The Clinical manifestations, electroencephalogram features and surgical treatment and of epilepsy secondary to Sturge-Weber syndrome in children_Journal of Epilepsy

Authors：

YU Guojing ¹ ,  JI Taoyun ^1,2 , CHE Yuanyuan ¹ , WANG Wen ¹ , LIU Tong ¹ , WANG Hongjie ¹ , WANG Ruofan ^1,2 , WANG Shuang ^1,2 , LIU Qingzhu ¹ , CAI Lixin ¹ , LIU Xiaoyan ^1,2

1. Department of Pediatrics, Peking University First Hospital, Beijing 100034, China;
2. Pediatric Epilepsy Center, Peking University First Hospital, Beijing 100034, China;

Corresponding?author：

JI Taoyun, Email: jitaoyun@163.com

Keywords：

Sturge-Weber syndrome; Refractory epilepsy; EEG; Surgical treatment

DOI：

10.7507/2096-0247.20220011

Video：

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Objective To investigate the clinicalmanifestations, electroencephalogram (EEG) characteristics, surgical treatment and prognosis of epilepsy secondary to Sturge-Weber syndrome (SWS) in children.Methods The data of 7 children with epilepsysecondaryto Sturge-Weber syndrome who were treated by surgery from May 2015 to May 2020 in our Children's Epilepsy Center were retrospectively reviewed. Their demographic characteristics, seizure forms, results of EEG and cranial imaging investigations, surgical methods, postoperative pathological reports and prognosis during follow-upwere summarized and analyzed. The prognosis were evaluated byEngel classificationat the last time point during follow-up. Results Totally 7 pediatric patients were enrolled, including 1 male (16/25, 64.0%) and 6 females.All the 7 cases presented with focal seizures at the onset among whom 2 cases developed status epilepticus during the course of the disease(epilepsiapartialiscontinuain 1 case),one case had epileptic spasmsand 1 case was characterized by cluster seizures. The interictal EEG manifestations of the cases gradually deteriorated as the course of the disease prolonged, including the slow wave on the affected side gradually increases (7/7), the amplitude gradually decreases (7/7), and the physiological wave disappears (4/7). Besides, no epileptiform discharges/incidental or a few epileptiform dischargeswere found in their interictal EEGs. Four cases underwent hemispherotomy, 1 case underwent temporo-parieto-occipital disconnection and 2 cases underwent lesion resection. The cases were followed up for 6 months to 5 years, and the average follow-up duration was 79.29 months. Six cases were rated as Engel Ⅰa during the regularfollow-up. Only 1 case was rated asEngel Ⅱ and Engel Ⅲ at 3 month and 1 year after the operation. Conclusion Children with epilepsy secondary to SWS usually present with focal seizures and have diverse seizure forms. The EEG show characteristic changes. For the caseswith drug refractory epilepsy, detailed preoperative evaluation and reasonable surgical methods can result in a better therapeutic effect.

Citation： YU Guojing, JI Taoyun, CHE Yuanyuan, WANG Wen, LIU Tong, WANG Hongjie, WANG Ruofan, WANG Shuang, LIU Qingzhu, CAI Lixin, LIU Xiaoyan. The Clinical manifestations, electroencephalogram features and surgical treatment and of epilepsy secondary to Sturge-Weber syndrome in children. Journal of Epilepsy, 2022, 8(1): 41-47. doi: 10.7507/2096-0247.20220011 Copy

1.	Kaseka ML, Bitton JY, Décarie JC, et al. Predictive factors for epilepsy in pediatric patients with Sturge-Weber syndrome. Pediatr Neurol, 2016, 64: 52-58.
2.	Marana Pérez AI, Ruiz-FalcóRojas ML, Puertas Martín V, et al. Analysis of Sturge-Weber syndrome: a retrospective study of multiple associated variables. Neurologia( Barcelona, Spain), 2017, 32(6): 363-370.
3.	Sudarsanam, Annapurna, Ardern-Holmes, Simone L. Sturge–Weber syndrome: From the past to the present. Eur J PaediatrNeurol, 2014, 18(3): 257-266.
4.	Shirley M D, Tang H, Gallione C J, et al. Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N. Engl J Med, 2013, 368(21): 1971-1979.
5.	Polubothu S, A-Olabil, Carmen Del Boente M, et al. GNA11 mutation as a cause of Sturge-Weber syndrome: expansion of the phenotypic spectrum of G-protein related mosaicism and the associated clinical diagnoses. J Invest Dermatol, 2020, 140(5): 1110-1113.
6.	Sujansky E, Conradi S. Outcome of Sturgee Webersyndrome in 52 adults. Am J Med Genet, 1995, 57: 35-45.
7.	Jagtap S, Srinivas G, Harsha KJ, et al. SturgeeWeber syndrome: clinicalspectrum, disease course, and outcome of 30 patients. J ChildNeurol, 2013, 28(6): 725-731.
8.	Pascual-Castroviejo I, Pascual-Pascual SI, Velazquez-Fragua R, et al. SturgeeWeber syndrome: study of 55 patients. Can J NeurolSci, 2008, 35: 301-307.
9.	Anne M. Comi. Chapter 11 - Sturge–Weber syndrome[M]// Handbook of Clinical Neurology. Elsevier Health Sciences, 2015.
10.	Comi, Anne M. Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge-Weber syndrome. The Neurologist, 2011, 17(4): 179-184.
11.	Weber, F. P . Right-sided hemi-hypotrophy resulting from right-sided congenital spastic hemiplegia, with a morbid condition of the left side of the brain, revealed by radiograms. Journal of Neurology Neurosurgery & Psychiatry, 1922, 16: 102-108.
12.	Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: A review. Pediatric neurology, 2004, 30(5): 303.
13.	許新科, 謝艷平, 李軍亮, 等. 兒童Sturge-Weber綜合征繼發癲癇的外科治療. 中華小兒外科雜志, 2020, 41(2): 140-144.
14.	李沐陽, 張華強, 安陽, 等. Sturge-Weber綜合征繼發藥物難治性癲癇的手術治療及文獻回顧. 中國腦血管病雜志, 2020, 17(1): 44-48.
15.	BAFLA, CsabaJuhász, CJAL, et al. Neurological complications of Sturge-Weber syndrome: Current status and unmet needs. Pediatric Neurology, 2019, 98: 31-38.
16.	Eric H. Kossoff, Lisa Ferenc, Anne M. Comi. An infantile-onset, severe, yet sporadic seizure pattern is common in Sturge-Weber syndrome. Epilepsia, 2009, 53(5): 8-906-912.
17.	Brenner RP, Sharbrough FW. Electroencephalographic evaluationin Sturge Weber syndrome. Neurology, 1976, 26: 629-632.
18.	劉曉燕. 臨床腦電圖學. 2006.
19.	Fukuyama Y, Tsuchiya S. A study on Sturge-Webersyndrome. Report of a case associated with infantile spasms and electroencephalographic evolution in five cases. EurNeurol, 1979, 18: 194-204.
20.	Reith W, Yilmaz U, Zimmer A. Sturge-Weber-Syndrom. Der Radiologe, 2013, 53(12): 1099-1103.
21.	Michaela Z, Mackay M T, Leventer R J, et al. Retrospective review of screening for Sturge‐Weber syndrome with brain magnetic resonance imaging and electroencephalography in infants with high‐risk port‐wine stains. Pediatric Dermatology, 2018, 16(2): 206-212.
22.	張玉珍, 尹秋鳳, 蔡靜, 等. 兒童Sturge-Weber綜合征影像學診斷. 中國臨床醫學影像雜志, 2020, 313): 154-158.
23.	劉長青, 陳思暢, 關宇光, 等. Sturge-Weber綜合征所致難治性癲癇的手術治療. 中國臨床神經外科雜志, 2017, (6): 379-381.
24.	Bianchi F, Auricchio AM, Battaglia DI, et al. Sturge-Weber syndrome: an update on the relevant issues for neurosurgeons. Child s Nervous System, 2020, (7): 256-261.
25.	Kossoff EH, Buck C, Freeman JM. Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide. Neurology, 2002, 59(11): 1735.
26.	Arzimanoglou AA, Andermann F, Aicardi J, et al. Sturge-Weber syndrome: indications and results of surgery in 20 patients. Neurology, 2000, 55(10): 1472-1479.

1. Kaseka ML, Bitton JY, Décarie JC, et al. Predictive factors for epilepsy in pediatric patients with Sturge-Weber syndrome. Pediatr Neurol, 2016, 64: 52-58.
2. Marana Pérez AI, Ruiz-FalcóRojas ML, Puertas Martín V, et al. Analysis of Sturge-Weber syndrome: a retrospective study of multiple associated variables. Neurologia( Barcelona, Spain), 2017, 32(6): 363-370.
3. Sudarsanam, Annapurna, Ardern-Holmes, Simone L. Sturge–Weber syndrome: From the past to the present. Eur J PaediatrNeurol, 2014, 18(3): 257-266.
4. Shirley M D, Tang H, Gallione C J, et al. Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N. Engl J Med, 2013, 368(21): 1971-1979.
5. Polubothu S, A-Olabil, Carmen Del Boente M, et al. GNA11 mutation as a cause of Sturge-Weber syndrome: expansion of the phenotypic spectrum of G-protein related mosaicism and the associated clinical diagnoses. J Invest Dermatol, 2020, 140(5): 1110-1113.
6. Sujansky E, Conradi S. Outcome of Sturgee Webersyndrome in 52 adults. Am J Med Genet, 1995, 57: 35-45.
7. Jagtap S, Srinivas G, Harsha KJ, et al. SturgeeWeber syndrome: clinicalspectrum, disease course, and outcome of 30 patients. J ChildNeurol, 2013, 28(6): 725-731.
8. Pascual-Castroviejo I, Pascual-Pascual SI, Velazquez-Fragua R, et al. SturgeeWeber syndrome: study of 55 patients. Can J NeurolSci, 2008, 35: 301-307.
9. Anne M. Comi. Chapter 11 - Sturge–Weber syndrome[M]// Handbook of Clinical Neurology. Elsevier Health Sciences, 2015.
10. Comi, Anne M. Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge-Weber syndrome. The Neurologist, 2011, 17(4): 179-184.
11. Weber, F. P . Right-sided hemi-hypotrophy resulting from right-sided congenital spastic hemiplegia, with a morbid condition of the left side of the brain, revealed by radiograms. Journal of Neurology Neurosurgery & Psychiatry, 1922, 16: 102-108.
12. Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: A review. Pediatric neurology, 2004, 30(5): 303.
13. 許新科, 謝艷平, 李軍亮, 等. 兒童Sturge-Weber綜合征繼發癲癇的外科治療. 中華小兒外科雜志, 2020, 41(2): 140-144.
14. 李沐陽, 張華強, 安陽, 等. Sturge-Weber綜合征繼發藥物難治性癲癇的手術治療及文獻回顧. 中國腦血管病雜志, 2020, 17(1): 44-48.
15. BAFLA, CsabaJuhász, CJAL, et al. Neurological complications of Sturge-Weber syndrome: Current status and unmet needs. Pediatric Neurology, 2019, 98: 31-38.
16. Eric H. Kossoff, Lisa Ferenc, Anne M. Comi. An infantile-onset, severe, yet sporadic seizure pattern is common in Sturge-Weber syndrome. Epilepsia, 2009, 53(5): 8-906-912.
17. Brenner RP, Sharbrough FW. Electroencephalographic evaluationin Sturge Weber syndrome. Neurology, 1976, 26: 629-632.
18. 劉曉燕. 臨床腦電圖學. 2006.
19. Fukuyama Y, Tsuchiya S. A study on Sturge-Webersyndrome. Report of a case associated with infantile spasms and electroencephalographic evolution in five cases. EurNeurol, 1979, 18: 194-204.
20. Reith W, Yilmaz U, Zimmer A. Sturge-Weber-Syndrom. Der Radiologe, 2013, 53(12): 1099-1103.
21. Michaela Z, Mackay M T, Leventer R J, et al. Retrospective review of screening for Sturge‐Weber syndrome with brain magnetic resonance imaging and electroencephalography in infants with high‐risk port‐wine stains. Pediatric Dermatology, 2018, 16(2): 206-212.
22. 張玉珍, 尹秋鳳, 蔡靜, 等. 兒童Sturge-Weber綜合征影像學診斷. 中國臨床醫學影像雜志, 2020, 313): 154-158.
23. 劉長青, 陳思暢, 關宇光, 等. Sturge-Weber綜合征所致難治性癲癇的手術治療. 中國臨床神經外科雜志, 2017, (6): 379-381.
24. Bianchi F, Auricchio AM, Battaglia DI, et al. Sturge-Weber syndrome: an update on the relevant issues for neurosurgeons. Child s Nervous System, 2020, (7): 256-261.
25. Kossoff EH, Buck C, Freeman JM. Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide. Neurology, 2002, 59(11): 1735.
26. Arzimanoglou AA, Andermann F, Aicardi J, et al. Sturge-Weber syndrome: indications and results of surgery in 20 patients. Neurology, 2000, 55(10): 1472-1479.

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