Patients present with motor arrest, unresponsiveness, head and/or eye deviation to one side, staring, fluttering of eyelids, grunting, cyanosis, diffuse hypertonia and unilateral or bilateral clonic jerks of the limbs. [orpha.net]

Newly diagnosed epilepsy in children: presentation at diagnosis. Epilepsia 1999; 40(4): 445-52. Kamada K, Moller M, Saguer M, Kassubek J, Kaltenhauser M, Kober H, Uberall M, Lauffer H, Wenzel D, Vieth J. [medigraphic.com]

• Epilepsy

  Benign partial epilepsy with centro-temporal spikes. In: Engel; Pedley T. eds. Epilepsy. A Comprehensive Textbook. Philadelphia: Lippincott-Raven 1998; 2307-2316. Stephani U, Doose H. Benign idiopathic partial epilepsy and brain lesion. [medigraphic.com]

  If you would like to know more about epilepsy in general, please contact the Epilepsy Action Helpline. [epilepsy.org.uk]

  The aim of this study was to determine whether these two epilepsy syndromes are allelic disorders. [onlinelibrary.wiley.com]

  Later seizures have been reported, including occasional febrile seizures and idiopathic epilepsy syndromes in childhood, in particular Rolandic epilepsy. The documents contained in this web site are presented for information purposes only. [orpha.net]

• Seizure

  The seizures affect girls and boys equally. [epilepsy.org.uk]

  Clinical description Seizure onset is usually between the second and the eighth day of life, in otherwise healthy newborns. Seizures are mostly focal involving alternatively both sides of the body and apnea is frequently associated. [orpha.net]

  Orpha Number: 140927 Definition Benign familial neonatal-infantile seizures (BFNIS) is a benign familial epilepsy syndrome with an intermediate phenotype between benign familial neonatal seizures (BFNS) and benign familial infantile seizures (BFIS; see [rarediseases.info.nih.gov]

  Observations of benign neonatal seizures and BECTS in the same individual are well documented. Neonatal seizures with benign course were found in increased numbers in a series of CTS carriers. [onlinelibrary.wiley.com]

• Convulsions

  There are 2 types of self-limited neonatal convulsions: • Self-limited familial neonatal convulsions • Self-limited non-familial (also called sporadic) neonatal convulsions In the first type, self-limited familial neonatal convulsions, someone else in [epilepsy.org.uk]

  Title Other Names: BFNIS; Convulsions benign familial neonatal; Epilepsy, benign neonatal-infantile; BFNIS; Convulsions benign familial neonatal; Epilepsy, benign neonatal-infantile; Benign familial infantile convulsions See More Categories: The following [rarediseases.info.nih.gov]

  Benign neonatal familial convulsions (BNFC) like BECTS is an idiopathic age‐dependent epilepsy with a benign course. Observations of benign neonatal seizures and BECTS in the same individual are well documented. [onlinelibrary.wiley.com]

  A syndrome called familial infantile convulsions and choreoathetosis (ICCA; see this term) has been observed in which BFIE patients present in childhood and/or adolescence with choreoathetotic dyskinetic attacks occurring spontaneously or following diverse [orpha.net]

  Benign familial neonatal convulsions followed by benign epilepsy with centro-temporal spikes in two siblings. Epilepsia 1999 40(1): 110-3. Bray PF, Wiser WC. Evidence for a genetic etiology of temporal central abnormalities infocal epilepsy. [medigraphic.com]

• Neonatal Seizures

  Benign neonatal familial convulsions (BNFC) like BECTS is an idiopathic age‐dependent epilepsy with a benign course. Observations of benign neonatal seizures and BECTS in the same individual are well documented. [onlinelibrary.wiley.com]

  Orpha Number: 140927 Definition Benign familial neonatal-infantile seizures (BFNIS) is a benign familial epilepsy syndrome with an intermediate phenotype between benign familial neonatal seizures (BFNS) and benign familial infantile seizures (BFIS; see [rarediseases.info.nih.gov]

• Meningism

  This may include a lumbar puncture test to look for meningitis or encephalitis, and a more detailed brain scan, the MRI scan. [epilepsy.org.uk]

Usually, patients require treatment for the first 6-12 months of life. However, it is important for clinicians and family to be aware that some patients require treatment beyond 12 months of age. Prognosis Prognosis is good. [orpha.net]

Treatment Treatment is not always necessary because the seizures may only happen for a few weeks. However, sometimes epilepsy medicine is prescribed, particularly if the seizures continue to happen. [epilepsy.org.uk]

You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments. [rarediseases.info.nih.gov]

Prognosis Prognosis is good. Seizures normally disappear during the first year of life and patients do not display any neurological sequelae. [orpha.net]

Prognosis of benign childhood epilepsy with centrotemporal spikes: A follow-up study of 168 patients. Epilepsia 1988; 29: 229-335. [medigraphic.com]

Etiology BFNE is a genetically heterogeneous disorder due to mutations in the KCNQ2 (20q13.33) and KCNQ3 (8q24) genes that both code for voltage-gated potassium channel subunits. [orpha.net]

Evidence for a genetic etiology of temporal central abnormalities infocal epilepsy. N Engl J Med 1964; 271: 926-33. Loiseau P, Duché B, Córdova S, Dartigues JF, Cohadon S. [medigraphic.com]

Summary Epidemiology Prevalence is currently unknown since this disorder is possibly overlooked. About 100 families have been reported to date. [orpha.net]