Abstracts

Rationale: Juvenile myoclonic epilepsy (JME) is an heterogeneous genetic generalized epilepsy (GGE) whose cardinal symptoms are myoclonic jerks of upper extremities particularly after awakening. Although several prognostic predictors have been recognized, clinical diversity of JME is remarkable and the severity of the disorder itself has only rarely been analyzed. Praxis induction (PI) is one of reflex epileptic traits present in JME, defined as precipitation of epileptic seizures (sz) or EEG discharges by complex, cognition guided tasks, often involving visuomotor coordination and decision making. Clinical expression of PI in the nosology of JME and prognosis has been approached in a few publications. The aim of this study is to characterize PI in patients with JME and its relation with prognosis. Methods: After answering a questionnaire encompassing clinical data and reflex traits and having a 4-6 h Video-EEG Neuropsychological Protocol (VNPP) aiming to characterize reflex traits, 133 out of 208 patients with GGE were considered to have JME syndrome, and were grouped as: Group I, positive questionnaire and VNPP for PI trait (28/133; 21.0%); Group 2, absence of any reflex traits in both, questionnaire and VNPP (20/133; 15.0%). All of them had been treated with antiepileptic drugs (AEDs) considered adequate for GGEs. Outcome regarding sz control was assessed at least one year after appropriated AEDs onset (mean 7.28 y; SD 3.36) and classified according to Prasad et al. (2003) as follows: generalized tonic-clonic seizures (GTCS): good (<1 sz/year), moderate (1-4 sz/y), poor (>4 sz/y); myoclonia/absences: good (< 5 sz/clusters/month), moderate (5-14 sz/month) and poor (>15 sz/month, or daily). Noncompliant patients and those with drugs/alcohol intake were excluded. Results: In Group 1, 13/28 (46.4%) were females, and in Group 2, 11/20 (55.0%). Mean age was 28.52 y (17-51) in Group 1, and 32.75 y (21-53) in Group 2. The interval between epilepsy onset and regular outpatient Epilepsy Clinic follow-up average was 9.09 y (SD=5.73) and 11.52 y (SD=9.28), respectively. In Group 1, epilepsy duration average was 15.81 y (5-38) and in Group 2, 20.80 y (4-42). While 19/20 (95.0%) of Group 2 patients still presented clear chronosensitivity, this JME characteristic was referred by 20/28 (71.4%) of Group 1 patients (p=0.04). At the first visit, 23/28 patients of Group 1 (82.1%) and 12/20 of Group 2 (60.0%) were receiving carbamazepine/phenytoin. At the last evaluation, 75.0% (21/28) of Group 1 and 90.0% (18/20) of Group 2 had good control of myoclonia/absences. However, 11/28 (39.3%) of Group 1 and 5/20 (25.0%) of Group 2 patients still had moderate or poor GTCS control (p=0.18). Group 2 had 17/20 (85.0%) patients with valproate in monotherapy, and Group 1, 11/28 (39.7%). Finally, while 17/28 (60.7%) of Group 1 were on polytherapy, just 3/20 (15.0%) patients of Group 2 were receiving more than one AED (p=0.003). Conclusions: PI trait, present in about one-fifth of patients with JME, is related to seizures without preferential circadian occurrence. Patients with JME and PI more often need to receive polytherapy.