Long term prognosis of juvenile absence epilepsy.

INTRODUCTION: Juvenile absence epilepsy (JAE) is a generalized form of epilepsy, characterized by absence seizures (AS) initiated in adolescence, with a typical EEG showing generalized spike-wave discharges. Apart from absences, other seizure types may be observed such as myoclonia and generalized tonic-clonic seizures (GTCS). Its long-term prognosis is uncertain. MATERIAL AND METHODS: We retrospectively selected all patients who met the 1989 ILAE diagnostic criteria for JAE. We analysed clinical variables, pharmacological treatment, and seizure remission with medical treatment and seizure relapse after stopping medical treatment. RESULTS: We identified 21 patients, 17 women and 4 men, 86% of whom had suffered GTCS and 14% myoclonias. Mean age at AS onset was 17 years old (range 10-44), 4 patients debuted with AS in adulthood. Mean follow up duration was 25 years (range 10-43). Ninety per cent of the patients were treated with valproate and 62% needed polytherapy. Currently 43% have achieved seizure freedom under medical treatment. All attempts to stop treatment failed, in some cases after long periods of seizure remission. CONCLUSIONS: Less than fifty per cent of patients with JAE achieve remission, antiepileptic treatment is mandatory during all life, despite having long periods of remission.

[Status epilepticus]. / Estatus epiléptico.

Status epilepticus is a neurological emergency that requires prompt care. The diagnosis and treatment must be continuous from the first minutes to its resolution. The most frequent cause in epileptic patients is drug change or non-compliance and in the non-epileptic patients are cerebrovascular diseases, head trauma, drug toxicity and metabolic disturbances. Status epilepticus can be convulsive or non-convulsive and continuous electroencephalographic monitoring is useful for diagnosis and to evaluate response to treatment. Benzodiazepines and phenytoin or fosphenytoin are first-line and second-line therapy. There is no agreement on third and fourth line therapy: phenobarbital, valproate, levetiracetam, propofol, midazolam, barbiturates and others. The prognosis of status epilepticus is related to etiology, age, type and duration of the status. Thus, drug treatment for status epilepticus should be started without delay.

Estatus epiléptico / Status epilepticus

El estatus epiléptico es una emergencia neurológica que requiere una atención inmediata. El diagnóstico y el tratamiento deben ser continuos a lo largo de los primeros minutos hasta su resolución. La causa más frecuente en pacientes epilépticos es el cambio o el incumplimiento de la medicación y en los no epilépticos son las lesiones vasculares, traumáticas, tóxicas y metabólicas. El estatus epiléptico puede ser convulsivo o no convulsivo y la monitorización electroencefalográfica continua es de gran ayuda para el diagnóstico y para valorar la respuesta al tratamiento. Las benzodiazepinas y la fenitoína o fosfenitoína son los fármacos de elección de primera y segunda línea. No existe consenso sobre los tratamientos de tercera y cuarta línea, entre los que se encuentran: fenobarbital, valproato, levetiracetam, propofol, midazolam, los barbitúricos y otros. El pronóstico dependerá de la causa, la edad, el tipo de estatus y la duración del mismo. Por ello, el tratamiento debe ser lo más precoz posible (AU)

Status epilepticus is a neurological emergency that requires prompt care. The diagnosis and treatment must be continuous from the first minutes to its resolution. The most frequent cause in epileptic patients is drug change or non-compliance and in the non-epileptic patients are cerebrovascular diseases, head trauma, drug toxicity and metabolic disturbances. Status epilepticus can be convulsive or non-convulsive and continuous electroencephalographic monitoring is useful for diagnosis and to evaluate response to treatment. Benzodiazepines and phenytoin or fosphenytoin are first-line and second-line therapy. There is no agreement on third and fourth line therapy: phenobarbital, valproate, levetiracetam, propofol, midazolam, barbiturates and others. The prognosis of status epilepticus is related to etiology, age, type and duration of the status. Thus, drug treatment for status epilepticus should be started without delay (AU)

Pronóstico a largo plazo de la epilepsia ausencia juvenil / Long term prognosis of juvenile absence epilepsy

Introducción: La epilepsia ausencia juvenil (EAJ) es un tipo de epilepsia generalizada idiopática que se caracteriza por la presencia de crisis de ausencia (CA) que comienzan en la adolescencia, con un EEG típico de punta-onda generalizada, y que puede acompañarse de mioclonías y crisis tónico-clónicas generalizadas (CTCG). El pronóstico a largo plazo es incierto. Material y métodos: Seleccionamos de manera retrospectiva todos los pacientes que cumplían los criterios diagnósticos de EAJ de la ILAE 1989, analizamos las variables clínicas, el tratamiento farmacológico, el estar libre de crisis y la posibilidad de retirar el tratamiento. Resultados: Encontramos 21 pacientes, 17 mujeres y 4 varones, el 86% presentó también CTCG y el 14% mioclonías. La edad de inicio de las CA fue de 17 años (rango: 10-44). Cuatro pacientes comenzaron con CA en la edad adulta. El seguimiento medio fue de 25 años (intervalo: 10-43). El 90% recibió tratamiento con valproato y el 62% requirió politerapia. El 43% de los pacientes están actualmente libres de crisis, aunque todos ellos en tratamiento farmacológico. Todos los intentos de retirar la medicación fracasaron, pese a largos períodos sin crisis.Conclusiones: Menos de la mitad de los pacientes con EAJ están libres de crisis. El tratamiento antiepiléptico es necesario durante toda la vida a pesar de largos períodos de remisiones (AU)

Introduction: Juvenile absence epilepsy (JAE) is a generalized form of epilepsy, characterizedby absence seizures (AS) initiated in adolescence, with a typical EEG showing generalized spikewavedischarges. Apart from absences, other seizure types may be observed such as myocloniaand generalized tonic-clonic seizures (GTCS). Its long-term prognosis is uncertain Material and methods: We retrospectively selected all patients who met the 1989 ILAE diagnostic criteria for JAE. We analysed clinical variables, pharmacological treatment, and seizure remission with medical treatment and seizure relapse after stopping medical treatment. Results: We identified 21 patients, 17 women and 4 men, 86% of whom had suffered GTCS and 14% myoclonias. Mean age at AS onset was 17 years old (range 10-44), 4 patients debuted with AS in adulthood. Mean follow up duration was 25 years (range 14-43). Ninety per cent of the patients were treated with valproate and 62% needed polytherapy. Currently 43% have achieved seizure freedom under medical treatment. All attempts to stop treatment failed, in some cases after long periods of seizure remission. Conclusions: Less than fifty per cent of patients with JAE achieve remission, antiepileptictreatment is mandatory during all life, despite having long periods of remission (AU)