Benign Myoclonic Epilepsy in Infancy

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Background

  • One of the idiopathic generalized epilepsies
  • 1% to 2% of epilepsies that start before the age of 3 years
  • Seizures start between 0.5 and 3 years of age in developmentally normal children. However, some cases have a later onset up to 4 years
  • A family history of febrile seizures and epilepsy is often reported
  • Remission usually occurs within 1 year (6 months to 5 years) from onset.
  • The outcome is generally benign. In rare cases, myoclonic epilepsy such as Juvenile Myoclonic Epilepsy may follow Benign Myoclonic Epilepsy in Infancy

Clinic

  • Myoclonic jerks involving mostly the upper part of the body, singular or clusters
  • Consciousness is normally intact
  • Photosensitivity in 20% of the patients
  • Unexpected acoustic or tactile stimuli can elicit a the myoclonic jerks in 10%.
  • During sleep in most of the patients the myoclonic seizures persists

Treatment

  • Valproic acid was effective in 23 of 30 treated patients [1]
  • Patients with acoustic and somatosensory evoked myoclonic seizures may not need treatment.

EEG



5 year old girl with 3 Hz generelized polyspike waves and myoclonic jerks (double banana and EMG on deltoid muscle)

5 year old girl with 3 Hz generelized polyspike waves and myoclonic jerks (double banana and EMG on deltoid muscle) EEGpedia.png




Notes

  1. Auvin, S. , Pandit, F. , De Bellecize et al, Benign Myoclonic Epilepsy in Infants: Electroclinical Features and Long‐term Follow‐up of 34 Patients. Epilepsia, 2006 47: 387-393. doi:10.1111/j.1528-1167.2006.00433.x