Hyperactivity and autistic features in a child with lissencephaly-pachygyria complex with hypoxic ischemic encephalopathy

  • Indu Surendran
  • Nidhi Chauhan
  • Chirag Ahuja
  • Ruchita Shah

Abstract

Lissencephaly–pachygyria complex or spectrum is a rare malformation of cortical development characterised by neuronal migration defect and abnormal formation of cerebral gyri or convolutions. The main features of lissencephaly are abnormally thick cortex along with absent (agyria) or abnormally developed (pachygyria) cerebral convolutions making the brain appear smooth. We present a case of nine-year-old girl with lissencephaly-pachygyria complex with hypoxic ischemic encephalopathy. She presented with severe intellectual disability, seizures, autistic features with marked sensory issues, self-stimulating behaviors, hyperactivity and aggression. We describe the clinical manifestations and highlight the role of non-pharmacological interventions that include family psycho-education, sensory and occupational therapy, functional behavior analysis and therapy, and addressing caregiver burn-out.

Published
2019-10-07
Section
Case Reports