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Sleep-related hypermotor epilepsy (SHE), previously Nocturnal Frontal Lobe Epilepsy (NFLE), is characterized by hypermotor seizures arising predominantly from sleep. Recognized etiologies include acquired injuries, structural anomalies and genetic causes [1]. The first gene for SHE, CHRNA4 (Cholinergic Receptor Nicotinic Alpha 4 Subunit MIM *118504), was identified in 1995 by linkage analysis in a large pedigree showing an autosomal dominant pattern of transmission (ADSHE). Subsequently, mutations in two homologous genes, CHRNB2 (Cholinergic Receptor Nicotinic Beta 2 Subunit, MIM *118507) and CHRNA2 (Cholinergic Receptor Nicotinic Alpha 2 Subunit, MIM *118502) have been highlighted [2].