Nocturnal paroxysmal dystonia related to a prerolandic dysplasia

Nocturnal paroxysmal dystonia (NPD) is a rare disorder characterized by att acks of short-lived dystonic, tonic and choreoatetoid movements occurring m ainly during sleep. Although seizures are believed to arise from the fronta l lobe, their localization is, however, uncertain due to the lack of ictal clinical-EEG correlations. Two patients are reported with episodes clinical ly compatible with NPD who also experienced occasional generalized tonic-cl onic seizures in which there was a frontal (prerolandic) dysplasia detected by MRI. In one patient interictal/ictal SPECTs suggested that the seizure focus was over the area of dysplasia. Both patients support the notion that NPD is a type of epilepsy arising from the frontal lobe, possibly originat ing in the prerolandic region. (C) 2001 Published by Elsevier Science B.V.