Benign familial infantile convulsions: a clinical study of seven Dutch families

Petra Callenbach, René F.M. De Coo, Alla A. Vein, Willem Frans M. Arts, Jan C. Oosterwijk, Gerard Hageman, Robert ten Houten, Gisela M. Terwindt, Dick Lindhout, Rune R. Frants, Oebele F. Brouwer

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28 Citations (Scopus)


Benign familial infantile convulsions (BFIC) is a recently identified partial epilepsy syndrome with onset between 3 and 12 months of age. We describe the clinical characteristics and outcome of 43 patients with BFIC from six Dutch families and one Dutch-Canadian family and the encountered difficulties in classifying the syndrome. Four families had a pure BFIC phenotype; in two families BFIC was accompanied by paroxysmal kinesigenic dyskinesias; in one family BFIC was associated with later onset focal epilepsy in older generations. Onset of seizures was between 6 weeks and 10 months, and seizures remitted before the age of 3 years in all patients with BFIC. In all, 29 (67%) of the 43 patients had been treated with anti-epileptic drugs for a certain period of time. BFIC is often not recognized as (hereditary) epilepsy by the treating physician. Seizures often remit shortly after the start of anti-epileptic drugs but, because of the benign course of the syndrome and the spontaneous remission of seizures, patients with low seizure frequency do not necessarily have to be treated. If prescribed, anti-epileptic drugs can probably be withdrawn after 1 or 2 years of seizure freedom.
Original languageEnglish
Pages (from-to)269-283
Number of pages15
JournalEuropean Journal of Paediatric Neurology
Issue number5
Publication statusPublished - 2-Sep-2002


  • Benign familial infantile convulsions
  • Epilepsy
  • Genetics
  • Paroxysmal kinesigenic dyskinesias
  • anticonvulsive agent
  • carbamazepine
  • lamotrigine
  • phenobarbital
  • phenytoin
  • valproic acid
  • vigabatrin
  • adolescent
  • adult
  • article
  • benign childhood epilepsy
  • clinical article
  • clinical feature
  • clinical study
  • controlled study
  • disease classification
  • disease course
  • drug withdrawal
  • dyskinesia
  • family
  • female
  • focal epilepsy
  • genetic disorder
  • human
  • infant
  • male
  • Netherlands
  • newborn
  • onset age
  • phenotype
  • priority journal
  • remission

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