Neonatal sporadic hyperekplexia: a rare and often unrecognized entity.

We report the case of a newborn infant with transient generalized stiffness, obvious from the first days of life, increased muscle tone and repeated myoclonic jerks, who was unsuccessfully treated with phenobarbital until the diagnosis of neonatal sporadic hyperekplexia. To our knowledge this is the first case successfully treated with clobazam, a 1.5 dibenzodiazepine.