Ketogenic diet in the treatment of 5 cases of febrile infection-related epilepsy syndrome and literature review_Journal of Epilepsy

Authors：

DENG Yao , YU Tao , CHEN Xiaolu ,  LUO Rong

Department of Pediatric Neurology, West China Second University Hospital, Key Laboratory of Birth Defects and Related Disease of Women and Children (Sichuan University), Ministry of Education, Sichuan University, Chengdu 610041, China;

Corresponding?author：

LUO Rong, Email: 1109329811@qq.com

Keywords：

Febrile infection-related epilepsy syndrome; Refractory epilepsy; Ketogenic diet; Children

DOI：

10.7507/2096-0247.20210045

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Objective According to the diagnosis and treatment analysis of 5 children with Febrile infection-related epilepsy syndrome (FIRES) and literature review, to explore the therapeutic effect of Ketogenic diet (KD).Methods A retrospective analysis of the clinical status of 5 children with KD treatment of FIRES admitted to the Department of Pediatric Neurology, West China Second Hospital of Sichuan University from August 2016 to September 2019, combined with literature data, summarized their disease characteristics, prognosis and KD treatment effects.Results The 5 cases of FIRES children were (5.8±2.0) years old and had a male to female ratio of 2∶3. They were all induced by fever followed by a status epilepticus that was difficult to control with drugs. The interval between fever and first seizure was 4 to 7 days, and the prodromal symptoms were higher respiratory tract infections, dizziness, vomiting, fatigue, listlessness, loss of appetite, etc., convulsions manifested as focal or focal secondary systemic or general seizures, EEG showed slowing background rhythm, changes in multifocal epileptic discharge, early course of disease The cranial imaging examination was basically normal. As the course of the disease progressed, changes in brain atrophy gradually appeared, and abnormal signal shadows were seen in the forehead, parietal, occipital lobe, and periventricular. A variety of anti-epileptic drugs, hormones, gamma globulin, plasma exchange and other treatments have poor therapeutic effects and severe cognitive impairment. The KD treatment started to take effect within 2 weeks, and the convulsions were reduced. One case was completely controlled, and the cognitive function basically returned to normal with only mild learning disabilities; the convulsions were reduced by more than 50% in 2 cases, leaving mild to moderate cognitive impairment, The other 2 cases had poor long-term treatment effects, left intractable seizures and severe mental retardation.Conclusion FIRES is a serious epileptic encephalopathy, most of which leave severe cognitive impairment and refractory seizures. Drug therapy and prognosis are poor. KD treatment is beneficial to control seizures in children with FIRES in the acute stage.

Citation： DENG Yao, YU Tao, CHEN Xiaolu, LUO Rong. Ketogenic diet in the treatment of 5 cases of febrile infection-related epilepsy syndrome and literature review. Journal of Epilepsy, 2021, 7(4): 281-287. doi: 10.7507/2096-0247.20210045 Copy

1.	Van B, Hausler M, Plecko-startinig B, et al. Febrile infection-related epilepsy syndrome without detectable auto-antibodies and response to immunotherapy: a case series and discussion of epileptogenesis in FIRES. Neuropediatrics, 2012, 43(4): 209-216.
2.	Kramer U, Chi C S, Lin K L, et al. Febrile infection-related epilepsy syndrome(FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children. Epilepsia, 2011, 52(11): 1956-1965.
3.	Nabbout R, Mazzuca M, Hubert P, et al. Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children(FIRES). Epilepsia, 2010, 51(10): 2033-2037.
4.	兒童發熱誘導的學齡期兒童難治性癲癲性腦病協作組. 發熱誘導的學齡期兒童難治性癲癇性腦病的臨床特點及預后分析. 中華兒科雜志, 2012, 50(8): 575-579.
5.	吳春風, 廖建湘, 鄭幗, 等. 生酮飲食治療發熱感染相關性癲癇綜合征1例. 中國實用兒科雜志, 2013, 28(5): 393-394.
6.	Roberto H, Caraballo, Gabriela Reyes, et al. Febrile infection-related epilepsy syndrome: A study of 12 patients. Seizure, 2013, 22(7): 553-559.
7.	Rani K. S, Sucheta M. J, Denise M. P, et al Cognitive Outcomes in Febrile Infection-Related Epilepsy Syndrome Treated With the Ketogenic Diet. Pediatrics, 2014, 134(5): 1431-1436.
8.	陳小璐, 康曉東, 蔡淺云, 等. 生酮飲食治療熱性感染相關性癲癇綜合征 1 例并文獻復習. 中華實用兒科臨床雜志, 2018, 33(15): 1182-1184.
9.	楊偉明, 孫丹. 熱性感染相關性癲癇綜合征 1 例病例報道并文獻復習. 江漢大學學報(自然科學版), 2019, 47(5): 459-463.
10.	Sio-Kuan Lam, Wen-Yu Lu, Wen-Chin Weng, et al. The short-term and long-term outcome of febrile infection-related epilepsy syndrome in children. Epilepsy& Behavior, 2019, 95: 117-123.
11.	王國麗, 鄧小鹿, 彭鏡, 等. 發熱感染相關性癲癇綜合征 12 例診治分析及治療進展. 中國醫師雜志, 2019, 21(9): 1297-1301.
12.	Specchio N, Pietrafusa N. New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome. Dev Med Child Neurol, 2020, 62(8): 897-905.
13.	Mikaeloff Y, Jambaque I, Hertz-Pannier L, et al. Devastating epileptic encephalopathy in school-aged children (DESC): a pseudo encephalitis. Epilepsy, 2006, 69: 67-79.
14.	Wheless JW. Treatment of refractory convulsive status epilepticus In children: other therapies. Semin PediatrNeurol, 2010, 17: 190-194.
15.	Van Baalen A, Vezzani A, Hausler M, et al. Febrile infection-related epilepsy syndrome: clinical review and hypotheses of epileptogenesis. Neuropediatrics, 2017, 48: 5-18.
16.	Sakuma H, Tanuma N, Kuki I, et al. Intrathecal overproduction of proinflammatory cytokines and chemokines in febrile infection-related refractory status epilepticus. J Neurol Neurosurg Psychiatry, 2015, 86: 820-822.
17.	Clarkson BDS, LaFrance-Corey RG, Kahoud RJ, et al. Functional deficiency in endogenous interleukin-1 receptor antagonist in patients with febrile infection-related epilepsy syndrome. Ann Neurol, 2019, 85: 526-37.
18.	Sakuma H, Awaya Y, Shiomi M, et al. Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. Acta Neurol Scand, 2010, 121: 251-256.
19.	Appavu B, Vanatta L, Condie J, et al. Ketogenic diet treatment for pediatric super-refractory status epilepticus. Seizure, 2016, 41: 62-65.
20.	Nicolas G, Lawrence J H, Claudine S, et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. Epilepsia, 2018, 599(3): 745-752.
21.	Andreas V, Annamaria V, Martin H, et al. Febrile Infection-Related Epilepsy Syndrome: Clinical Review and Hypotheses of Epileptogenesis. Neuropediatrics, 2017, 48(1): 5-18.
22.	Hsiu-Fen Lee, Ching-Shiang Chi. Febrile infection-related epilepsy syndrome (FIRES): therapeutic complications, long-term neurological and neuroimaging follow-up. Seizure, 2018, 56: 53-59.

1. Van B, Hausler M, Plecko-startinig B, et al. Febrile infection-related epilepsy syndrome without detectable auto-antibodies and response to immunotherapy: a case series and discussion of epileptogenesis in FIRES. Neuropediatrics, 2012, 43(4): 209-216.
2. Kramer U, Chi C S, Lin K L, et al. Febrile infection-related epilepsy syndrome(FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children. Epilepsia, 2011, 52(11): 1956-1965.
3. Nabbout R, Mazzuca M, Hubert P, et al. Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children(FIRES). Epilepsia, 2010, 51(10): 2033-2037.
4. 兒童發熱誘導的學齡期兒童難治性癲癲性腦病協作組. 發熱誘導的學齡期兒童難治性癲癇性腦病的臨床特點及預后分析. 中華兒科雜志, 2012, 50(8): 575-579.
5. 吳春風, 廖建湘, 鄭幗, 等. 生酮飲食治療發熱感染相關性癲癇綜合征1例. 中國實用兒科雜志, 2013, 28(5): 393-394.
6. Roberto H, Caraballo, Gabriela Reyes, et al. Febrile infection-related epilepsy syndrome: A study of 12 patients. Seizure, 2013, 22(7): 553-559.
7. Rani K. S, Sucheta M. J, Denise M. P, et al Cognitive Outcomes in Febrile Infection-Related Epilepsy Syndrome Treated With the Ketogenic Diet. Pediatrics, 2014, 134(5): 1431-1436.
8. 陳小璐, 康曉東, 蔡淺云, 等. 生酮飲食治療熱性感染相關性癲癇綜合征 1 例并文獻復習. 中華實用兒科臨床雜志, 2018, 33(15): 1182-1184.
9. 楊偉明, 孫丹. 熱性感染相關性癲癇綜合征 1 例病例報道并文獻復習. 江漢大學學報(自然科學版), 2019, 47(5): 459-463.
10. Sio-Kuan Lam, Wen-Yu Lu, Wen-Chin Weng, et al. The short-term and long-term outcome of febrile infection-related epilepsy syndrome in children. Epilepsy& Behavior, 2019, 95: 117-123.
11. 王國麗, 鄧小鹿, 彭鏡, 等. 發熱感染相關性癲癇綜合征 12 例診治分析及治療進展. 中國醫師雜志, 2019, 21(9): 1297-1301.
12. Specchio N, Pietrafusa N. New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome. Dev Med Child Neurol, 2020, 62(8): 897-905.
13. Mikaeloff Y, Jambaque I, Hertz-Pannier L, et al. Devastating epileptic encephalopathy in school-aged children (DESC): a pseudo encephalitis. Epilepsy, 2006, 69: 67-79.
14. Wheless JW. Treatment of refractory convulsive status epilepticus In children: other therapies. Semin PediatrNeurol, 2010, 17: 190-194.
15. Van Baalen A, Vezzani A, Hausler M, et al. Febrile infection-related epilepsy syndrome: clinical review and hypotheses of epileptogenesis. Neuropediatrics, 2017, 48: 5-18.
16. Sakuma H, Tanuma N, Kuki I, et al. Intrathecal overproduction of proinflammatory cytokines and chemokines in febrile infection-related refractory status epilepticus. J Neurol Neurosurg Psychiatry, 2015, 86: 820-822.
17. Clarkson BDS, LaFrance-Corey RG, Kahoud RJ, et al. Functional deficiency in endogenous interleukin-1 receptor antagonist in patients with febrile infection-related epilepsy syndrome. Ann Neurol, 2019, 85: 526-37.
18. Sakuma H, Awaya Y, Shiomi M, et al. Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. Acta Neurol Scand, 2010, 121: 251-256.
19. Appavu B, Vanatta L, Condie J, et al. Ketogenic diet treatment for pediatric super-refractory status epilepticus. Seizure, 2016, 41: 62-65.
20. Nicolas G, Lawrence J H, Claudine S, et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. Epilepsia, 2018, 599(3): 745-752.
21. Andreas V, Annamaria V, Martin H, et al. Febrile Infection-Related Epilepsy Syndrome: Clinical Review and Hypotheses of Epileptogenesis. Neuropediatrics, 2017, 48(1): 5-18.
22. Hsiu-Fen Lee, Ching-Shiang Chi. Febrile infection-related epilepsy syndrome (FIRES): therapeutic complications, long-term neurological and neuroimaging follow-up. Seizure, 2018, 56: 53-59.

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