Fetuses with congenital heart disease (CHD) have circulatory abnormalities that may compromise cerebral oxygen delivery. We believe that some CHD fetuses with decreased cerebral oxygen supply have autoregulation of blood flow that enhances cerebral perfusion (brain sparing). We hypothesize that cerebral autoregulation occurs in CHD fetuses, and the degree of autoregulation is dependent on the specific CHD and correlates with intrauterine head circumferences. CHD fetuses were compared to normal fetuses. Data included cardiac diagnosis, cerebral and umbilical artery Doppler, head circumference, weight, and gestational age. The cerebral-to-placental resistance ratio (CPR) was assessed as a measure of cerebral autoregulation. CPR = cerebral/umbilical resistance index (RI) and RI = systolic-diastolic/systolic velocity (normal CPR > 1). CPR > 1 was found in 95% of normal vs 44% of CHD fetuses. The incidence of CPR < 1 was greatest in hypoplastic left or right heart fetuses. Compared to normal, cerebral RI was decreased in CHD fetuses. The CPR vs gestational age relationship, and the relationship among weight, head circumference, and CPR differed across normal and CHD fetuses. Fetuses > 2 kg with CHD and a CPR < 1 had smaller head circumferences than normal. Brain sparing occurs in CHD fetuses. Fetuses with single ventricular physiology are most affected. Inadequate cerebral flow in CHD fetuses, despite autoregulation, may alter brain growth.

Abstract-Objective:To review evidence on the assessment of the child with status epilepticus (SE). Methods: Relevant literature were reviewed, abstracted, and classified. When data were missing, a minimum diagnostic yield was calculated. Recommendations were based on a four-tiered scheme of evidence classification. Results: Laboratory studies (Na ϩϩ or other electrolytes, Ca ϩϩ , glucose) were abnormal in approximately 6% and are generally ordered as routine practice. When blood or spinal fluid cultures were done on these children, blood cultures were abnormal in at least 2.5% and a CNS infection was found in at least 12.8%. When antiepileptic drug (AED) levels were ordered in known epileptic children already taking AEDs, the levels were low in 32%. A total of 3.6% of children had evidence of ingestion. When studies for inborn errors of metabolism were done, an abnormality was found in 4.2%. Epileptiform abnormalities occurred in 43% of EEGs of children with SE and helped determine the nature and location of precipitating electroconvulsive events (8% generalized, 16% focal, and 19% both). Abnormalities on neuroimaging studies that may explain the etiology of SE were found in at least 8% of children. Recommendations: Although common clinical practice is that blood cultures and lumbar puncture are obtained if there is a clinical suspicion of a systemic or CNS infection, there are insufficient data to support or refute recommendations as to whether blood cultures or lumbar puncture should be done on a routine basis in children in whom there is no clinical suspicion of a systemic or CNS infection (Level U). AED levels should be considered when a child with treated epilepsy develops SE (Level B). Toxicology studies and metabolic studies for inborn errors of metabolism may be considered in children with SE when there are clinical indicators for concern or when the initial evaluation reveals no etiology (Level C). An EEG may be considered in a child with SE as it may be helpful in determining whether there are focal or generalized epileptiform abnormalities that may guide further testing for the etiology of SE, when there is a suspicion of pseudostatus epilepticus (nonepileptic SE), or nonconvulsive SE, and may guide treatment (Level C). Neuroimaging may be considered after the child with SE has been stabilized if there are clinical indications or if the etiology is unknown (Level C). There is insufficient evidence to support or refute routine neuroimaging in a child presenting with SE (Level U).

Summary:Purpose: Status epilepticus (SE) is a major medical and a neurologic emergency associated with significant morbidity and mortality. The current definition of SE is continuous seizure activity or intermittent seizure activity without regaining consciousness, lasting a 3 0 min. Epilepsy monitoring unit data indicate that many seizures self-terminate within minutes. Thus consideration was recently given to include seizure episodes lasting 3 1 0 min in the definition of SE. Because no large studies have been conducted on seizures lasting 10-29 min, this study was initiated to compare cases of SE and 10 to 29-min seizure episodes seen within the same period.Methods: Patients seen at the Medical College of Virginia Hospitals of Virginia Commonwealth University over the same 2-year period were studied. Two hundred twenty-six prospective SE cases (91 children and 135 adults) and 81 retrospective 10-to 29-min seizure episodes (31 children and 50 adults) were compared. A standardized data-entry-form system was compiled on each patient and was used to evaluate the data collected.Results: The 10-to 29-min seizure patients and the SE cases had similar demographic characteristics, such as sex, race, and age, and also had similar etiologies. The majority (93%) of SE cases required anticonvulsant (AED) treatment to control and stop seizure activity. In the 10-to 29-min group, 43% stopped seizing spontaneously, and the remainder (57%) required AED treatment to stop seizure activity. The mortality for the SE patients was 19% compared with 2.6% for 10-to 29-min group (p < 0,001). In the 10-to 29-min group that stopped seizing spontaneously, the mortality was 0. In the 10-to 29-min patients that required AED treatment, the mortality was 4.4%.Conclusions: The results demonstrate that a significant number of patients experience seizure activity lasting from 10-to 29-min. Approximately half of these seizure events stopped spontaneously and did not require AED treatment. The other half of the patients responded quickly to medications and stopped seizing before the 30-min definition for SE. The overall mortality of this group was significantly lower than that of the patients with SE. The results demonstrate that further studies on the 10-to 29-min seizure group are needed to differentiate seizures that will stop spontaneously and those that will only stop with AED treatment. Because almost half of the prolonged seizures stopped spontaneously, further studies are needed before including prolonged seizure activity in the definition of SE.

In cases of refractory status epilepticus (RSE) unresponsive to sequential trials of multiple agents, a suspension of topiramate administered via nasogastric tube was effective in aborting RSE, including one patient in a prolonged pentobarbital coma. Effective dosages ranged from 300 to 1,600 mg/d. Except for lethargy, no adverse events were reported.

No abstract