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Benign Neonatal Epilepsy (Benign Familial Neonatal Convulsions)

A condition marked by recurrent seizures that occur during the first 4-6 weeks of life despite an otherwise benign neonatal course. Autosomal dominant familial and sporadic forms have been identified. Seizures generally consist of brief episodes of tonic posturing and other movements, apnea, eye deviations, and blood pressure fluctuations. These tend to remit after the 6th week of life. The risk of developing epilepsy at an older age is moderately increased in the familial form of this disorder. (Neurologia 1996 Feb;11(2):51-5)
Also Known As:
Benign Familial Neonatal Convulsions; Epilepsy, Benign Neonatal; Benign Familial Infantile Convulsions; Benign Familial Infantile Convulsions Syndrome; Benign Familial Neonatal Epilepsy; Benign Familial Neonatal-Infantile Seizures; Benign Infantile Familial Convulsions; Benign Neonatal Convulsions; Benign Neonatal Epilepsy, Nonfamilial; Benign Neonatal Non-Familial Convulsions; Benign Neonatal Nonfamilial Epilepsy; Benign Non-Familial Neonatal Convulsions; Convulsions Benign Familial Neonatal; Convulsions, Benign Familial Infantile, 1; Convulsions, Benign Familial Infantile, 3; Convulsions, Benign Neonatal, Familial; Convulsions, Benign Neonatal, Non-Familial; Epilepsy, Benign Neonatal, Familial; Epilepsy, Benign Neonatal, Non-Familial; Epilepsy, Benign Neonatal, Nonfamilial; Epilepsy, Benign Neonatal-Infantile; Familial Benign Neonatal Convulsions; Familial Benign Neonatal Epilepsy; Non-Familial Benign Neonatal Convulsions; Non-Familial Benign Neonatal Epilepsy; Seizures, Benign Familial Infantile, 1; Seizures, Benign Familial Infantile, 3; Seizures, Benign Familial Neonatal-Infantile; Benign Familial Neonatal Infantile Seizures; Benign Neonatal Convulsion; Benign Neonatal Epilepsies; Benign Neonatal Non Familial Convulsions; Benign Neonatal-Infantile Epilepsies; Benign Neonatal-Infantile Epilepsy; Benign Non Familial Neonatal Convulsions; Convulsion, Benign Neonatal; Convulsions, Benign Neonatal; Epilepsies, Benign Neonatal; Epilepsies, Benign Neonatal-Infantile; Epilepsy, Benign Neonatal Infantile; Neonatal Convulsion, Benign; Neonatal Epilepsies, Benign; Neonatal Epilepsy, Benign; Neonatal-Infantile Epilepsies, Benign; Neonatal-Infantile Epilepsy, Benign; Non Familial Benign Neonatal Convulsions; Non Familial Benign Neonatal Epilepsy; Seizures, Benign Familial Neonatal Infantile; Neonatal Convulsions, Benign
Networked: 100 relevant articles (3 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Epileptic Syndromes
2. Epilepsy (Aura)
3. Generalized Epilepsy with Febrile Seizures Plus
4. Myoclonic Epilepsies (Myoclonic Encephalopathy)
5. Febrile Seizures (Febrile Seizure)

Experts

1. Hirose, Shinichi: 9 articles (01/2019 - 09/2002)
2. Taglialatela, Maurizio: 6 articles (01/2019 - 01/2002)
3. Berkovic, Samuel F: 5 articles (10/2012 - 09/2002)
4. Scheffer, Ingrid E: 5 articles (10/2012 - 09/2002)
5. Miceli, Francesco: 4 articles (03/2014 - 01/2006)
6. Soldovieri, Maria Virginia: 4 articles (03/2014 - 01/2006)
7. Mulley, John C: 4 articles (06/2007 - 09/2002)
8. Ishii, Atsushi: 3 articles (01/2019 - 03/2009)
9. Hui, Zhi-Yan: 3 articles (11/2018 - 08/2011)
10. Zhou, Xi-Hui: 3 articles (11/2018 - 08/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Benign Neonatal Epilepsy:
1. Potassium Channels (Potassium Channel)IBA
2. Sodium Channels (Sodium Channel)IBA
3. Mutant Proteins (Protein, Mutant)IBA
4. Voltage-Gated Sodium Channel beta-1 SubunitIBA
5. Cholinergic ReceptorsIBA
6. Voltage-Gated Potassium Channels (Voltage-Gated Potassium Channel)IBA
7. Nicotinic Receptors (Nicotinic Acetylcholine Receptor)IBA
8. Ion Channels (Ion Channel)IBA
06/01/2000 - "We studied the main ion channel disorders related to simply inherited idiopathic epileptic syndromes in which four genes have been codified to date: benign familial neonatal convulsions, generalized epilepsy with febrile seizures plus and autosomal dominant nocturnal frontal lobe epilepsy. "
06/01/2000 - "Two human epilepsy syndromes, benign familial neonatal convulsions and generalized epilepsy with febrile seizures plus, represent K+ and Na+ channelopathies, and other newly defined syndromes have now been mapped to chromosomal regions that are rich in ion channel genes. "
12/01/2006 - "Thus, the discovery of KCNQ2 mutations in benign familial neonatal convulsions, SCN1A mutations in severe myoclonic epilepsy of infancy and in generalized epilepsy with febrile seizures plus, and CHRA4 and CHRB2 mutations in autosomal-dominant nocturnal frontal lobe epilepsy, has led to the establishment of epilepsy as a disorder of ion channel function and, furthermore, has led to the introduction of genetic tests that are available clinically to aid in diagnosis and treatment. "
01/01/2005 - "Genetic analyses of familial epilepsies over the past decade have identified mutations in several different ion channel genes that result in neonatal or early-onset seizure disorders, including benign familial neonatal convulsions (BFNC), generalized epilepsy with febrile seizures plus (GEFS+), and severe myoclonic epilepsy of infancy (SMEI). "
12/01/2000 - "Recently, gene defects underlying four monogenic epilepsies (generalized epilepsy with febrile seizures, autosomal dominant nocturnal frontal lobe epilepsy, benign familial neonatal convulsions and episodic ataxia type 1 with partial seizures) have been identified, shedding new light on the pathophysiology of epilepsy as these diseases are caused by ion channel mutations. "
9. Voltage-Gated Sodium ChannelsIBA
10. PotassiumIBA

Therapies and Procedures

1. Therapeutics
2. Ligation