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Benign Neonatal Epilepsy

MedGen UID:
82856
•Concept ID:
C0270851
•
Disease or Syndrome
Synonyms: Benign Neonatal Convulsion; Benign Neonatal Convulsions; Benign Neonatal Epilepsies; Benign Neonatal-Infantile Epilepsies; Benign Neonatal-Infantile Epilepsy; Convulsion, Benign Neonatal; Convulsions, Benign Neonatal; Epilepsies, Benign Neonatal; Epilepsies, Benign Neonatal-Infantile; Epilepsy, Benign Neonatal; Epilepsy, Benign Neonatal Infantile; Epilepsy, Benign Neonatal-Infantile; Neonatal Convulsion, Benign; Neonatal Convulsions, Benign; Neonatal Epilepsies, Benign; Neonatal Epilepsy, Benign; Neonatal-Infantile Epilepsies, Benign; Neonatal-Infantile Epilepsy, Benign
SNOMED CT: Benign neonatal seizures (38281008); Benign neonatal convulsions (38281008)

Definition

An inherited or sporadic disorder characterized by epileptic seizures in the first four to six weeks of life. The seizures tend to subside after the fifteenth week of life. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Benign Neonatal Epilepsy

Professional guidelines

PubMed

Evaluation of the feasibility, diagnostic yield, and clinical utility of rapid genome sequencing in infantile epilepsy (Gene-STEPS): an international, multicentre, pilot cohort study.
D'Gama AM, Mulhern S, Sheidley BR, Boodhoo F, Buts S, Chandler NJ, Cobb J, Curtis M, Higginbotham EJ, Holland J, Khan T, Koh J, Liang NSY, McRae L, Nesbitt SE, Oby BT, Paternoster B, Patton A, Rose G, Scotchman E, Valentine R, Wiltrout KN; Gene-STEPS Study Group; IPCHiP Executive Committee, Hayeems RZ, Jain P, Lunke S, Marshall CR, Rockowitz S, Sebire NJ, Stark Z, White SM, Chitty LS, Cross JH, Scheffer IE, Chau V, Costain G, Poduri A, Howell KB, McTague A
Lancet Neurol 2023 Sep;22(9):812-825. doi: 10.1016/S1474-4422(23)00246-6. PMID: 37596007
Epilepsy Syndromes in the First Year of Life and Usefulness of Genetic Testing for Precision Therapy.
Bayat A, Bayat M, Rubboli G, Møller RS
Genes (Basel) 2021 Jul 8;12(7) doi: 10.3390/genes12071051. PMID: 34356067Free PMC Article
Phenotypic spectrum and genetics of SCN2A-related disorders, treatment options, and outcomes in epilepsy and beyond.
Wolff M, Brunklaus A, Zuberi SM
Epilepsia 2019 Dec;60 Suppl 3:S59-S67. doi: 10.1111/epi.14935. PMID: 31904126

Recent clinical studies

Etiology

Early initial video-electro-encephalography combined with variant location predict prognosis of KCNQ2-related disorder.
Xu Y, Dou YL, Chen X, Dong XR, Wang XH, Wu BB, Cheng GQ, Zhou YF
BMC Pediatr 2021 Oct 28;21(1):477. doi: 10.1186/s12887-021-02946-z. PMID: 34711204Free PMC Article
The ILAE classification of seizures and the epilepsies: Modification for seizures in the neonate. Position paper by the ILAE Task Force on Neonatal Seizures.
Pressler RM, Cilio MR, Mizrahi EM, Moshé SL, Nunes ML, Plouin P, Vanhatalo S, Yozawitz E, de Vries LS, Puthenveettil Vinayan K, Triki CC, Wilmshurst JM, Yamamoto H, Zuberi SM
Epilepsia 2021 Mar;62(3):615-628. Epub 2021 Feb 1 doi: 10.1111/epi.16815. PMID: 33522601
Levetiracetam Versus Phenobarbital for Neonatal Seizures: A Randomized Controlled Trial.
Sharpe C, Reiner GE, Davis SL, Nespeca M, Gold JJ, Rasmussen M, Kuperman R, Harbert MJ, Michelson D, Joe P, Wang S, Rismanchi N, Le NM, Mower A, Kim J, Battin MR, Lane B, Honold J, Knodel E, Arnell K, Bridge R, Lee L, Ernstrom K, Raman R, Haas RH; NEOLEV2 INVESTIGATORS
Pediatrics 2020 Jun;145(6) Epub 2020 May 8 doi: 10.1542/peds.2019-3182. PMID: 32385134Free PMC Article
Serum Phenobarbitone Levels in Term and Near Term Neonates with Seizures.
Wasim S, Upadhyay A, Roy M, Saxena P, Chillar N
Indian Pediatr 2016 May 8;53(5):388-90. doi: 10.1007/s13312-016-0858-4. PMID: 27254045
Neonatal seizures: gaps between the laboratory and the clinic.
Lombroso CT
Epilepsia 2007;48 Suppl 2:83-106. doi: 10.1111/j.1528-1167.2007.01070.x. PMID: 17571356

Diagnosis

The ILAE classification of seizures and the epilepsies: Modification for seizures in the neonate. Position paper by the ILAE Task Force on Neonatal Seizures.
Pressler RM, Cilio MR, Mizrahi EM, Moshé SL, Nunes ML, Plouin P, Vanhatalo S, Yozawitz E, de Vries LS, Puthenveettil Vinayan K, Triki CC, Wilmshurst JM, Yamamoto H, Zuberi SM
Epilepsia 2021 Mar;62(3):615-628. Epub 2021 Feb 1 doi: 10.1111/epi.16815. PMID: 33522601
Levetiracetam Versus Phenobarbital for Neonatal Seizures: A Randomized Controlled Trial.
Sharpe C, Reiner GE, Davis SL, Nespeca M, Gold JJ, Rasmussen M, Kuperman R, Harbert MJ, Michelson D, Joe P, Wang S, Rismanchi N, Le NM, Mower A, Kim J, Battin MR, Lane B, Honold J, Knodel E, Arnell K, Bridge R, Lee L, Ernstrom K, Raman R, Haas RH; NEOLEV2 INVESTIGATORS
Pediatrics 2020 Jun;145(6) Epub 2020 May 8 doi: 10.1542/peds.2019-3182. PMID: 32385134Free PMC Article
Monogenic idiopathic epilepsies.
Gourfinkel-An I, Baulac S, Nabbout R, Ruberg M, Baulac M, Brice A, LeGuern E
Lancet Neurol 2004 Apr;3(4):209-18. doi: 10.1016/S1474-4422(04)00706-9. PMID: 15039033
Ion channel variation causes epilepsies.
Moulard B, Picard F, le Hellard S, Agulhon C, Weiland S, Favre I, Bertrand S, Malafosse A, Bertrand D
Brain Res Brain Res Rev 2001 Oct;36(2-3):275-84. doi: 10.1016/s0165-0173(01)00104-7. PMID: 11690625
Inherited epilepsies.
Walsh LE, McCandless D
Semin Pediatr Neurol 2001 Sep;8(3):165-76. doi: 10.1053/spen.2001.26450. PMID: 11575846

Therapy

Levetiracetam Versus Phenobarbital for Neonatal Seizures: A Randomized Controlled Trial.
Sharpe C, Reiner GE, Davis SL, Nespeca M, Gold JJ, Rasmussen M, Kuperman R, Harbert MJ, Michelson D, Joe P, Wang S, Rismanchi N, Le NM, Mower A, Kim J, Battin MR, Lane B, Honold J, Knodel E, Arnell K, Bridge R, Lee L, Ernstrom K, Raman R, Haas RH; NEOLEV2 INVESTIGATORS
Pediatrics 2020 Jun;145(6) Epub 2020 May 8 doi: 10.1542/peds.2019-3182. PMID: 32385134Free PMC Article
Molecular pharmacology and therapeutic potential of neuronal Kv7-modulating drugs.
Miceli F, Soldovieri MV, Martire M, Taglialatela M
Curr Opin Pharmacol 2008 Feb;8(1):65-74. Epub 2007 Dec 3 doi: 10.1016/j.coph.2007.10.003. PMID: 18061539
Monogenic idiopathic epilepsies.
Gourfinkel-An I, Baulac S, Nabbout R, Ruberg M, Baulac M, Brice A, LeGuern E
Lancet Neurol 2004 Apr;3(4):209-18. doi: 10.1016/S1474-4422(04)00706-9. PMID: 15039033
Neonatal epileptic encephalopathy.
Clayton PT, Surtees RA, DeVile C, Hyland K, Heales SJ
Lancet 2003 May 10;361(9369):1614. doi: 10.1016/s0140-6736(03)13312-0. PMID: 12747882
The clinical conundrum of neonatal seizures.
Levene M
Arch Dis Child Fetal Neonatal Ed 2002 Mar;86(2):F75-7. doi: 10.1136/fn.86.2.f75. PMID: 11882546Free PMC Article

Prognosis

Heart rate variability in neonatal patients with seizures.
Statello R, Carnevali L, Alinovi D, Pisani F, Sgoifo A
Clin Neurophysiol 2018 Dec;129(12):2534-2540. Epub 2018 Oct 25 doi: 10.1016/j.clinph.2018.10.001. PMID: 30384023
Benign infantile seizures.
Mishra D, Nikunj NK, Juneja M, Talukdar B
Indian Pediatr 2015 Feb;52(2):151-2. doi: 10.1007/s13312-015-0592-3. PMID: 25691188
Seizures in the newborn.
Sankar MJ, Agarwal R, Aggarwal R, Deorari AK, Paul VK
Indian J Pediatr 2008 Feb;75(2):149-55. doi: 10.1007/s12098-008-0023-8. PMID: 18334796
Inherited epilepsies.
Walsh LE, McCandless D
Semin Pediatr Neurol 2001 Sep;8(3):165-76. doi: 10.1053/spen.2001.26450. PMID: 11575846
Migrating partial seizures in infancy: two new cases.
Wilmshurst JM, Appleton DB, Grattan-Smith PJ
J Child Neurol 2000 Nov;15(11):717-22. doi: 10.1177/088307380001501102. PMID: 11108504

Clinical prediction guides

Heart rate variability in neonatal patients with seizures.
Statello R, Carnevali L, Alinovi D, Pisani F, Sgoifo A
Clin Neurophysiol 2018 Dec;129(12):2534-2540. Epub 2018 Oct 25 doi: 10.1016/j.clinph.2018.10.001. PMID: 30384023
Association of a synonymous SCN1B variant affecting splicing efficiency with Benign Familial Infantile Epilepsy (BFIE).
Usluer S, Kayserili MA, Eken AG, Yiş U, Leu C, Altmüller J, Thiele H, Nürnberg P, Sander T, Çağlayan SH
Eur J Paediatr Neurol 2017 Sep;21(5):773-782. Epub 2017 May 13 doi: 10.1016/j.ejpn.2017.05.001. PMID: 28566192
Benign infantile seizures.
Mishra D, Nikunj NK, Juneja M, Talukdar B
Indian Pediatr 2015 Feb;52(2):151-2. doi: 10.1007/s13312-015-0592-3. PMID: 25691188
Dominant-negative effects of KCNQ2 mutations are associated with epileptic encephalopathy.
Orhan G, Bock M, Schepers D, Ilina EI, Reichel SN, Löffler H, Jezutkovic N, Weckhuysen S, Mandelstam S, Suls A, Danker T, Guenther E, Scheffer IE, De Jonghe P, Lerche H, Maljevic S
Ann Neurol 2014 Mar;75(3):382-94. Epub 2014 Mar 18 doi: 10.1002/ana.24080. PMID: 24318194
Ion channel variation causes epilepsies.
Moulard B, Picard F, le Hellard S, Agulhon C, Weiland S, Favre I, Bertrand S, Malafosse A, Bertrand D
Brain Res Brain Res Rev 2001 Oct;36(2-3):275-84. doi: 10.1016/s0165-0173(01)00104-7. PMID: 11690625

Recent systematic reviews

KCNQ2-Related Epilepsy: Genotype-Phenotype Relationship with Tailored Antiseizure Medication (ASM)-A Systematic Review.
Falsaperla R, Criscione R, Cimino C, Pisani F, Ruggieri M
Neuropediatrics 2023 Oct;54(5):297-307. Epub 2023 Mar 22 doi: 10.1055/a-2060-4576. PMID: 36948217
Anticonvulsants for neonates with seizures.
Booth D, Evans DJ
Cochrane Database Syst Rev 2004 Oct 18;(4):CD004218. doi: 10.1002/14651858.CD004218.pub2. PMID: 15495087

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