RT Book, Section
A1 Connolly, Mary B.
A2 Duchowny, Michael
A2 Cross, J. Helen
A2 Arzimanoglou, Alexis
SR Print(0)
ID 1138409959
T1 Benign Myoclonic Epilepsy in Infancy
T2 Pediatric Epilepsy
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9780071496216
LK neurology.mhmedical.com/content.aspx?aid=1138409959
RD 2024/04/20
AB Benign  myoclonic  epilepsy  in  infancy  (BMEI),  first  described  by  Dravet  and  Bureau  in  1981,  is  a  rare  epilepsy  syndrome.1  It  is  classified  among  the  idiopathic  generalized  epilepsies  and  typically  begins  by  the  age  of  3  years.2  BMEI  is  characterized  by  brief  myoclonic  seizures  without  other  seizure  types  in  developmentally  normal  children.  The  myoclonic  seizures  may  occur  spontaneously  or  be  provoked  by  contact  or  noise.  A  history  of  simple  febrile  seizures  may  be  present.  In  a  series  of  88  patients  with  BMEI,  26.1%  had  a  history  of  simple  febrile  seizures.3  There  is  a  family  history  of  epilepsy  or  febrile  seizures  in  50%  or  more  of  patients.  Myoclonic  seizures  are  easily  controlled  with  medication  and  usually  remit  later  in  childhood.  Generalized  tonic–clonic  seizures  may  occur  in  late  childhood  or  adolescence.  Neurodevelopmental  outcome  is  variable;  most  children  have  normal  development  but  may  experience  educational  difficulties.