy. Epilepsia, 1975, 16(8):457-461.
3. Crumrine PK. Lennox-Gastaut syndrome. J Child Neurol, 2002, 17(Suppl.1):70-75.
4. Arzimanoglou A, French J, Blume WT, et al. Lennox-Gastautsyndrome:a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol, 2009, 8(2):82-93.
5. Blume WT. Lennox-Gastaut syndrome:potential mechanisms ofcognitive regression. Ment Retard Dev Disabil Res Rev, 2004, 10(1):150-153.
6. Li LM, Fish DR, Sisodiya SM, et al. High resolution magneticresonance imaging in adults with partial or secondary generalizedepilepsy attending a tertiary referral unit. J Neurol NeurosurgPsychiatry, 1995, 59(11):384-387.
7. Goldsmith IL, Zupanc ML, Buchhalter JR. Long-term seizure outcomein 74 patients with Lennox-Gastaut syndrome:effects of incorporatingMRI head imaging in defining the cryptogenic subgroup. Epilepsia, 2000, 41(3):395-399.
8. Bladin P. Adult Lennox Gastaut syndrome:patients with large focalstructural lesions. Clin Exp Neurol, 1985, 21(7):105-114.
9. You S, Lee J, Ko T. Epilepsy surgery in a patient with Lennox-Gastautsyndrome and cortical dysplasia. Brain Dev, 2007, 29(5):167-170.
10. Wyllie E, Lachhwani DK, Gupta A, et al. Successful surgery forepilepsy due to early brain lesions despite generalized EEG findings.Neurology, 2007, 69(12):389-397.
11. Salek-Haddadi A, Diehl B, Hamandi K, et al. Hemodynamiccorrelates of epileptiform discharges:an EEG-fMRI study of 63patients with focal epilepsy. Brain Res, 2006, 1088(112):148-166.
12. Gotman J, Pittau F. Combining EEG and fMRI in the study of epilepticdischarges. Epilepsia, 2011, 52(Suppl.4):38-42.
13. Hamandi K, Salek-Haddadi A, Laufs H, et al. EEG-fMRI of idiopathicand secondarily generalized epilepsies. Neuroimage, 2006, 31(9):1700-1710.
14. Siniatchkin M, Coropceanu D, Moeller F, et al. EEG-fMRI revealsactivation of brainstem and thalamus in patients with Lennox-Gastautsyndrome. Epilepsia, 2011, 52(10):766-774.
15. Pillay N, Archer JS, Badawy RA, et al. Networks underlyingparoxysmal fast activity and slow spike and wave in Lennox-Gastautsyndrome. Neurology, 2013, 81(9):665-673.
16. Carney PW, Masterton RA, Harvey AS, et al. The core network inabsence epilepsy. Differences in cortical and thalamic BOLDresponse. Neurology, 2010, 75(8):904-911.
17. Masterton RA, Abbott DF, Fleming SW, et al. Measurement andreduction of motion and ballistocardiogram artefacts fromsimultaneous EEG and fMRI recordings. Neuroimage, 2007, 37(11):202-211.
18. Flanagan D, Abbott DF, Jackson GD. How wrong can we be? Theeffect of inaccurate mark-up of EEG/fMRI studies in epilepsy. ClinNeurophysiol, 2009, 120(8):1637-1647.
19. Abbott D, Jackson G. iBrain-software for analysis and visualizationof functional MRimages. Neuroimage, 2001, 13(Suppl 2):59.
20. Abbott D, Masterton R, Waites A, et al. The iBrain(TM) analysistoolbox for SPM. The 17th Annual Meeting of the Organisationfor Human Brain Mapping. 364 WTh, Quebec City, Canada, 2011.
21. Masterton RA, Harvey AS, Archer JS, et al. Focal epileptiform spikesdo not show a canonical BOLD response in patients with benignrolandic epilepsy (BECTS). Neuroimage, 2010, 51(8):252-260.
22. Fox MD, Snyder AZ, Vincent JL, et al. The human brain isintrinsically organized into dynamic, anticorrelated functionalnetworks. Proc Natl Acad Sci USA, 2005, 102(7):9673-9678.
23. Intusoma U, Abbott DF, Masterton RA, et al. Tonic seizures ofLennox-Gastaut syndrome:periictal single-photon emission computedtomography suggests a corticopontine network. Epilepsia, 2013, 54(12):2151-2157.
24. Blumenfeld H, Westerveld M, Ostroff RB, et al. Selective frontal, parietal, and temporal networks in generalized seizures. Neuroimage, 2003, 19(7):1556-1566.
25. Enev M, McNally KA, Varghese G, et al. Imaging onsetand propagation of ECT-induced seizures. Epilepsia, 2007, 48(10):238-244.
26. Bai X, Vestal M, Berman R, et al. Dynamic time course of typicalchildhood absence seizures:EEG, behavior, and functional magneticresonance imaging. J Neurosci, 2010, 30(11):5884-5893.
27. Moeller F, Siebner HR, Wolff S, et al. Changes in activity of striatothalamo-cortical network precede generalized spike wave discharges.Neuroimage, 2008, 39(12):1839-1849.
28. Carney PW, Masterton RA, Flanagan D, et al. The frontal lobe inabsence epilepsy:EEG-fMRI findings. Neurology, 2012, 78(2):1157-1165.
29. Velasco M, Velasco F, Alcala H, et al. Epileptiform EEG activity ofthe centromedian thalamic nuclei in children with intractablegeneralized seizures of the Lennox-Gastaut syndrome. Epilepsia, 1991, 32(4):310-321.
30. Velasco M, Velasco F, Velasco AL. Temporo-spacial correlationsbetween cortical and subcortical EEG spike-wave complexes of theIdiopathic Lennox-Gastaut syndrome. Stereotact Funct Neurosurg, 1997, 69(10):216-220.
31. Logothetis NK, Wandell BA. Interpreting the BOLD signal. Annu RevPhysiol, 2004, 66(8):735-769.

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