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...
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Abbreviations
- EME:
-
Early myoclonic encephalopathy
- MMPSI:
-
Malignant migrating partial seizures in infancy
- MISF:
-
Multiple independent spike foci
References
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 13–23
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 39–50
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 140:248–252
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 73–76
Ohtahara S, Yamatogi Y (2006) Ohtahara syndrome: with special reference to its developmental aspects for differentiating from early myoclonic encephalopathy. Epilepsy Res 70S:S58–S67
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 11:332–337
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Ohtahara, S., Yamatogi, Y. (2010). Early Myoclonic Encephalopathy. In: Panayiotopoulos, C.P. (eds) Atlas of Epilepsies. Springer, London. https://doi.org/10.1007/978-1-84882-128-6_119
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DOI: https://doi.org/10.1007/978-1-84882-128-6_119
Publisher Name: Springer, London
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