Manejo del estado epiléptico en niños: encuesta en hospitales pediátricos de la Ciudad Autónoma de Buenos Aires / Management of status epilepticus in childhood: a survey conducted at pediatric hospitals in the City of Buenos Aires

Introducción. El estado epiléptico constituye la emergencia neurológica más frecuente. Si bien la mortalidad en niños es baja, su morbilidad puede superar el 20 %. Objetivo. Conocer las pautas de manejo del estado epiléptico referidas por médicos pediatras que atienden esta patología en forma habitual. Población y métodos. Estudio descriptivo, transversal, basado en una encuesta a médicos de tres hospitales pediátricos monovalentes de gestión pública de la Ciudad Autónoma de Buenos Aires. Resultados. Se administraron 292 encuestas (la tasa de respuesta completa alcanzó el 86 %); el 77 % se administró a pediatras y el 16 %, a especialistas en cuidados intensivos. Un 47 % de los participantes refiere indicar la primera benzodiacepina en el tiempo correcto; el 56 % utilizar diazepam intrarrectal en ausencia de un acceso intravenoso; el 95 % elige lorazepam como benzodiacepina inicial en caso de contar con acceso intravenoso; el 58 % refiere iniciar la etapa de fármacos de segunda línea en tiempo adecuado; el 84 % opta por fenitoína como fármaco inicial de segunda línea, un 33 % no cronometra el tiempo durante el tratamiento. La adherencia global a las recomendaciones internacionales fue del 17 %. Conclusiones. Nuestro estudio advierte una baja adherencia referida de los pediatras a las guías internacionales, en particular en las decisiones tiempo-dependientes. También se observó mayor heterogeneidad en las conductas terapéuticas a medida que se avanza en el algoritmo de tratamiento.

Introduction. Status epilepticus is the most common neurological emergency. Although mortality in children is low, morbidity may exceed 20%. Objective. To evaluate the management of status epilepticus by pediatricians who usually treat this condition. Population and methods. Descriptive, cross-sectional study based on a survey administered to physicians from 3 pediatric hospitals in the City of Buenos Aires. Results. A total of 292 surveys were administered (complete response rate as high as 86%); 77% were administered to pediatricians and 16% to intensive care specialists. Forty-seven percent of the participants reported that they administer the first dose of a benzodiazepine within the correct timeframe; 56% use intrarectal diazepam when intravenous access is not available; 95% choose lorazepam as the initial benzodiazepine if an intravenous access is available; 58% initiate the administration of a second-line drug within the correct timeframe; 84% administer phenytoin as the first-choice, second-line drug; and 33% do not measure treatment time. Overall adherence to international recommendations was 17%. Conclusions. Our study highlights poor adherence of pediatricians to international guidelines, particularly in time-dependent decisions. Greater heterogeneity was observed in treatment approaches as the treatment algorithm progressed.

Estado de mal epiléptico / Status epilepticus

El estado de mal epiléptico (EME) es la emergencia más frecuente en la neuropediatría. Es el resultado de un fallo en los mecanismos responsables de terminar la convulsión o de la iniciación de mecanismos que provocan una convulsión anormalmente prolongada. Esta definición se relaciona con el momento de iniciar el tratamiento. En general, el primer punto de tiempo o t1, es el momento cuando el tratamiento debería comenzarse, que es a los 5 minutos para las convulsiones tónico-clónicas generalizadas y a los 10 minutos para las focales con o sin compromiso de la conciencia. El segundo punto de tiempo o t2 marca el momento en el cual el daño neuronal o de las redes neuronales puede comenzar e indica que el EME debería ser controlado, que para los casos de mal tónico-clónico generalizados debe ser de 30 minutos. Todos los protocolos de tratamiento diferencian estadios en donde se utilizan diferentes fármacos: temprano o 1, establecido o 2, refractario o 3, súper-refractario o 4; y enfatizan el rápido reconocimiento y tratamiento de la actividad epiléptica persistente en cada estadio con el objetivo de reducir la morbimortalidad y las secuelas a largo plazo (después de t2).

Status epilepticus (SE) is one of the most common neurologic emergencies in pediatrics. It is a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which leads to abnormally, prolonged seizures. This definition provides a good guidance, when emergency treatment must be considered. In general, time point t1 is the time when treatment should be started, which is at 5 minutes for generalized tonic-clonic seizures, and at 10 min for focal seizures with or without impairment of consciousness. Time-point t2 marks the time at which neuronal damage or self-perpetuating alteration of neuronal networks may begin and indicates that SE should be controlled latest by that time; 30 min in case of generalized tonic-clonic seizures. All treatment protocols recognize a staged approach to treatment with different drugs used in early (stage I), established (stage II), refractory (stage III) and super-refractory SE (stage IV); and emphasize prompt recognition and treatment of persisting seizure activity at each stage aiming to reduce morbidity, mortality, and long-term consequences of status epilepticus (beyond t2).

Catatonia ictal en el Servicio de Urgencia: una rara forma de presentación del estado epiléptico no convulsivo / Ictal catatonia presentation as a non-convulsive status epilepticus: a case report

The differential diagnosis of non-convulsive status epilepticus (NCSE) is often complex due to a wide clinical variability of its presentation, including psychiatric manifestations. We report a 68 years old male with a history of depression treated with venlafaxine, mirtazapine, quetiapine and risperidone, presenting in the emergency room with confusion and generalized rigidity. A brain CT scan did not show lesions. A neuroleptic syndrome was initially suspected. At the third day the obtundation worsened and an electroencephalogram (EEG) was performed, which showed epileptiform abnormalities. Treatment with valproic acid resulted in disappearance of such abnormalities. After three weeks of mechanical ventilation, the patient was extubated and remained lucid and partially orientated in time and space.

Estatus epiléptico no convulsivo / Nonconvulsive status epilepticus

El estatus epiléptico no convulsivo (EENC) es una situación clínica en la cual los pacientes presentan un grado variable de alteración del nivel de conciencia o de su estado mental basal, asociado a descargas o paroxismos electrográficos de forma continua y sin presentar movimientos convulsivos, 0 estos son muy sutiles. Es una entidad de relativamente reciente descripción, que va adquiriendo cada vez una mayor importancia entre las causas de deterioro del nivel de conciencia, siendo en parte, probablemente infradiagnosticada, a lo que contribuye el desconocimiento del clínico, la necesidad de realizar un electroencefalograma (EEG) y la falta de unos criterios diagnósticos consensuados. El objetivo de este artículo es hacer una revisión del EENC haciendo un especial énfasis en aquellas situaciones en las cuales el clínico se pueda enfrentar con dicha patología...

Nonconvulsive status epilepticus (NCSE) is a situation in which patients have a varying degree of altered level of consciousness or mental condition associated with continuously electrographic discharges or paroxysms, without present convulsive movements or that they are very subtle. It is an entity of relatively recent description, which becomes more important as cause of altered level of consciousness, and it was thought likely being underdiagnosed, contributing the ignorance of clinician, the need for an electroencephalogram and lack of consensus diagnostic criteria. The aim of this article is to review the non-convulsive status epilepticus with special emphasis on those situations in which the clinician may face with this pathology...

¿Crisis febriles complejas o síndrome de Dravet?: Descripción de 3 casos clínicos / Complex febrile Seizures or Dravet syndrome?: Description of 3 case reports

Introduction: Dravet syndrome (DS) is one of the most intractable forms of epilepsy that begins in infancy. This syndrome is characterized by beginning with complex febrile seizures (FS) in a healthy infant and progresses to refractory epilepsy with psychomotor regression. The detection of a SCN1A mutation encoding the sodium channel can confirm the diagnosis. Objective: To report 3 confirmed cases of genetically DS. Case reports: We describe 3 girls diagnosed with complex FS that started when they were between 2 and 7 months old. FS were frequent, hemi generalized and myoclonic associated with recurrent febrile status epilepticus (SE). Despite FS and SE recurrence, the psychomotor development, electrophysiological studies and magnetic resonance imaging (MRI) of the brain were normal. After a year, they developed afebrile seizures progressing to refractory epilepsy with developmental regression. A molecular study detected SCN1A mutation confirming DS. The specific antiepileptic treatment and prevention of febrile episodes allowed partial control of epilepsy with some recovery of psychomotor skills. Conclusions: The high frequency complex FS associated with recurrent SE in a previously healthy infant should alert about the possibility of DS. Molecular diagnostics helps us to establish a drugs and non-drug therapies treatment, as well as long-term prognosis and genetic counseling.

Introducción: El Síndrome de Dravet (SD) es una de las formas más intratables de epilepsia que debuta en lactantes con convulsiones febriles (CF) complejas recurrentes que evolucionan posteriormente a epilepsia refractaria con regresión psicomotora. La detección de una mutación del canal de Sodio (SCN1A) permite certificar el diagnóstico. Objetivo: Reportar 3 casos de SD confirmados genéticamente. Casos clínicos: Se describen 3 niñas con diagnóstico de CF complejas iniciadas entre los 2 y 7 meses de edad. Las CF eran frecuentes, hemigeneralizadas, mioclónicas asociadas a status epilepticus (SE) febriles recurrentes. A pesar de la recurrencia de CF y SE, tanto el desarrollo psicomotor como los estudios electrofisiológicos y la resonancia magnética (RM) cerebral, fueron normales. Posterior al año iniciaron crisis afebriles que evolucionaron a epilepsia refractaria con regresión del desarrollo. El estudio molecular detectó la mutación SCN1A confirmando SD. El tratamiento antiepiléptico específico y la prevención de cuadros febriles permitieron un control parcial de la epilepsia con recuperación de algunas habilidades psicomotoras. Conclusiones: La alta frecuencia de CF complejas asociadas a SE recurrentes en un lactante previamente sano, debe alertar sobre la posibilidad de un SD. El diagnóstico molecular nos permite instaurar un tratamiento antiepiléptico y terapias no farmacológicas además de un pronóstico a largo plazo y consejería genética.

Estado epiléptico super-refractario resuelto con hipotermia terapéutica / Super-refractory status epilepticus solved with therapeutic hypothermia

El estado epiléptico refractario es definido como la crisis convulsiva que persiste a pesar del tratamiento adecuado con benzodiacepinas y otras drogas anticonvulsivantes, y el estado epiléptico super-refractario, como aquel que persiste luego del uso de agentes anestésicos por más de 24 horas. Toda forma de estado epiléptico debe ser tratada rápidamente para disminuir la morbimortalidad; sin embargo, hay escasa evidencia para escoger un tratamiento específico sobre otros. La hipotermia puede resultar un tratamiento seguro y útil en el manejo de estos pacientes. Se reporta el caso de un paciente de 14 años con estado epiléptico super-refractario, tratado con hipotermia terapéutica en forma exitosa...

importance of imaging for status epilepticus patients to rule out fractures - a case report

Status epilepticus could be the first presentation of underlying epilepsy or may occur in patients with known epilepsy. The literature has reported many cases of seizures severe enough to cause dislocations or fracture-dislocations of the shoulder or hip joints and death from hemorrhaging. Although shoulder and hip injuries due to seizures are rare, such events are known to occur, especially in patients with a history of osteoporosis or other risk factors for fractures. We describe a case of a 35-year-old healthy male with no history of osteoporosis or reduced bone density. His first presentation of epilepsy manifested with status epilepticus. This prolonged seizure resulted in bilateral acetabular fracture with left proximal humerus fracture without any trauma or falls. Although fractures associated with seizures have been reported in the literature, dislocation and fractures from seizures involving both the hip and the shoulder joints have only been described in a few cases. Two of these cases involved patients with known epilepsy, osteoporosis and osteomalacia. To the best of our knowledge, this is the first described case of a patient with bilateral hip and one shoulder fracture that had no previous history of epilepsy or seizures, except for febrile seizure as a child, which did not requiring any treatment. He was also not taking any antiepileptic medications, which are known to reduce bone density. This case shows the severity of status epilepticus and the importance of screening for fractures in patients, especially in those with risk factors for reduced bone density

Refractory nonconvulsive status epilepticus in coma: analysis of the evolution of ictal patterns / Estado de mal epiléptico não convulsivo refratário no coma: análise da evolução dos padrões ictais

OBJECTIVE: Nonconvulsive status epilepticus (NCSE) is currently considered as one of the most frequent types of status epilepticus (SE). The objective of the present study was to identify the natural history of the electrographical evolution of refractory NCSE and to establish the relationship between ictal patterns and prognosis. METHODS: We analyzed, retrospectively, 14 patients with loss of consciousness and NCSE. The ictal patterns were classified as discrete seizures (DS), merging seizures (MS), continuous ictal discharges (CID), continuous ictal discharges with flat periods (CID-F), and periodic lateralized epileptiform discharges (PLEDs). RESULTS: The ictal patterns were DS (n=7; 50.0%), PLEDs (n=3; 1.4%), CID (n=2; 14.3%), MS (n=1; 7.1%), and CID-F (n=1; 7.1%). CONCLUSIONS: NCSE electrographic findings are heterogeneous and do not follow a stereotyped sequence. PLEDs were related to a higher probability of neurological morbidity and mortality.

OBJETIVO: Estado de mal epiléptico não convulsivo (EMENC) é atualmente considerado uma das formas mais frequentes de estado de mal epiléptico. O objetivo deste estudo foi identificar a história natural da evolução eletrográfica do EMENC refratário, bem como estabelecer relações entre padrões ictais e o prognóstico. MÉTODOS: Foram analizados, retrospectivamente, 14 pacientes com comprometimento da consciência e EMENC. Os padrões ictais foram classificados em crises isoladas (CI), crises subintrantes (CS), descarga ictal contínua (DIC), descarga ictal contínua com períodos de atenuação (DIC-A) e descargas epileptiformes periódicas lateralizadas (PLEDs). RESULTADOS: Os padrões ictais observados foram CI (n=7; 50,0%), PLEDs (n=3; 1,4%), DIC (n=2; 14,3%), CS (n=1; 7,1%) e DIC-A (n=1; 7,1%). CONCLUSÕES: Achados eletrográficos no EMENC refratário são heterogêneos e não obedecem a uma sequência estereotipada. As PLEDs estão associadas à maior probabilidade de morbidade e mortalidade neurológica.

The impact of EEG in the diagnosis and management of patients with acute impairment of consciousness / Impacto do EEG no diagnóstico e conduta dos pacientes com alteração aguda do estado da consciência

OBJECTIVES: To assess the frequency of electroencephalogram (EEG) requests in the emergency room (ER) and intensive care unit (ICU) for patients with impairment of consciousness (IC) and its impact in the diagnosis and management. METHODS: We followed patients who underwent routine EEG from ER and ICU with IC until discharge or death. RESULTS: During the study, 1679 EEGs were performed, with 149 (8.9 percent) from ER and ICU. We included 65 patients and 94 EEGs to analyze. Epileptiform activity was present in 42 (44.7 percent). EEG results changed clinical management in 72.2 percent of patients. The main reason for EEG requisition was unexplained IC, representing 36.3 percent of all EEGs analyzed. Eleven (33 percent) of these had epileptiform activity. CONCLUSION: EEG is underused in the acute setting. The frequency of epileptiform activity was high in patients with unexplained IC. EEG was helpful in confirming or ruling out the suspected initial diagnosis and changing medical management in 72 percent of patients.

OBJETIVO: Avaliar a frequência de exames de eletroencefalograma (EEG) solicitados no pronto-socorro (PS) e na unidade de terapia intensiva (UTI) em pacientes com rebaixamento do nível de consciência, bem como seu impacto no diagnóstico e na conduta. MÉTODOS: Acompanhamos pacientes submetidos ao EEG do PS e da UTI com rebaixamento do nível de consciência até a alta ou óbito. RESULTADOS: Realizamos 1679 EEGs no período de estudo; destes, 149 (8,9 por cento) foram solicitados no PS e na UTI. Incluímos 65 pacientes e 94 EEGs para análise; destes, 42 (44,7 por cento) apresentavam atividade epileptiforme. O EEG mudou a conduta em 72 por cento dos pacientes. A razão principal para solicitação do EEG foi rebaixamento do nível de consciência de origem inexplicável (36,3 por cento dos EEGs). Destes, 33 por cento tinham atividade epileptiforme. CONCLUSÃO: Embora o EEG seja pouco usado em condições agudas, a frequência de atividade epileptiforme foi alta nos pacientes com rebaixamento do nível de consciência de origem inexplicável. O EEG foi decisivo para o esclarecimento diagnóstico e implicou mudança da conduta em 72 por cento dos pacientes.

An Overlooked Cause of Impaired Consciousness in a Hemodialysis Patient

No abstract available.

Estado epiléptico no convulsivo infantil / Non convulsive status epilepticus in children

Introduction: Status Epilepticus (SE) is a medical emergency with high morbimortality. Non-convulsive Status Epilepticus (NCSE) is defined as electroencephalographic crises in the absence of clinical motor symptoms. Objective: A restrospective study of NCSE in our population. Patients and Methods: Charts of 30 patients in a Child Neurology Clinic seen between December 1999 and June 2008 were reviewed. According to the characteristics of the episode, they were classified as a) Typical absence, b) Atypical absence, c) Partial Complex episode. Results: Of the 30 patients, 15 (50%) were males. Median age was 46 months. Clinically, 63% of the children suffered from complex partial seizures, 30% atypical absences, 7% typical absences. Cryptogenic SE was most frequent (47%), with a mortality of 3%. Discussion: Pediatric patients have a higher risk of NCSE than adults do. Most patients already carried a diagnosis of Epilepsy, cerebral infarcts were the second most frequent cause of NCSE. They should all be monitored through EEGs. Neuroimages are of great value since outcome depends on the etiology. In summary, clinical suspicion, a history of epilepsy supported by an EEG (best if prolonged) allow early diagnosis and treatment.

Introducción: El Estado Epiléptico (EE) es una emergencia médica con alta morbimortalidad. El Estado Epiléptico No Convulsivo (EENC) es definido por la presencia de crisis electroencefalograficas en ausencia de crisis motoras clínicas. Objetivo: Realizar una descripción retrospectiva de los EENC que se presentaron en la población pediátrica atendida en nuestro centro. Pacientes y Método: Se revisaron los registros de 30 pacientes controlados neurología infantil que presentaron EENC entre diciembre de 1999 y junio de 2008. Según el tipo de crisis se clasificó en a) EENC ausencia típica; b) EENC ausencias atípicas y c) EENC parcial complejo. Según etiología se uso la clasificación de Hauser modificada. Resultados: Se analizaron 30 pacientes. Quince (50%) varones. La mediana de edad fue 46 meses. 63% fueron EENC parcial complejo, 30% EENC ausencias atípicas y 7% EENC ausencias típicas. El EE criptogénico fue el más frecuente (47%). La mortalidad fue de 3%. Discusión: Los pacientes pediátricos tienen mayor riesgo de EENC en relación a los adultos. La gran mayoría de los pacientes con EENC ya tenían el diagnóstico de epilepsia. Los infartos cerebrales fueron la segunda causa de EENC, estos deben ser monitorizados con EEG ante la sospecha de EENC. Las neuroimágenes para precisar el diagnóstico son de gran valor dado que el pronóstico de los pacientes con EENC depende de la etiología subyacente. En conclusión, la sospecha clínica, el antecedente de epilepsia y el apoyo con un EEG, especialmente prolongado, permite el diagnóstico oportuno y el tratamiento precoz.

Isoniazid Toxicity Presenting As Status Epilepticus and Severe Metabolic Acidosis.

Isoniazid (INH) is an integral component of treatment of tuberculosis. An acute overdose is potentially fatal and is characterized by the clinical triad of repetitive seizures unresponsive to the usual anticonvulsants, metabolic acidosis with a high anion gap and coma. The diagnosis of INH overdose should be considered in any patient who presents to emergency medical services (EMS) with the triad. We report a patient presenting with multiple generalised tonic clonic (GTC) convulsions with severe metabolic acidosis as a manifestation of INH toxicity. ©

Estado epiléptico en pediatría / Status epilepticus in childhood

El estado epiléptico es la forma de presentación de crisis de epilepsia más alarmante y severa. Es un cuadro clínico de alta morbimortalidad con un alto riesgo de daño cerebral entre los pacientes que sobreviven, cuyo pronóstico está determinado por la edad de presentación, duración de la crisis y etiología subyacente.

Estado epileptico no-convulsivo consideraciones sobre fisiopatología y diagnóstico / Non-convulsive status epilepticus: pathophysiology and diagnosis

El estado epiléptico no convulsivo es una condición médica donde se asocia actividad epiléptica ictal prolongada, con síntomas clínicos no convulsivos y que responde a anticonvulsivantes. Esta definición, aunque amplia, permite incorporar diferentes situaciones especialmente en los niños, que son edad dependiente. En diversos modelos animales se ha comprobado que la presencia de actividad ictal continua no es inocua y en series clínicas, esta se asocia a mayor morbimortalidad. Para el diagnóstico y control de tratamiento es fundamental alto grado de sospecha y contar con monitoreo electroencefalográfico continuo.

Non convulsive status epilepticus is a term used to denote a range of conditions in wich electrographic seizure activity is prolonged, results in nonconvulsive clinical symptoms and response to antiepileptic therapy This wide definitions permits to incorporate age dependent syndrome in the child. The continuous epileptic activity is not innocuous in animal models and clinical trails are associated an in crease of acute morbidity and mortality in nonconvulsive status epílepticus. The continuous EEG monitoring is the excellent methods for the diagnosis the Non convulsive status epílepticus, associated with high grade of clinical suspect.

Estado epiléptico en niños: Actualización / Status epilepticus in children: Update

El estado epiléptico es la emergencia médica neurológica más frecuente en la infancia que requiere un diagnóstico y tratamiento inmediato y agresivo. La morbimortalidad está relacionada directamente a la duración del estado epiléptico aunque el pronóstico final depende sobre todo de la causa que lo originó. Se revisan conceptos, características clínicas y tratamiento del estado epiléptico convulsivo, estado epiléptico no convulsivo y del estado epiléptico refractario poniendo énfasis en la necesidad de contar con un monitoreo electroencefalográfico continuo para el manejo adecuado de esta emergencia médica.

Status epilepticus is the most common medical neurological emergency during the infancy that needs an immediately and aggressive diagnostic and treatment. The morbidity and mortality are depending of the duration of the status epilepticus although the final outcome is more related to etiology of the status epilepticus. This review addresses some of the current issues concerning definitions, clinical characteristics and the treatment of the convulsive status epilepticus, non convulsive status epilepticus and refractory status epilepticus. The importance of continuous electroencephalographic monitoring for the adequate management of this medical emergency is emphasized.

Vômitos e depressão de consciência como manifestação de estado de mal epileptico em criança: relato de caso / Vomiting and consciousness depression as manifestation of status epilepticus in child: case report / Vomitos y depression de la consciencia como manifestación de estado de mal convulsivo en niño: relato de caso

Objetivo: descrever um caso de estado de mal epiléptico não convulsivo com sinais e sintomas de distúrbio autonômico, denominado síndromede Panayitopoulos, para facilitar o reconhecimento pelo pediatra. Relato do caso: um menino de 8 anos de idade, foi atendido no pronto socorro com história de dois anos, durante os quais apresentou 5 crises, que duravam cerca de 30 min, nas quais a criança vomitava, ficava pálida, confusa e não responsiva a estímulos, recuperando-se completamente nas horas seguintes.Os antecedentes gestacionais, de parto e do desenvolvimento neuropsicomotor eram normais, assim como o exame clínico e neurológico. A tomografia computadorizada de crânio teve resultado normal, assim como os exames para erro inato do metabolismo. O eletroencefalograma (EEG) mostrou atividade epileptiforme focal naregião occipito-temporal posterior esquerda. Foi diagnosticada a síndrome de Panayitopoulos, reconhecida na forma de estado de mal epilépticoautonômico recorrente. A criança foi medicada com carbamazepina durante 12 meses e, desde o término há 18 meses permanece sem crises. Conclusão:os pediatras devem suspeitar de SP nascrianças com manifestações autonômicas súbitas, prolongadas ou recorrentes, com recuperaçãocompleta entre os episódios.

Objective: to describe a case of non-convulsive epilepsy with signs and symptoms of autonomous disorder,called Panayitopoulos syndrome, in order to facilitate its recognition by the pediatrician. Case report: a 8 year-old boy come to the emergency care unit with a history of two years when he presented 5 crises, each one lasting approximately 30 minutes, with vomiting, pallor, mental confusion no responsive to stimulations, with full recovering in the following hours. The gestational background, delivery and neuropsychomotor development were normal, as well as the clinic andneurological exame. The skull computerized tomography showed a normal result, as well as the tests for innate metabolism errors. The electroencephalogram (EEG) showed focal epileptiform activity in the leftposterior occipital-temporal region. The Panayitopoulos syndrome (SP) was diagnosed, recognized in the form of status epilepticus with recurring autonomic epilepsy. The child was medicated with carbamazepine for 12 months and remains without crises ever since, for 18 months. Conclusion: pediatricians should suspect of SP in children with sudden, prolonged or recurring autonomic manifestations, with full recovery between episodes. The confirmation should be obtainedthrough EEG.

Objetivo: describir um caso de estado de mal epiléptico sin convulsiones, com señales y symptomas de distúrbio autonômico, denominado de síndromede Panayiotopoulos, para facilitar lo reconocimiento por parte del pediatra. Relato del caso: un niño de 8 años de edad fue atendido in una sala de emergência com historia de dos años en que presentó 5 crises con cerca de 30 minutos de duración, com vômitos, palidez, confusión mental y sonolência,recuperando-se al completo durante lãs horas seguientes. Los antecedentes gestacionales y de lo desarrollo neuropsicomotor eram normales, asique lo examen clinico y neurológico. La tomografia de craneo resulto normal, asi como los exames de erros inatos del metabolismo. El electroencelalograma(EEG) monstró actividad epileptica focalna area occipito-temporal posterior isquierda. Fue diagnosticada la síndrome de Panayiotopoulos en la forma de status epileticus recorrente. El niñofue medicado con carbamazepina durante un año, sin nuevas crises durante 18 meses. Conclusión: los pediatras deben sospechar de SP en los casos con manifestaciones clinicas autonômicas subitas, prolongadas o recorrentes, y recuperación entre losepisódios. La comprovación se hace con el EEG.

MR imaging of cerebral and cerebellar hypervascularity associated with status epilepticus: case report.

A 20 year old male presented to the emergency department with generalized tonic clonic convulsions and up rolling of eye balls. He had history of seizure disorder for three years and on regular medical treatment and is compliant to medication. A non-contrast CT scan of the head was only done on 14th day of admission which showed hypodense areas in the right and left cerebellar hemisphere. MR imaging was performed four days later revealed high signal intensity in the both cerebellar hemispheres, both external capsules, both occipital and right parietal regions on fluid-attenuated inversion recovery (FLAIR). The post contrast MR imaging revealed diffuse cerebral and cerebellar hypervascularity in the similar region. This change of diffuse hypervascularity of both cerebral and cerebellar associated with seizure activity on post-contrast magnetic resonance imaging (MRI) has not been reported in any literature.

Update on status epilepticus.

Status epilepticus is a medical emergency and is secondary to a range of insults to the central nervous system. The authors reviewed the current management of this disorder in light of the latest developments from recent trials and guidelines. Important principles in management include early recognition of status epilepticus, identification of the underlying cause and prompt treatment to terminate seizures and reduce complications. The role of electroencephalographic monitoring and different treatment regimens are examined.