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Electronic monitoring of the fetal heart rate during labor (EFM), originally designed to assess fetal stress and allow the early detection of the compromised fetus, has instead led to increasing maternal morbidity without decreasing fetal morbidity. The unintended consequences of this technologic advance have led to the creation of a pseudodisease and unwarranted intervention in response to its detection. Is it ethical to introduce a new technology without adequate assessment of its possible consequences? Are we about to repeat this (error resulting from the introduction of EFM?) There is increasing interest in monitoring the function of the newborn brain, to enable the early detection of subclinical seizures. The monitor may also be used for assessing brain function in older children and adults who are comatose or paralyzed and cannot appropriately respond to stimuli. Use of this amplitude-integrated electroencephalography (aEEG) in the newborn for detection of seizures and other brain abnormalities is not dissimilar to the use of electronic fetal heart rate monitoring. Whether seizures or subclinical seizures themselves cause harm to the developing nervous system is unclear. The effectiveness of medications for treatment of seizures in the newborn has not been established. Therefore, the consequences of introducing automated EEG for the detection of subclinical neonatal seizures are likely to be similar to the results of the introduction of EFM: creation of another pseudodisease, followed by unwarranted intervention, and increased legal liability. What are the ethics of continued approval and introduction of unevaluated technology? What is the wisdom of its use? Beware of the unintended consequences.

Purpose: To determine the long-term outcome of children with difficult-to-control seizures who remained on the ketogenic diet for <1 year. Methods: Between 1994 and 1996, 150 children with epilepsy, refractory to at least two medications, initiated the ketogenic diet according to the Hopkins protocol. Three to six years after diet initiation, all the families were contacted by telephone or questionnaire to assess their child's current seizure status, medications, and therapies. Results: Sixty-seven children discontinued the diet within 1 year of initiation. Follow-up data were available for 54 of these children. Ten subsequently had surgery, and three underwent VNS implantation. These operated-on children were significantly more likely to be >50% controlled at follow-up than were those managed with medications alone (p < 0.05). A statistically significant difference in long-term outcome was noted between those who responded while on the diet, even if they discontinued it before 1 year, and those who did not (p < 0.05), but no statistical correlation was found between length of time that they had remained on the diet and long-term prognosis. Conclusions: Almost half of the children who discontinued the diet during the first year had a decrease in seizures when assessed 3–6 years later. Twenty-two percent of these had become seizure free without surgery. We were unable to ascertain whether this may have been due to new medications. Those who saw some improvement while on the diet were more likely to have a favorable long-term outcome. Resective surgery, in children who were candidates, or vagal nerve stimulation (VNS) implantation, was more likely to result in significant seizure improvement than was management with medications alone. Whether or not the diet was effective, most families did not regret trying it and would recommend it to others. [ABSTRACT FROM AUTHOR]

Rasmussen’s encephalitis, originally thought to be a chronic form of viral encephalitis, is now thought to be an autoimmune disease of the brain and is more properly termed Rasmussen’s syndrome. Starting in one area of one side of the brain, the disease appears to gradually and progressively involve that side of the brain causing progressive and intractable focal seizures, a hemiparesis, and expressive aphasia when the left hemisphere is involved. Immune therapy with steroids, immunoglobulins, or plasmaphoresis provide only temporary relief from seizures. Neither antibodies to Glu-R3 nor cortical biopsy are helpful in the diagnosis. Hemispherectomy of one form or another is the only curative therapy, and there is no evidence that one form of hemispherectomy is preferable to another. Immuno-ablative therapy may be a therapy of the future. [Copyright &y& Elsevier]

Purpose: Rasmussen syndrome (RS) is a rare form of epilepsy characterized by progressive destruction of a single hemisphere. To characterize the profile of cortical involvement in RS, we studied the pathological changes in the cerebral cortex of 45 hemispherectomies performed at Johns Hopkins Hospital between 1985 and 2002. Methods: The patterns of pathologic changes and stages of cortical abnormalities were studied by histology and immunocy to chemistry methods. The burden of pathology (BP) was quantified in all brain regions of each of the 45 hemispheres. Results: Our study demonstrated significant heterogeneity in the stages of cortical pathology and the multifocal nature of the disease. These stages varied from early inflammation defined by infiltration of T lymphocytes and neuroglial reactions, to more severe stages with extensive neuronal cell death and cavitation of the cerebral cortex. A greater BP was significantly associated with an early age at onset (p = 0.01) and longer duration of disease (p ≤ 0.001). The BP was similar in all brain regions except the occipital lobe, where the BP was significantly lower (p = 0.032). Conclusions: The multifocal distribution of pathologic changes, as well as the heterogeneity in the stages of cortical damage in each patient, is consistent with an ongoing and progressive immune-mediated process of neuronal damage that involves neuroglial and lymphocytic responses, resembling other autoimmune CNS disorders such as multiple sclerosis. [ABSTRACT FROM AUTHOR]

The article offers information on the blinded-crossover study of the ketogenic diet to evaluate the effectiveness of the diet in children with epilepsy. It states that the study found a decline in drop seizures as identified by parents and a decrease in electroencephalography (EEG)-identified events. It says that the study raises fundamental questions on assessment of outcomes of therapeutic interventions in epilepsy.

Discusses the results of a survey on the factors associated with brain disorders among children. Causes of severe mental retardation; Discussion on cerebral palsy; Information on women at high risk for bearing a neurologically handicapped child; Challenges in detecting asphyxia; Discussion on the level of certainty about the prenatal and perinatal causes of most neurological disorders.