Publication: Cognición en epilepsia generalizada idiopática: estudio clínico y de vídeo-electroencefalograma
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2018-03-23
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Universidad Complutense de Madrid
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Abstract
Las Epilepsias Generalizadas Idiopáticas (EGI) constituyen una quinta parte de todos los casos de epilepsia. Se trata de un conjunto de síndromes de inicio en la infancia o adolescencia caracterizados por la combinación variable de crisis de ausencia, tónico clónicas generalizadas (TCG) y mioclónicas; por una frecuente sensibilidad a los fármacos antiepilépticos, especialmente el valproato (VPA); y por la presencia de rasgos reflejos como la fotosensibilidad. El hallazgo característico en el electroencefalograma (EEG) es la descarga de punta-onda (PO) o polipunta-onda (PPO) generalizada a 3-4 Hz, que suele mostrar activación con el sueño no REM, la hiperventilación (HV), la Estimulación Lumínica Intermitente (ELI) y/o la realización de ciertas praxias. Los cuatro síndromes principales son: Epilepsia de ausencias infantiles (EAI); Epilepsia de Ausencias Juveniles (EAJ); Epilepsia Mioclónica Juvenil (EMJ); y Epilepsia con Crisis Tónico Clónicas Sólo (EGI-Sólo TCG). Entre los rasgos reflejos característicos de la EGI, la activación o inducción con praxias (IP) se define por la inducción de actividad epileptiforme (AE) y crisis epilépticas con actividades que requieren concentración y algún movimiento dirigido de las manos (jugar al ajedrez, escribir, o hacer un puzzle). Es más frecuente y característica en EMJ (12,7-84%), si bien se ha descrito en todos los síndromes dentro de la EGI...
Idiopathic Generalized Epilepsies (IGE) account for 15-20% of cases of epilepsy. They comprise a group of different syndromes starting during childhood or adolescence and characterized by a variable combination of absences, generalized tonic-clonic (GTC) and myoclonic seizures. They have a good response to antiepileptic drugs, particularly to valproic acid (VPA), and present typical reflexes such as photosensitivity. Hallmark on EEG are generalized epileptiform discharges (ED) of spike-and-wave (SW) or Polyspike-and-wave (PSW) that are typically induced by non-REM sleep, hyperventilation (HV), Intermittent Photic Stimulation (IPS) and certain praxis. Principal IGE Syndromes are Childhood Absences Epilepsy (CAE), Juvenile Absences Epilepsy (JAE), Juvenile Myoclonic Epilepsy (JME), and Epilepsy with Generalized Tonic –Clonic Seizures Alone (IGE-GTCS). Paxis induction (PI) in IGE is defined as the precipitation of ED and/or seizures followed by complex and cognition-guided tasks often involving visuomotor coordination with hands (playing chess, writing, etc). PI is typical and more frequent in JME (12.7-84%), although if it has been described in all IGE syndromes. IGE is also characterized by an intellectual functioning within the normal range. Nevertheless, cognition in IGE has been mainly studied in patients with JME. Several authors have found an executive dysfunction associated with a specific personality profile in this group; firstly described by Janz in a published series of cases of JME (1957) as “unsteadiness, lack of discipline and hedonism”...
Idiopathic Generalized Epilepsies (IGE) account for 15-20% of cases of epilepsy. They comprise a group of different syndromes starting during childhood or adolescence and characterized by a variable combination of absences, generalized tonic-clonic (GTC) and myoclonic seizures. They have a good response to antiepileptic drugs, particularly to valproic acid (VPA), and present typical reflexes such as photosensitivity. Hallmark on EEG are generalized epileptiform discharges (ED) of spike-and-wave (SW) or Polyspike-and-wave (PSW) that are typically induced by non-REM sleep, hyperventilation (HV), Intermittent Photic Stimulation (IPS) and certain praxis. Principal IGE Syndromes are Childhood Absences Epilepsy (CAE), Juvenile Absences Epilepsy (JAE), Juvenile Myoclonic Epilepsy (JME), and Epilepsy with Generalized Tonic –Clonic Seizures Alone (IGE-GTCS). Paxis induction (PI) in IGE is defined as the precipitation of ED and/or seizures followed by complex and cognition-guided tasks often involving visuomotor coordination with hands (playing chess, writing, etc). PI is typical and more frequent in JME (12.7-84%), although if it has been described in all IGE syndromes. IGE is also characterized by an intellectual functioning within the normal range. Nevertheless, cognition in IGE has been mainly studied in patients with JME. Several authors have found an executive dysfunction associated with a specific personality profile in this group; firstly described by Janz in a published series of cases of JME (1957) as “unsteadiness, lack of discipline and hedonism”...
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Tesis inédita de la Universidad Complutense de Madrid, Facultad de Medicina, Departamento de Medicina, leída el 29-06-2017