Epilessia e sindrome di Down in età adulta

Even if Down’s syndrome is the major cause of genetic mental retardation, epilepsy is reported to have a greater prevalence in these patients than in general population, since they achieve older ages when the prevalence of epilepsy in this population ranges 46%. The aim of the present study is to describe the electroclinical characteristics of epilepsy in adult patients with Down’s syndrome followed at the Epilepsy Center, San Paolo Hospital in Milan. Twenty adult patients with Down’ s syndrome (10 males, 10 females), with a mean age 44.5 years (range: 28-64 years), were found. Mean age at epilepsy onset was 33 years (range: 6-60 years). Eleven out of 20 patients presented focal epilepsy, while 6 showed a Late-Onset Myoclonic Epilepsy (LOMEDS). The EEG pattern of our patients was characterized by a progressive slowing of the background activity and a prevalence of paroxysmal activities over the fronto-temporal regions. In the patients diagnosed as LOMEDS the EEGs showed generalized polyspike-waves. Nearly half of the patients (40%) suffered from drug-resistant epilepsy and 4 subjects presented an epileptic status at the beginning or in the course of the disease. Epilepsy in adult patients with Down’s syndrome shows peculiar electro-clinical characteristics which should be early recognized, since as longevity of these patients is increasing, they could be encountered in clinical practice more frequently than currently acknowledged.