Benign familial and non-familial infantile seizures (Fukuyama-Watanabe-Vigevano syndrome): a study of 14 cases from Saudi Arabia

Brain Dev. 2010 May;32(5):378-84. doi: 10.1016/j.braindev.2009.04.014. Epub 2009 May 22.

Abstract

Purpose: Benign infantile seizures [BIS], familial and non-familial, represent a benign, age-related idiopathic syndrome of infancy. The aim of the current paper is to document the presence of the syndrome in Saudi Arabia and in Arab populations and to discuss the characteristic electroclinical features and the benign nature of this syndrome.

Patients and methods: A case series of 275 patients with epileptic seizures (age range: 2 months-13 years) were followed over a period of 3 years and 7 months. The inclusion criteria for BIS were as follows (1) age of seizure onset between 2 and 24 months, (2) normal development before, during and after the onset of seizures, (3) normal interictal EEG, (4) normal brain imaging, and (5) good response to treatment. We analyzed these infants with respect to age at seizure onset, sex, physical and neurological examination, consanguinity, frequency and type of convulsions, associated conditions and laboratory and radiological investigations. A waking and sleeping interictal EEG was performed on all patients, and for one patient (No. 1), ictal EEG and video clips were recorded.

Results: Fourteen infants (12.0%) showed electroclinical features consistent with BIS. Eleven patients fulfilled the criteria of benign non-familial infantile seizures (BNFIS), and for three patients, their family pedigrees showed the possibility of benign familial infantile seizures (BFIS). All of the patients responded to anti-epileptic treatment, and 50% of them responded within 3 months.

Conclusions: To our knowledge, this is the first study to document the presence of BIS (Fukuyama-Watanabe-Vigevano syndrome) in Saudi Arabian and Arab populations. We highlighted the characteristic features of BIS and demonstrated the benign nature of the syndrome.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Electroencephalography
  • Epilepsy / etiology
  • Epilepsy / genetics
  • Epilepsy / physiopathology*
  • Female
  • Humans
  • Infant
  • Male
  • Pedigree
  • Saudi Arabia
  • Syndrome
  • Video Recording