Epilepsy Nocturnal Frontal Lobe 3 (ENFL3)

OMIM number: 605375

Comments closing date: 05/09/2025

Nocturnal frontal lobe focal epilepsy (ENFL3) is a rare autosomal dominant inherited disorder
caused by a gene variant (CHRNB2). Offspring of affected individuals have a 50% chance of
inheriting the gene. An HFEA licence exists for EFNL5. Onset is commonly in childhood and
results in multiple seizures during sleep. These seizures cluster together and may be as many as
8 at a time. Involuntary body movements may accompany the seizures including unintelligible
speech, hyper extension of head and neck, upper limb jerking movement. Some of those affected
may have developmental delay as a result of multiple seizures from an early age. Affected
children/adults may suffer fragmentation of sleep due to nighttime disturbance with a resulting
tiredness on waking and ongoing impact of daily living. Some individuals will have mental health
problems and memory deficit. There is no curative treatment, but management will involve medication to control the seizures. Around one third of patients may be resistant to the medication
treatment or it may take considerable time to stabilise the seizures. Surgery to the frontal lobe
can be considered in extreme cases. Those who have developmental delay will require ongoing
social and educational support.

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