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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; Convulsion, Benign Neonatal; Convulsions, Benign Neonatal; Epilepsies, Benign Neonatal; Epilepsy, Benign Neonatal; Neonatal Convulsion, Benign; Neonatal Convulsions, Benign; Neonatal Epilepsies, Benign; Neonatal 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: 37596007Free PMC Article
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

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
Andreas Rett and benign familial neonatal convulsions revisited.
Zimprich F, Ronen GM, Stögmann W, Baumgartner C, Stögmann E, Rett B, Pappas C, Leppert M, Singh N, Anderson VE
Neurology 2006 Sep 12;67(5):864-6. doi: 10.1212/01.wnl.0000234066.46806.90. PMID: 16966552
Ion channels and epilepsy.
Lerche H, Jurkat-Rott K, Lehmann-Horn F
Am J Med Genet 2001 Summer;106(2):146-59. doi: 10.1002/ajmg.1582. PMID: 11579435

Diagnosis

ILAE Genetic Literacy Series: Self-limited familial epilepsy syndromes with onset in neonatal age and infancy.
Millevert C, Weckhuysen S; ILAE Genetics Commission
Epileptic Disord 2023 Aug;25(4):445-453. Epub 2023 Jun 22 doi: 10.1002/epd2.20026. PMID: 36939707
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
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

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
Low-dose carbamazepine therapy for benign infantile convulsions.
Matsufuji H, Ichiyama T, Isumi H, Furukawa S
Brain Dev 2005 Dec;27(8):554-7. doi: 10.1016/j.braindev.2005.01.005. PMID: 16310589
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
Andreas Rett and benign familial neonatal convulsions revisited.
Zimprich F, Ronen GM, Stögmann W, Baumgartner C, Stögmann E, Rett B, Pappas C, Leppert M, Singh N, Anderson VE
Neurology 2006 Sep 12;67(5):864-6. doi: 10.1212/01.wnl.0000234066.46806.90. PMID: 16966552
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

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
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
Heart and respiration rate changes in the neonate during electroencephalographic seizure.
Greene BR, de Chazal P, Boylan G, Reilly RB, O'Brien C, Connolly S
Med Biol Eng Comput 2006 Mar;44(1-2):27-34. doi: 10.1007/s11517-005-0001-5. PMID: 16929918
New human sodium/glucose cotransporter gene (KST1): identification, characterization, and mutation analysis in ICCA (infantile convulsions and choreoathetosis) and BFIC (benign familial infantile convulsions) families.
Roll P, Massacrier A, Pereira S, Robaglia-Schlupp A, Cau P, Szepetowski P
Gene 2002 Feb 20;285(1-2):141-8. doi: 10.1016/s0378-1119(02)00416-x. PMID: 12039040
Benign familial infantile convulsions: mapping of a novel locus on chromosome 2q24 and evidence for genetic heterogeneity.
Malacarne M, Gennaro E, Madia F, Pozzi S, Vacca D, Barone B, dalla Bernardina B, Bianchi A, Bonanni P, De Marco P, Gambardella A, Giordano L, Lispi ML, Romeo A, Santorum E, Vanadia F, Vecchi M, Veggiotti P, Vigevano F, Viri F, Bricarelli FD, Zara F
Am J Hum Genet 2001 Jun;68(6):1521-6. Epub 2001 Apr 20 doi: 10.1086/320596. PMID: 11326335Free PMC Article

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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