Muerte súbita en epilepsia: casuística de una unidad médica de epilepsia española / Sudden unexpected death in epilepsy: Incidence at a Spanish epilepsy unit

INTRODUCCIÓN: La muerte súbita en epilepsia (SUDEP) es la causa más frecuente de muerte atribuible a la propia enfermedad. Casi toda la información sobre esta entidad procede de estudios realizados en el centro/norte de Europa y Estados Unidos. Presentamos la casuística de SUDEP de la Unidad Médica de Epilepsia de nuestro hospital. MÉTODOS: Estudiamos una cohorte histórica hospitalaria española, sin selección de pacientes por su gravedad, con 2.309 pacientes, de edad ≥ 14 años, entre enero de 2000 y junio de 2013. La identificación de los fallecidos se realizó a través de los Registros Civiles. Las causas de muerte se establecieron mediante certificados de defunción, autopsias forenses, informes de mortalidad hospitalarios, de médicos de familia y de testigos de los fallecimientos. Calculamos la incidencia y la mortalidad proporcional. RESULTADOS: Identificamos 7 casos de SUDEP definitivas (2 SUDEP-plus), uno probable y uno posible. Considerando solo los casos con autopsia, la incidencia es de 0,44/1.000 persona-año; la mortalidad proporcional es del 4,6%. Son 4 varones y 3 mujeres. La edad media es de 38,14 años. Casi todos los fallecimientos ocurrieron sin testigos, en la cama. La etiología de la epilepsia es sintomática remota o criptogénica. Menos 2 pacientes, todos tenían crisis generalizadas. Ninguno estaba en remisión. CONCLUSIONES: Pensamos que la incidencia y la mortalidad proporcional de SUDEP de nuestro estudio se asemejan a las encontradas en estudios poblacionales por el carácter escasamente seleccionado de nuestra cohorte. Los factores de riesgo para SUDEP encontrados en nuestros pacientes son concordantes con los reconocidos en la bibliografía

INTRODUCTION: Sudden unexpected death in epilepsy (SUDEP) is the leading cause of death in patients with epilepsy. Most studies concerning this issue have been conducted in central and northern European countries and the United States. We conducted an epidemiologic study of SUDEP at our hospital's epilepsy unit. METHODS: This retrospective cohort study included all epileptic patients aged ≥14 years, regardless of epilepsy severity, who were treated at the outpatient epilepsy unit of our hospital between 2000 and 2013. The study included 2,309 patients. Deceased patients were identified using civil records. The cause of death was obtained from death certificates, autopsy reports, hospital reports, general practitioner records, and witnesses of the event. We calculated the incidence and proportional mortality of SUDEP based on our data. RESULTS: We identified 7 cases of definite SUDEP (2 patients with SUDEP plus), one case of probable SUDEP, and one case of possible SUDEP. Considering only cases of definite SUDEP, incidence was estimated at 0.44 cases per 1,000 patient-years and proportional mortality at 4.6%. Mean age of patients with definite SUDEP was 38.14 years; 4 were men and 3 were women. Most deaths occurred while patients were in bed and were therefore unwitnessed. Epilepsy in these patients was either remote symptomatic or cryptogenic. All patients but 2 had generalised seizures. None of the patients was in remission. CONCLUSIONS: SUDEP incidence and proportional mortality rates in our study are similar to those reported by population studies. This may be due to the fact that we did not select patients by severity. Risk factors for SUDEP in our sample are therefore consistent with those reported in the literature

Perfil de consumo de antiepilépticos en los pacientes epilépticos del Área Oeste de Málaga / Consumption patterns of antiepileptic drugs among epileptic patients in the Western Málaga district

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Síndrome de hiperinsulinismo-hiperamoniemia y epilepsia mioclónica grave de la infancia / Hyperinsulinism and hyperammonaemia syndrome and severe myoclonic epilepsy of infancy

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[Amyotrophic lateral sclerosis and anti-CV2 antibodies. Paraneoplastic association?]. / Esclerosis lateral amiotrófica y anticuerpos anti-CV2. ¿Asociación paraneoplásica?

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a form of motor neuron disease that primarily affects upper and lower motor neurons. Its etiology is unknown, it has a progressive course and is often fatal. Very rarely, its appearance as paraneoplastic syndrome (PNS) has been described. Certain clinical patterns of motor neuron disease suggest this association. The anti-CV2 are a type of onconeuronal antibody, invariably associated to tumor and described in different PNSs as paraneoplastic encephalomyelitis, cerebellar degeneration and peripheral neuropathy. CLINICAL CASE: 29 years old male with criteria of probably ALS. Due to his atypical onset (patient's age), onconeuronal antibodies were requested, detecting anti- CV2+. After three years of follow-up and exhaustive search for tumors, with progression of the disease, there is currently no evidence of associated cancer. DISCUSSION: The study of the motor neuron/ALS disease as paraneoplastic syndrome, due to its rareness, has led to reviews in order to verify this relationship. When ALS exists, we should rule out association to tumor when the presentation is early (< 30 years) or late (> 70 years), when it is associated to other neurological symptoms/signs (sensory symptoms, ataxia, etc.), when anti-Hu antibodies or others are present and/or when there is paraproteinemia and/or pleocytosis-high protein levels in cerebral spinal fluid. We present a case of probable ALS with anti-CV2+, with no evidence of underlying cancer. After an extensive bibliographic search, we have found no description of this association. We also have no knowledge of the existence of anti-CV2 antibodies outside of the tumor context, so that we believe that our patient probably has an occult neoplasm.