Benign familial neonatal seizures

Henry Hasson MD (Dr. Hasson of Albert Einstein College of Medicine has no relevant financial relationships to disclose.)
Solomon L Moshé MD (Dr. Moshe of Albert Einstein College of Medicine received honorariums from UCB as a member of a data and safety monitoring board.)
Jerome Engel Jr MD PhD, editor. (Dr. Engel of the David Geffen School of Medicine at the University of California, Los Angeles, has no relevant financial relationships to disclose.)
Originally released October 18, 1993; last updated May 1, 2016; expires May 1, 2019

This article includes discussion of benign familial neonatal seizures, benign-familial neonatal seizures, benign-familial neonatal convulsions, benign neonatal familial convulsions, and BFNS. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Benign familial neonatal seizures is a syndrome characterized by focal clonic seizures, occasionally with a generalized tonic-clonic component, that occurs in full-term neonates. The seizures usually begin within 2 to 8 days of birth and remit by 16 months. As the name implies, this syndrome is usually familial and self-limited, but a work-up is necessary to rule out other causes of seizures. This syndrome is caused by a mutation in the gene that encodes voltage-gated K+ channel subunits (KCNQ2, KCNQ3). These subunits are responsible for the M-current, which is important in neuronal stability. There is no specific treatment at this time; however, there are several new drugs for which the mechanism of action looks promising.

Key points

 

• Seizures occur in full-term neonates, usually beginning within 2 to 8 days from birth.

 

• The disorder is familial and benign.

 

• There is no specific treatment. Antiseizure medications are sometimes used.

 

• This syndrome is caused by mutations in the gene encoding voltage-gated K+ channels.

Historical note and terminology

Antoni described a hereditary form of neonatal seizures with good outcome for neurologic development (Antoni 1941). Rett and Teubel were the first to describe the syndrome in a family (Rett and Teubel 1964). The term “benign” was not in the original description by Rett but was added 4 years later. Interestingly, a reanalysis of Rett's original kindred 40 years later revealed that they met current clinical and genetic criteria for benign familial neonatal seizures (Zimprich 2006). The first case reported in the English literature appeared in 1968 (Bjerre and Corelius 1968). Since 1989, benign familial neonatal seizures have appeared in the classification of epilepsy and epilepsy syndromes. They are classified in the chapter of Idiopathic Generalized Epilepsies.

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