Early myoclonic encephalopathy
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| Early myoclonic encephalopathy[1] | |
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| Specialty | Neurology |
Early myoclonic encephalopathy (EME) is an epilepsy syndrome where myoclonic seizures develop in the neonatal period. After several months, the seizure pattern may develop to infantile spasms (West syndrome). Various genetic and metabolic disorders are responsible. The seizures are resistant to treatment. The neurology is very abnormal and patients often do not live beyond one year.[2][3]
Signs and symptoms
It is characterised by tonic spasms that can be either generalized or lateralized which may occur singly or in clusters. The onset is in early infancy, within the first 3 months of age, but may be seen as early as first 2 weeks of age.[4]
Causes
Genetics
Acquired
Mechanism
Diagnosis
EEG
Electroencephalogram (EEG) is characterised by suppression burst pattern. Subsequently it may transition hypsarhythmia, a chaotic pattern on EEG [4]
Prevention
Management
Prognosis
The prognosis is usually poor. Mortality rate is high during early infancy and almost half of the children expire by 2 years of age.[4] Most of the children who survive, suffer from psychomotor impairment.[4]
Epidemiology
References
- ^ Berg, AT; Berkovic, SF; Brodie, MJ; Buchhalter, J; Cross, JH; et al. (Apr 2010). "Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009". Epilepsia. 51 (4): 676–85. doi:10.1111/j.1528-1167.2010.02522.x. PMID 20196795.
- ^ Richardson S, Alarcon G, Nashef L, Cross H, Nightingale J. Epilepsy (Oxford Specialist Handbooks in Neurology). Oxford University Press;2009. p.82. ISBN 0-19-857073-2
- ^ Djukic A, Vigevano F, Plouin P, Moshé S. Early Myoclonic Encephalopathy. In: Dichter MA, Engel J, Pedley TA, Aicardi J, editors. Epilepsy: a comprehensive textbook. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2008. ch. 224. ISBN 0-7817-5777-0
- ^ a b c d Beal, Jules C.; Cherian, Koshi; Moshe, Solomon L. (November 2012). "Early-Onset Epileptic Encephalopathies: Ohtahara Syndrome and Early Myoclonic Encephalopathy". Pediatric Neurology. 47 (5): 317–323. doi:10.1016/j.pediatrneurol.2012.06.002. PMID 23044011.
External links
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