Epilepsia
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Review
The natural history of myoclonic astatic epilepsy (Doose syndrome) and Lennox-Gastaut syndrome.
The purpose of this article is to present a short review of the natural history of myoclonic astatic epilepsy (MAE; Doose syndrome) and the Lennox-Gastaut syndrome (LGS). In the 1989 classification of the International League Against Epilepsy (ILAE, 1989), MAE and LGS were initially included in group 2.2: "Cryptogenic or symptomatic generalized epilepsies and syndromes." The subsequent classification of the Proposed Diagnostic Scheme for People with Epileptic Seizures and with Epilepsy (see Ref. 8) placed MAE in axis 3 in the "generalized epilepsy" group and LGS, severe myoclonic epilepsy of infancy (SMEI or Dravet syndrome) and atypical benign partial epilepsy/pseudo-Lennox syndrome (ABPE/PLS) in the "epileptic encephalopathy" group. The semiology of MAE and LGS and their differential diagnosis from SMEI and ABPE/PLS are described. ⋯ The course of MAE is highly variable with regard to seizure outcome (complete remission in some cases, persistent epilepsy in others) and cognitive development (normal or delayed). The course of LGS and SMEI is generally poor, both with regard to the epilepsy and to the cognitive development whereas the course and seizure outcome of ABPE/PLS is favorable; the patients will be seizure-free at puberty. However, the neuropsychological outcome is less favorable; most patients remain mentally retarded.
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Antiepileptic drug (AED) guidelines are developed to improve medical decision making, to provide guidance and recommendation for patient management, to develop standards to judge or assess clinical practice, and to keep the cost-benefit ratio at an acceptable level. These guidelines are derived from evidence-based medicine (EBM), a four-tiered grading system that is used to analyze clinical trials and published experiments independent of clinical bias and experience. Although guidelines may not answer all questions it is critical that clinicians using them consider the available evidence, as well as the quality of the evidence, when incorporating the information in their decision making.
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Comparative Study
EEG/fMRI study of ictal and interictal epileptic activity: methodological issues and future perspectives in clinical practice.
Electroencephalography/functional magnetic resonance imaging (EEG/fMRI) has been proposed recently as a tool to study electrophysiological activity and, consequently, detect brain regions activated during epileptiform EEG abnormalities. The purpose of the study was to review our two-year experience with studying ictal and interictal activities in patients with epilepsy. ⋯ By defining the electro-clinical and hemodynamic correlates of EEG activity, fMRI may shed light on the neurophysiological mechanisms underlying epileptic phenomena. However, as several methodological issues have yet to be addressed, further studies are warranted to assess the reliability and usefulness of EEG/fMRI in clinical practice.
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Status epilepticus (SE) is a medical emergency which can lead to significant morbidity and mortality and requires prompt diagnosis and treatment. SE is differentiated into generalized or partial SE on the basis of its electro-clinical manifestations. The guidelines for the management of SE produced by the Italian League against Epilepsy also distinguish three different stages of SE (initial, established and refractory), based on time elapsed since the onset of the condition and responsiveness to previously administered drugs. ⋯ Refractory SE requires admission to an intensive care unit (ICU) to allow adequate monitoring and support of respiratory, metabolic and hemodynamic functions and cerebral electrical activity. In refractory SE, general anesthesia may be required. Propofol and thiopental represent first line agents in this setting, after careful assessment of potential risks and benefits.
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Dr. Jordan (PRO) argues that when refractory NCSE is combined with acute brain injury, it produces synergistically compounded brain damage that is time dependent. Treating to EEG burst-suppression is the most rapid and effective method for stopping NCSE. ⋯ Hirsch (CON) argues that treating with coma-inducing medication is highly risky and has a high mortality rate, often due to iatrogenic complications. It remains unclear if nonconvulsive seizures cause permanent neuronal injury. Nonconvulsive seizures should be diagnosed and treated as quickly as possible,but with non-coma-inducing treatments in most cases [corrected]