A newly recognized autosomal dominant epilepsy syndrome with linkage to chromosome 2p11.1-q12.2 is described in an Italian pedigree of 8 patients reported from the Neurosciences Unit, Institute of Child Health and Great Ormond Street Hospital for Children, London, UK. Onset between ages 12 and 50 years, the syndrome is characterized by distal, semi-continuous rhythmic cortical myoclonus, generalized tonic-clonic and complex partial epileptic seizures (ADCME). The majority had only occasional seizures. Seizures were intractable in 3 who developed mild mental retardation. Valproate was most effective, while carbamazepine controlled seizures but exacerbated myoclonus. Interictal EEG abnormalities were generalized and focal frontotemporal. EMG studies of the myoclonus confirmed that EMG activity was preceded by EEG activity by 8-15 ms, recorded over the contralateral rolandic area, suggesting a cortical origin. 
COMMENT. This autosomal dominant cortical myoclonus epilepsy syndrome (ADCME) in an Italian pedigree shares some of the characteristics of familial adult myoclonic epilepsy (FAME) described in pedigrees from Japan, except that FAME is linked to chromosome 8q23.3-q24.1, whereas ADCME linkage is to chromosome 2p11.1-q12.2. Clinical characteristics of ADCME include mainly adult onset, nonprogressive course, rhythmic myoclonus enhanced during movement, generalized tonic-clonic seizures, and sometimes complex partial seizures.