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Benign infantile familial convulsions.

F Vigevano1, L Fusco, M Di Capua

  • 1Section of Neurophysiology, Bambino Gesu Children's Hospital, Rome, Italy.

European Journal of Pediatrics
|August 1, 1992
PubMed
Summary

This study identified a benign, familial epilepsy syndrome in infants presenting with clusters of partial-onset seizures. Genetic predisposition is suggested by a family history of similar early-onset, benign convulsions.

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Area of Science:

  • Pediatric Neurology
  • Clinical Genetics
  • Epileptology

Background:

  • Infantile epilepsy presents diagnostic challenges.
  • Familial epilepsy syndromes require further characterization.
  • Understanding benign early-onset seizures is crucial for prognosis.

Purpose of the Study:

  • To describe a specific epilepsy syndrome in infants.
  • To investigate the clinical and EEG characteristics of these seizures.
  • To explore potential genetic factors.

Main Methods:

  • Case series of five infants with early-onset convulsions.
  • Clinical seizure description and classification.
  • Electroencephalogram (EEG) analysis (interictal and ictal).
  • Family history assessment for genetic predisposition.

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Main Results:

  • Seizures occurred between 4-6 months, in clusters, and were controlled with phenobarbital or valproate.
  • Seizures were partial with secondary generalization, featuring head/eye deviation, hypertonia, and limb jerks.
  • Normal interictal EEG, but ictal EEG showed central-occipital onset.
  • Normal laboratory, radiological, and neurological findings.
  • Family history of similar benign convulsions in paternal relatives suggested genetic inheritance.

Conclusions:

  • A benign familial epilepsy syndrome in infancy is described.
  • The syndrome is characterized by specific seizure semiology and EEG findings.
  • A genetic predisposition, likely inherited paternally, is suggested.