Early Myoclonic Encephalopathy

  • Shunsuke Ohtahara
  • Yasuko Yamatogi
Reference work entry

Short Description

Early myoclonic encephalopathy (EME) is a rare epileptic syndrome characterized by (1) very early onset, mostly during the neonatal period, (2) fragmentary erratic myoclonias, massive myoclonias, and focal seizures, and (3) suppression-burst pattern (SB) in EEG. Seizures, particularly focal motor seizures, are intractable, and life and psychomotor prognoses are very severe (Dalla Bernardina et al.1983; Aicardi 1992; Aicardi and Ohtahara 2005; Ohtahara and Yamatogi 2006).

Classification

EME, first described by Aicardi and Goutières in 1978, is classified into symptomatic generalized epilepsy according to ILAE classification of epilepsies and epileptic syndromes (1989). Diagnostic scheme of ILAE (2001) proposed to categorize it into “epileptic encephalopathy” with progressive deterioration due to epileptic abnormalities.

Clinical Manifestation

The earliest symptom is usually fragmentary or segmental erratic myoclonias which may occur within the first several hours of...

Keywords

Focal Seizure Epileptic Syndrome West Syndrome Propionic Acidemia Methylmalonic Acidemia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

List of Abbreviations

EME

Early myoclonic encephalopathy

MMPSI

Malignant migrating partial seizures in infancy

MISF

Multiple independent spike foci

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References

  1. Aicardi J (1992) Early myoclonic encephalopathy (neonatal myoclonic encephalopathy). In: Roger J, Bureau M, Dravet Ch, Dreifuss FE, Perret A, Wolf P (eds) Epileptic syndromes in infancy, childhood and adolescence, 2nd edn. John Libbey, London, pp 13–23Google Scholar
  2. Aicardi J, Ohtahara S (2005) Severe neonatal epilepsies with suppression-burst pattern. In: Roger J, Bureau M, Dravet Ch, Genton P, Tassinari CA, Wolf P (eds) Epileptic syndromes in infancy, childhood and adolescence, 4th edn. John Libbey, Montrouge, pp 39–50Google Scholar
  3. Dalla Bernardina B, Dulac O, Fejerman N, Dravet C, Capovilla G, Bondavalli S, Colamaria V, Roger J (1983) Early myoclonic epileptic encephalopathy (EMEE). Eur J Pediatr 140:248–252CrossRefPubMedGoogle Scholar
  4. Dulac O (2005) Malignant migrating partial seizures in infancy. In: Roger J, Bureau M, Dravet Ch, Genton P, Tassinari CA, Wolf P (eds) Epileptic syndromes in infancy, childhood and adolescence, 4th edn. John Libbey, Montrouge, pp 73–76Google Scholar
  5. Ohtahara S, Yamatogi Y (2006) Ohtahara syndrome: with special reference to its developmental aspects for differentiating from early myoclonic encephalopathy. Epilepsy Res 70S:S58–S67CrossRefGoogle Scholar
  6. Otani K, Abe J, Futagi Y, Yabuuchi H, Aotani H, Takeuchi T (1989) Clinical and electroencephalographical follow-up study of early myoclonic encepahlopathy. Brain Dev 11:332–337PubMedGoogle Scholar

Copyright information

© Springer-Verlag London Limited 2010

Authors and Affiliations

  • Shunsuke Ohtahara
    • 1
  • Yasuko Yamatogi
    • 2
  1. 1.Department of Child NeurologyOkayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Japan
  2. 2.Health and Welfare ScienceOkayama Prefectural University Japan

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