Current treatment of myoclonic astatic epilepsy: clinical experience at the Children's Hospital of Philadelphia.
OBJECTIVE
Myoclonic astatic epilepsy (MAE) is a generalized epilepsy of early childhood. Little is known about the use of newer antiepileptic treatments (AET) in MAE. The purpose of this study was to describe the characteristics, treatment, and outcome of a contemporary MAE cohort exposed to the new generation AET.


METHODS
Charts of subjects with MAE treated between 1998 and 2005 were reviewed.


RESULTS
Twenty-three subjects (19 boys), with a median (range) follow-up of 38 (2- 86) months were identified. Thirty-nine percent had a family history of epilepsy, and 39% had family history of febrile seizures. Age at seizure onset was a median of 36 (12-24) months. Initial EEG was normal in 30%. When seizures ceased, EEG background and epileptiform abnormalities persisted in 17 and 58%, respectively. On average, each subject was exposed to five AET. The most frequently used AET was valproate (83%). Seizure freedom occurred spontaneously in three subjects, with ethosuximide and levetiracetam in one each, valproate and lamotrigine in two each, topiramate in three and the ketogenic diet (KD) in five subjects. By 36 months after seizure onset, 67% achieved seizure freedom. At the last visit, 43% were developmentally normal, 52% had mild, and 5% had moderate cognitive disabilities. Time to seizure freedom did not correlate with cognitive outcome.


CONCLUSIONS
The new generation of AET may offer significant benefit to children with MAE. The KD was the most effective AET in this series, and perhaps should be considered earlier in treatment.
Kilaru
Sudha
S
Division of Neurology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, U.S.A.
Bergqvist
A G Christina
AGC
Division of Neurology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, U.S.A.
D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D003072:Cognition Disorders / Q000188:drug therapy / Q000628:therapy; D004041:Dietary Fats / Q000008:administration & dosage / Q000378:metabolism; D004569:Electroencephalography / Q000706:statistics & numerical data; D004831:Epilepsies, Myoclonic / Q000178:diet therapy / Q000188:drug therapy / Q000628:therapy*; D005013:Ethosuximide / Q000627:therapeutic use; D005260:Female; D006776:Hospitals, Pediatric; D006801:Humans; D007659:Ketones / Q000378:metabolism; D007662:Ketosis / Q000139:chemically induced* / Q000378:metabolism*; D000077213:Lamotrigine; D000077287:Levetiracetam; D008297:Male; D010414:Pennsylvania; D010889:Piracetam / Q000031:analogs & derivatives / Q000627:therapeutic use; D012189:Retrospective Studies; D016019:Survival Analysis; D013577:Syndrome; D014227:Triazines / Q000627:therapeutic use
D000927:Anticonvulsants; D004041:Dietary Fats; D007659:Ketones; D014227:Triazines; D000077287:Levetiracetam; D005013:Ethosuximide; D000077213:Lamotrigine; D010889:Piracetam