Prader-Willi syndrome is a multisystemic genetic disorder that can be associated with epilepsy. There is insufficient information concerning the clinical and electroencephalographic characteristics of epilepsy and the long-term outcome of these patients. The aim of this study is to describe seizure types, electroencephalographic patterns and long-term seizure outcome in Prader-Willi syndrome patients suffering from epilepsy. We retrospectively studied 38 patients with Prader-Willi syndrome and seizures. Results of neuroimaging studies were obtained for 35 individuals. We subdivided these patients into two groups: group A, 24 patients, without brain lesions; and group B, 11 patients, with brain abnormalities. All patients were re-evaluated after a period of at least 10 years. Twenty-one patients (55.2 %) were affected by generalized epilepsy and 17 patients (44.8 %) presented focal epilepsy. The most common seizure type was generalized tonic-clonic seizure. The mean age at seizure onset was 4.5 years (ranged from 1 month to 14 years). In the follow-up period, seizure freedom was achieved in 32 patients (84.2 %). Seizure freedom was associated with electroencephalographic normalization, while the six children presenting drug-resistant epilepsy showed persistence of electroencephalographic abnormalities. Group B patients showed a higher prevalence of drug-resistant epilepsy. Patients with Prader-Willi syndrome were frequently affected by generalized seizures. Most of the patients had a favorable evolution, although, patients with brain abnormalities presented a worse outcome, suggesting that the presence of these lesions can influence the response to antiepileptic therapy.
|Titolo:||Long-term outcome of epilepsy in patients with Prader-Willi syndrome.|
|Data di pubblicazione:||2015|
|Citazione:||Long-term outcome of epilepsy in patients with Prader-Willi syndrome. / Verrotti A; Cusmai R; Laino D; Falsaperla R; Margari L; Rizzo R; Savasta S; Grosso S; Striano P; Belcastro V; Franzoni E; Curatolo P; Giordano L; Freri E; Matricardi S; Pruna D; Toldo I; Tozzi E; Lobefalo L; Operto F; Altobelli E; Chiarelli F; Spalice A.. - In: JOURNAL OF NEUROLOGY. - ISSN 0340-5354. - 262:1(2015), pp. 116-123.|
|Appare nelle tipologie:||1.1 Articolo in rivista|