CARATTERISTICHE ELETROCLINICHE DELL'EPILESSIA NELLA SINDROME DI DOWN

In spite of numerous case reports and epidemiological surveys, the distribution of seizure types and epileptic syndromes in patients with Down syndrome (DS) and epilepsy is still largely unknown. We therefore decided to retrospectively study 38 cases of DS (17 male and 21 female) patients with epilepsy (32 cases) or with a fortuitous convulsive attack (6 cases). Epileptic syndromes were classified in the 32 epilepsy cases as: West syndrome, 10 cases (7F, 3M), i.e. 31%; Lennox-Gastaut syndrome, 5 cases (4F, 1M), i.e. 16%; symptomatic generalized epilepsy, 1 case (1F); idiopathic generalized epilepsy, 6 cases (3F, 3M), i.e. 19%; symptomatic partial epilepsy, 8 cases (3F, 5M), i.e. 25%. 2 cases were unclassifiable. In these 32 epileptic patients, the following particularities were noted: 1) in these DS patients, the West syndrome appears to have a relatively mild prognosis, as 8/10 remained seizure-free, 3 of whom without treatment; 2) no patient experienced febrile convulsions prior to the onset of epilepsy; 3) Lennox-Gastaut syndrome had a relatively late onset (mean age: 10 years, range 8-11.5); 4) reflex seizures were noted in 7 patients (22%). Among the 6 cases in which a long-term follow-up revealed no recurrence after a single seizure, 3 had experienced a seizure following open-heart surgery.