Encyclopedia of Clinical Neuropsychology

2018 Edition
| Editors: Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan

Myoclonic Epilepsy in Infancy

  • Jeffrey B. TitusEmail author
  • Dave Clarke
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_1572

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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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Comprehensive Epilepsy ProgramDell Children’s Medical Center of Central TexasAustinUSA
  2. 2.Department of PsychologyThe University of Texas at AustinAustinUSA
  3. 3.Department of PediatricsDell Medical School, The University of Texas at AustinAustinUSA