EYELID MIOCLONIA WITH AND WITHOUT ABSENCES: CLINICAL FORMS AND THEIR PROGNOSIS
Abstract number :
2.099
Submission category :
4. Clinical Epilepsy
Year :
2012
Submission ID :
16196
Source :
www.aesnet.org
Presentation date :
11/30/2012 12:00:00 AM
Published date :
Sep 6, 2012, 12:16 PM
Authors :
P. O. Concei o, F. P. Safatle, C. G. Uchida, K. Carvalho, I. S. Tudesco, R. Nojoza, G. M. Ara jo Filho, L. M. Guilhoto, E. M. Yacubian
Rationale: Eyelid myoclonia and absence or Jeavons Syndrome is considered a form of idiopathic generalized epilepsy, with eyelid myoclonia, childhood onset, photosensitivity and eye closure-induced seizures/EEG paroxysms. However, the main clinical features of Jeavons Syndrome, eyelid myoclonia, can be observed in different epileptic syndromes. This study aims to review electroclinical features of patients with idiopathic generalized epilepsy, presenting eyelid myoclonia with and without absences and classify them according different proposals. Methods: Clinical and video-EEG data of patients whith eyelid myoclonia or eyelid mioclonia and absences were analyzed and classified in electroclinical groups according to the following authors: a) Covanis et al. (2005): 1) early onset eyelid myoclonia (<4yrs.), normal MRI and intellectual disability; 2) generalized tonic-clonic seizures (GTCS) at onset, normal MRI and intellectual disability; 3) benign course with infrequent limb myoclonia; 4) classical form with eyelid myoclonia, onset in childhood, photosensitivity and eye closure-induced seizures/EEG paroxysms; 5) juvenile myoclonic epilepsy and later onset eyelid myoclonia; 6) eyelid myoclonia associated with frequent non convulsive status; b) Yalçin et al. (2006): overlap cases of EMA and JME; c) Caraballo et al. (2009): 1) classical JS; 2) eyelid mioclonia and absences with ID; 3) eyelid myoclonia with frequent GTCS and limb myoclonia; and d) Capovilla et al. (2009): eyelid myoclonia with intellectual disability and polyspike / polyspike and waves in ictal EEG. Results: Seventeen female patients aged 10-35 years (mean 23.6) were included. Applying the Covanis' classification, 12 (70.6%) could be classified, 5 in group 1; 5 in group 5; and 2 in group 4. Four patients (23.5%) were included in Yalçin group. When Caraballo's proposal was applied, 14 patients (82.3%) were classified, 9 in group 3; 3 in group 1 and 2 in group 2. Finally, one patient (5.9%) was included in Capovilla's group. All groups, but Covanis's fifth and Yalçin group, had more than 50% of patients drug resistant. Conclusions: The present data demonstrated that the proposed classifications are not able to include all clinical features of patients with eyelid myoclonia in idiopathic generalized epilepsy. Although using Caraballo's proposal the majority of patients could be classified, Covanis's classification was the only one which delineated difference among the 6 groups related to prognosis.
Clinical Epilepsy