Synonyms
Doose Syndrome (epilepsy with myoclonic atonic seizures); Dravet Syndrome (severe myoclonic epilepsy in infancy)
Definition
Myoclonic epilepsy in infancy has traditionally been divided into three types based upon the clinical features and prognosis, but they all share the common feature of myoclonic seizures with very sudden and brief muscle contractions (“jerks”) that typically involve the head and upper extremities.
Myoclonic epilepsy in infancy (MEI) was previously termed benign myoclonic epilepsy in infancy because of its single seizure type and excellent response to treatment. However, because of a risk for developmental problems in up to one-third of children with MEI, the term “benign” was removed.
Epilepsy with myoclonic atonic seizures (EMAS), previously termed myoclonic-astatic epilepsy, is also known as Doose Syndrome and is characterized by multiple seizure types with myoclonic, myoclonic-astatic, and generalized jerks. Sudden falls are a prominent and often...
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References
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Titus, J.B., Clarke, D. (2018). Myoclonic Epilepsy in Infancy. In: Kreutzer, J.S., DeLuca, J., Caplan, B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-57111-9_1572
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DOI: https://doi.org/10.1007/978-3-319-57111-9_1572
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