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Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) INCT/MCT Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) The incidence of sudden unexpected death in epilepsy (SUDEP) has been estimated from 0.5-1.4/1,000 person-years in people with treated epilepsy, and 9/1,000 person-years in candidates for epilepsy surgery. Potential risk factors for SUDEP include: age, early onset of epilepsy, duration of epilepsy, uncontrolled seizures, seizure type and winter temperatures. The arrythmogenic side-effect of antiepileptic drugs and seizures may increase the risk of SUDEP. In this report, we describe a patient with prolonged post-ictal tachycardia in EEG video recordings with a typical case of SUDEP: a 16-year-old boy with medically intractable complex partial seizures. Magnetic resonance imaging revealed left mesial temporal sclerosis. During non-invasive video-EEG monitoring, the patient presented a post-ictal heart rate increased for five hours. Two months after video-EEG, he died from SUDEP during a tonic-clonic secondary generalized seizure. The possibility of cardiac involvement in the pathogenesis of SUDEP has been suggested by many studies. Evaluation of this patient with EEG-video monitoring, including measurement of heart rate, contributed to an identification of ictal tachycardia that may have played a role in the SUDEP. Premature mortality seems to be increased in patients with epilepsy, and cardiac abnormalities may be a possible cause of SUDEP. (Cardiol J 2011; 18, 2: 194-196) Univ Sao Paulo, Ctr Cirurgia Epilepsia, Dept Neurociencias & Ciencias Comportamento, Fac Med Ribeirao Preto,Ctr Cirurgia Epilepsia CIR, BR-14048900 Sao Paulo, Brazil Univ Fed Sao Paulo, Disciplina Neurol Expt, Escola Paulista Med UNIFESP EPM, Sao Paulo, Brazil Univ Fed Sao Paulo, Dept Fisiol, Escola Paulista Med UNIFESP EPM, Sao Paulo, Brazil Univ Fed Sao Paulo, Disciplina Neurol Expt, Escola Paulista Med UNIFESP EPM, Sao Paulo, Brazil Univ Fed Sao Paulo, Dept Fisiol, Escola Paulista Med UNIFESP EPM, Sao Paulo, Brazil Web of Science

OBJETIVO: Avaliar os resultados iniciais de uma equipe cirúrgica no controle hormonal dos adenomas hipofisários secretores. MATERIAIS E MÉTODOS: Em cinco anos, foram operados 51 adenomas secretores (31 GH, 14 ACTH, 5 prolactina, 1 TSH). O controle hormonal foi GH basal < 2,5 ng/dL, cortisol livre urinário normal, redução dos níveis de prolactina, e T3 e T4 livre normais. RESULTADOS: As taxas de controle foram 36% na acromegalia e 57% no Cushing. Dois prolactinomas (40%) normalizaram a prolactina. Os hormônios tiroidianos normalizaram no adenoma secretor de TSH. O controle do hipercortisolismo correlacionou-se com o tempo de experiência da equipe (p = 0,01). CONCLUSÃO: Nossos resultados, limitados aos primeiros anos de experiência cirúrgica, situam-se abaixo da variação reportada em grandes casuísticas com maior tempo de experiência. Ao longo do tempo, observou-se melhora progressiva nos níveis de cortisol urinário no pós-operatório inicial da doença de Cushing em função da experiência cirúrgica. OBJECTIVE: To evaluate the initial results of a surgical team in the hormonal control of secreting pituitary adenomas. MATERIALS AND METHODS: In five years 51 functioning adenomas were operated (31 GH-secreting, 14 ACTH-secreting, 5 PRL-secreting and 1 TSH-secreting). Hormonal control was defined as GH < 2,5 ng/mL, normal free-urinary cortisol, lower prolactin and normal T3 and FT4. RESULTS: Control rates were 36% in acromegaly, and 57% in Cushing's disease. Two prolactinomas normalized prolactin levels. Thyroid hormone levels were normalized in the TSH-secreting adenoma. Control of hypercortisolism was positively correlated with years of experience (p = 0.01). CONCLUSION: Our results, although restricted to the beginning of our experience, lie below the reported range of other surgical series with much longer experience. During these years, there was a significant improvement in initial post surgery urinary cortisol levels in Cushing's disease as a function of surgical experience.

BACKGROUND AND PURPOSE: Rasmussen Encephalitis (RE) is characterized by intractable epilepsy, progressive hemiparesis and unilateral hemispheric atrophy. The progression of the symptoms usually occurs within months to few years. Antiepileptic drugs are usually not effective to control disease progression and epilepsy surgery in the form of hemispheric disconnection has been considered the treatment of choice. This work describes the clinical and electrographic analyses, as well as the post-operative evolution of patients with RE. PATIENTS AND METHODS: This work includes all the patients with RE evaluated from January 1995 to January 2008 by the Ribeirão Preto Epilepsy Surgery Program (CIREP) considering demographic data, interictal and ictal electroencephalographic (EEG) findings; anatomo-pathological findings and clinical outcome. RESULTS: Twenty-five patients were evaluated, thirteen were female. Mean age of epilepsy onset was 4.4±2.0 years. There were no differences between patients with slow and fast evolution with respect to age of epilepsy onset (p=0.79), age at surgery (p=0.24), duration of epilepsy (0.06), and follow-up (p=0.40). There were no correlations between the presence of bilateral EEG abnormalities or the absence of spikes and post-operative seizure outcome (p=0.06). Twenty-three patients underwent surgery. The mean follow-up was 75.3 months. Eleven patients had total seizure control. Twelve individuals persisted with seizures consisting of mild facial jerks (6 patients), occasional hemigeneralized tonic-clonic seizures (3 patients), and frequent tonic-clonic seizures (3 patients). Mental and language impairment was observed in 15 and 12 patients, after surgery, respectively. CONCLUSIONS: This retrospective study reported the clinical and electrographic analysis, as well as the evolution of 23 patients with RE. Fourteen patients achieved satisfactory seizure control, three patients had partial response to surgery, and five patients had maintenance of the pre-operative condition. All patients with left side involvement presented with some language and cognitive disturbance. INTRODUÇÃO E OBJETIVOS: A Encefalite de Rasmussen (ER) é caracterizada por epilepsia intratável, hemiparesia progressiva e atrofia hemisférica unilateral. A progressão dos sintomas geralmente ocorre em meses ou poucos anos. As drogas antiepilépticas são usualmente ineficazes no controle da progressão da doença e o tratamento cirúrgico, com desconexão hemisférica tem sido considerado o tratamento de escolha. Neste trabalho descreveremos os achados clínicos e eletrográficos, assim como a evolução pós-operatória de pacientes com ER. PACIENTES E MÉTODOS: foram incluídos todos os pacientes com ER avaliados no período de janeiro de 1995 a janeiro de 2008, no Centro de Cirurgia de Epilepsia de Ribeirão Preto (CIREP), sendo considerados os dados demográficos, os achados do eletrencefalograma (EEG) interictal e ictal, resultado anatomo-patológico e o seguimento clínico. RESULTADOS: Vinte e cinco pacientes foram avaliados, 13 eram do sexo feminino. A idade média de início da epilepsia foi de 4.4±2.0 anos. Não houve diferenças significativas entre os pacientes com evolução lenta ou rápida considerando-se a idade de início da epilepsia (p=0,79), idade da cirurgia (p=0,24), duração da epilepsia (p=0,06) e tempo de seguimento (p=0,40). Não houve correlação entre a presença de alterações bilaterais ou ausência de descargas ao EEG e o seguimento pós-operatório (p=0,06). Vinte e três pacientes foram submetidos à cirurgia. O tempo médio de seguimento foi de 75,3 meses. Onze pacientes evoluíram com controle total das crises. Doze pacientes permaneceram com crises que consistiram de clonias faciais sutis (6 pacientes), crises tônico-clônicas hemigeneralizadas ocasionais (3 pacientes) ou crises tônico-clônicas frequentes (3 pacientes). Alterações cognitivas e de linguagem foram observadas em 15 e 12 pacientes após a cirurgia, respectivamente. CONCLUSÕES: este estudo retrospectivo relatou os achados clínicos, eletrográficos e a evolução de 23 pacientes. Controle satisfatório das crises foi obtido em 14 pacientes. Três pacientes tiveram resposta parcial com a cirurgia e cinco pacientes mantiveram o quadro pré-operatório. Todos os pacientes com envolvimento do hemisfério cerebral esquerdo evoluíram com distúrbio de linguagem e cognitivo.

Com o objetivo de determinarmos, evolutivamente, a medida da velocidade do fluxo sangüíneo nas artérias intracranianas, em recém-nascidos prematuros (RNP) normais e com hemorragia intracerebral, avaliamos - no período de junho de 1994 a março de 1999 - 73 recém-nascidos prematuros. A idade gestacional variou de 28 a 36 semanas e o peso ao nascimento variou de 720g a 2530g. O diagnóstico da hemorragia intracerebral foi realizado utilizando-se a ecografia transfontanelar (EGT). Para avaliação seqüencial da medida da velocidade do fluxo sangüíneo nas artérias intracranianas os 73 foram submetidos a EGT, com Doppler pulsátil, no 3º, 7º, 30º e 90º dias de vida. Após obtermos os valores numéricos destas velocidades determinamos o indice de resistência (IR). Ao analisarmos os valores do IR, comparando-se os 2 grupos de RNP, concluimos que os valores do IR são sempre mais elevados nos RNP normais (RNP-N) que nos RNP com hemorragia intracerebral (RNP-HIC); que tanto no grupo de RNP normais quanto no grupo de RNP com hemorragia intracerebral os valores do IR decrescem significativamente com o acréscimo da idade dos neonatos. Analisando-se ainda, comparativamente, os valores do IR nos RNP com hemorragia intracerebral, em seus diversos graus, observamos não haver, evolutivamente, diferença estatisticamente significante. Analisando-se também , comparativamente, os valores do IR nos RNP com hemorragia intracerebral localizada no hemisfério cerebral direito ou esquerdo concluimos não haver diferença estatisticamente significante entre os valores do IR obtidos das artérias localizadas no hemisfério cerebral acometido comparados aos valores do IR obtidos do hemisfério cerebral não afetado. In order to ascertain the blood flow velocities in the intracranial arteries we evaluated 73 preterm neonates during a period ranging from June 1994 to March 1999. These preterm infants were divided in two separate groups, 18 healthy and 55 with intracranial hemorrhage. They were subjected to sequencial measurements of blood flow velocities in the intracranial arteries. The gestational age of the whole group varied from 28 to 36 weeks and birth weights between 720 and 2530 g. The diagnosis of the intracerebral hemorrhages in these preterm neonates were done using high resolution gray and color scale transfontanellar ultrasonography brain scans . The ultrasound evaluations were performed in the initial 3rd, 7th and 14th day of life. The 73 preterm infants were evaluated with sequencial measurements of blood flow velocity in the intracranial arteries using the Doppler technique through the anterior fontanelle. Doppler evaluation of the cerebral vessels were performed on days 3, 7, 30 and 90 of life. These evaluations were performed in the six intracranial arteries, meaning: right and left anterior and middle cerebral arteries and right and left internal carotid arteries. Doppler recordings were made using Duplex Color-Doppler system, pulse echo probe of 3,5; 5,0 and 7,5 MHz. Measuring the blood flow velocity in the cerebral arteries we obtained a maximum systolic velocity and end diastolic velocity with a rate in meters per second (m/s) for each cardiac cycle. After obtaining these numerical values for these velocities we obtained the resistance index (RI) or Pourcelot index. In a progressive way as the resistance index (RI) values were being obtained in each stage of this study they were also being checked in the cerebral arteries of healthy preterm infants and infants with intracranial hemorrhages. We also analyzed in a comparative method the values of the resistive index between the two groups of preterm infants observing their behaviour.The results obtained when comparing the RI values in the various arteries during the different stages of the study permitted us to conclude that the RI values of healthy pre-term infants were always larger than the RI values of pre-term infants with intracranial hemorrhage. We also conclude that the RI values in the healthy pre-term infants and in the pre-term infants with intracranial hemorrhages decreased progressively with the increasing age of neonates.