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• W4233313126 abstract "The myoclonic epilepsies are a group of syndromes in which myoclonic seizures are a prominent symptom. However, they persistently present a challenge during neurological training and are often difficult to comprehend. The molecular advances in our understanding of the progressive myoclonus epilepsies, the recognition of the patterns of idiopathic generalized epilepsy in adults, and the identification of myoclonic epilepsy syndromes in children have clarified considerably the dimensions of the problem. However, there are currently many questions about both malignant and benign myoclonic epilepsies that need resolution. Particularly striking is the tremendous range in the etiology of myoclonic disorders from the most malignant, as in the progressive myoclonus epilepsies, to the most benign and most easily treated, as in the idiopathic generalized epilepsies. In addition, it has recently become clear that some drugs are effective antimyoclonic agents, whereas others are promyoclonic and may contribute to the emergence or aggravation of myoclonus. The principles of treatment of myoclonus are not necessarily the same as those for the treatment of the epilepsies in general. Clearly, the myoclonic epilepsies are an intriguing group of disorders that will benefit from this instructive and highly relevant discussion. The following articles are compiled from the proceedings of a satellite symposium sponsored by the American Epilepsy Society, held in December 2001, and seek to present a thorough examination of myoclonic epilepsies, resulting in improved understanding, diagnosis, and more effective treatment. It is necessary to classify a patient's syndrome correctly since treatment, prognosis, and further research depend on accurate categorization. Dr. Ilo Leppik's article provides a detailed discussion of the various classifications of the myoclonic epilepsies and the importance of accurate classification. Likewise, it is also essential to examine the clinical description of myoclonic disorders. Dr. Edward Faught reviews the phenomenology of myoclonic seizures and the classification of myoclonus based on correlations with EEG and EMG. Molecular genetic research in myoclonus further assists in providing a more accurate syndrome classification and ultimately is expected to provide rational treatment through gene therapy. Dr. Antonio Delgado-Escueta provides an overview of the new advances in molecular genetics of benign or idiopathic and progressive myoclonus epilepsies in infants, children, adolescents, and adults. Finally, we must consider the treatment strategies for myoclonic epilepsy. More treatment options are available today and the clinician faces the challenge of determining the most appropriate approach. Dr. James Wheless discusses treatment options based on determining etiology and rational classification of the epilepsy syndrome. Despite the challenges involved in treatment, significant advances in both our understanding of the underlying etiology, classification, genetics, and treatment options for myoclonic epilepsies have recently surfaced, and current research continues to progress." @default.
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• W4233313126 date "2003-10-19" @default.
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• W4233313126 title "Introduction" @default.
• W4233313126 doi "https://doi.org/10.1046/j.1528-1157.44.s11.1.x" @default.
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