Chloral hydrate for progressive myoclonus epilepsy: a new look at an old drug.

This study demonstrates that chloral hydrate can be used to control daytime myoclonic exacerbations. It reports on four patients with progressive myoclonus epilepsy--three with Unverricht-Lündborg disease (EPM1) and one with progressive external ophthalmoplegia (PEO)--all of whom were taking more than one antiepileptic drug. Response to the liquid formulation was faster than response to the capsule and was preferred by the patients. The unusual feature was less than expected sedation or development of tolerance even at daily doses above 500 mg administered for years. Because chloral hydrate helped to improve quality of life, it should be made available to patients with progressive myoclonus epilepsy as adjunctive therapy. Recent evidence of interactions with various excitatory and inhibitory amino acid neurotransmitter-operated ion channels as a mechanism of action may provide insight into altered neurotransmission in progressive myoclonus epilepsy.