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Monozygotic twins with severe myoclonic epilepsy in infancy discordant for clinical features

Sahoko Miyama1, Tomohide Goto, Yushi Inoue

  • 1Department of Neurology, Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, Japan. DWM48541@biglobe.ne.jp

Pediatric Neurology
|July 22, 2008
PubMed

Insights

Identical twins with severe myoclonic epilepsy in infancy showed different disease courses. Bacterial meningitis in one twin altered epilepsy progression and language development.

Area of Science:

  • Neurology
  • Genetics
  • Pediatrics

Background:

  • Severe myoclonic epilepsy in infancy (SMEI) is a severe genetic epilepsy with significant developmental impact.
  • Monozygotic twins typically exhibit highly similar clinical presentations due to identical genetics.

Observation:

  • Identical male twins with SMEI presented with concordant seizure onset, symptoms, and development until 38 months.
  • Clinical courses diverged afterward, with delayed myoclonus and preserved language in twin 1 compared to twin 2.

Findings:

  • Twin 1 developed bacterial meningitis at 35 months, coinciding with the divergence in clinical course.
  • Bacterial meningitis appeared to influence the manifestation of SMEI, delaying myoclonus and preventing language regression in twin 1.

Implications:

  • Environmental factors, such as infections, can significantly modify the clinical trajectory of genetically determined epilepsies.
  • This case highlights the complex interplay between genetic predisposition and environmental insults in neurodevelopmental disorders.

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