Potential of essential fatty acid deficiency with extremely low fat diet in lipoprotein lipase deficiency during pregnancy: A case report

BACKGROUND: Pregnancy in patients with lipoprotein lipase deficiency is associated with high risk of maternal pancreatitis and fetal death. A very low fat diet (< 10% of calories) is the primary treatment modality for the prevention of acute pancreatitis, a rare but potentially serious complication of severe hypertriglyceridemia. Since pregnancy can exacerbate hypertriglyceridemia in the genetic absence of lipoprotein lipase, a further reduction of dietary fat intake to < 1–2% of total caloric intake may be required during the pregnancy, along with the administration of a fibrate. It is uncertain if essential fatty acid deficiency will develop in the mother and fetus with this extremely low fat diet, or whether fibrates will cross the placenta and concentrate in the fetus. CASE PRESENTATION: A 23 year-old gravida 1 woman with primary lipoprotein lipase deficiency was seen at 7 weeks of gestation in the Lipid Clinic for management of severe hypertriglyceridemia that had worsened with pregnancy. While on her habitual fat intake of 10% of total calories, her pregnancy resulted in an exacerbation of the hypertriglyceridemia, which prompted further restriction of fat intake to < 2% of total calories, as well as administration of gemfibrozil at a lower than average dose. The level of gemfibrozil, as the active metabolite, in the venous and arterial fetal cord blood was within the expected therapeutic range for adults. The clinical signs and a biomarker of essential fatty acid deficiency, namely the ratio of 20:3 [n-9] to 20:4 [n-6] fatty acids, were closely monitored throughout her pregnancy. Despite her extremely low fat diet, the levels of essential fatty acids measured in the mother and in the fetal blood immediately postpartum were normal. Normal essential fatty acid levels may have been achieved by the topical application of sunflower oil. CONCLUSIONS: An extremely low fat diet in combination with topical sunflower oil and gemfibrozil administration was safely implemented in pregnancy associated with the severe hypertriglyceridemia of lipoprotein lipase deficiency

Bradicardia como manifestação epiléptica em epilepsia temporal: relato de caso Bradycardia during temporal lobe seizure: case report

Descrevemos um caso de arritmia cardíaca como manifestação epiléptica. A monitorização video-eletrencefalográfica de uma paciente com 34 anos de idade que apresentava episódios de perda de consciência permitiu a detecção de períodos de assistolia como principal manifestação clínica, exigindo a implantação de marca-passo. O registro eletrencefalográfico concomitante mostrou atividade rítmica a 6-7 Hz de projeção na região temporal esquerda. A ressonância magnética mostrou lesão expansiva no giro para-hipocampal esquerdo. Alterações do ritmo cardíaco como taquicardia sinusal são frequentes em crises epilépticas. A descrição de bradicardia e/ou assistolia é rara. As conexões das estruturas mesiais temporais com estruturas profundas como o hipotálamo devem ser responsáveis pelas manifestações vegetativas durante crises epilépticas temporais.<br>We describe a patient who had cardiac arrhythmia as epileptic manifestation. In a 34-year-old woman who had many episodes of loss of consciousness, the simultaneous ECG and video-EEG monitoring recorded bradycardia with a short episode of asystolia (4 seconds) and left temporal rhythmic teta activity on EEG. MRI showed a small mass lesion in the left parahippocampal gyrus. Alterations in cardiac rhythm have been reported in epileptic seizures and taquycardia is the most common finding associated with them; bradyarrhythmia during seizures was uncommon. Many interconnections among insular cortex, limbic system and hypothalamus, may be responsible for vegetative manifestations in temporal lobe epilepsy

The mistery of Gustave Flaubert's death: could sudden unexpected death in epilepsy be part of the context? O mistério da morte de Gustave Flaubert: pode a morte súbita em epilepsia fazer parte o contexto?

Epilepsy is the most common serious neurological condition and sudden unexpected death in epilepsy (SUDEP) is the most important direct epilepsy-related cause of death. Information concerning risk factors for SUDEP is conflicting, but high seizure frequency is a potential risk factor. Additionally, potential pathomechanisms for SUDEP are unknown, but it is very probable that cardiac arrhythmias during and between seizures or transmission of epileptic activity to the heart via the autonomic nervous system potentially play a role. More than two decades ago, temporal lobe epilepsy was suggested as having been the ''nervous disease'' of Gustave Flaubert, one of the most important French novelists. In these lines, as the circumstances of his death were the subject of fabulous and mysterious speculations, we postulated in this paper that Falubert' death could be due SUDEP phenomenon.<br>A epilepsia é a condição neurológica crônica grave mais comum e a morte súbita em epilepsia (SUDEP) é a mais importante causa de morte diretamente relacionada à epilepsia. Informações sobre fatores de risco para SUDEP são conflitantes, porém, a alta freqüência de crises epilépticas é um fator de risco em potencial. Além disso, os mecanismos causais para SUDEP ainda não estão conhecidos, mas é muito provável que arritmias cardíacas durante e entre as crises epilépticas ou a transmissão da atividade epiléptica para o coração via sistema nervoso autônomo desempenhem um importante papel. Mais de duas décadas atrás, foi proposto que a "doença nervosa" de Gustave Flaubert, um dos mais importantes novelistas franceses, era epilepsia do lobo temporal. Nesse sentido, como a morte de Gustave Flaubert ainda seja motivo de misteriosa especulação, nosso artigo propõe que a mesma poderia estar relacionada ao fenômeno de SUDEP

Dynamic priority allocation via restless bandit marginal productivity indices

Priority allocation, Stochastic scheduling, Index policies, Restless bandits, Marginal productivity index, Indexability, Dynamic control of queues, Control by price, 90B36, 90C40, 90B05, 90B22, 90B18,