Successful treatment of migrating partial seizures in Wolf-Hirschhorn syndrome with bromide

Brain Dev. 2016 Aug;38(7):658-62. doi: 10.1016/j.braindev.2016.01.001. Epub 2016 Jan 18.

Abstract

A girl with mild psychomotor developmental delay developed right or left hemiclonic convulsion at 10months of age. One month later, clusters of hemiclonic or bilateral tonic seizures with eyelid twitching emerged, resulting in status epilepticus. Treatment with phenobarbital and potassium bromide completely terminated the seizures within 10days. Ictal electroencephalography revealed a migrating focus of rhythmic 3-4Hz waves from the right temporal to right frontal regions and then to the left frontal regions. Genetic analysis was conducted based on the characteristic facial appearance of the patient, which identified a 2.1-Mb terminal deletion on chromosome 4p. This is the first case of Wolf-Hirschhorn syndrome complicated by epilepsy with migrating partial seizures.

Keywords: 4p-Syndrome; Bromide; Migrating partial seizures in infancy; Status epilepticus.

Publication types

  • Case Reports

MeSH terms

  • Anticonvulsants / therapeutic use*
  • Brain / drug effects
  • Brain / physiopathology
  • Bromides / therapeutic use*
  • Chromosome Deletion
  • Chromosomes, Human, Pair 4
  • Comparative Genomic Hybridization
  • Electroencephalography
  • Epilepsy / complications
  • Epilepsy / drug therapy
  • Epilepsy / genetics
  • Epilepsy / physiopathology
  • Female
  • Humans
  • Infant
  • Phenobarbital / therapeutic use
  • Potassium Compounds / therapeutic use*
  • Seizures / complications*
  • Seizures / drug therapy*
  • Seizures / genetics
  • Seizures / physiopathology
  • Wolf-Hirschhorn Syndrome / complications*
  • Wolf-Hirschhorn Syndrome / drug therapy*
  • Wolf-Hirschhorn Syndrome / genetics
  • Wolf-Hirschhorn Syndrome / physiopathology

Substances

  • Anticonvulsants
  • Bromides
  • Potassium Compounds
  • potassium bromide
  • Phenobarbital