Brain Inflammation in an Infant With Hemimegalencephaly, Escalating Seizures, and Epileptic Encephalopathy

Se Hee Kim1, John J Millichap1, Sookyong Koh2

  • 1Epilepsy Center, Ann & Robert H. Lurie Children's Hospital of Chicago, Department of Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.

Insights

Hemimegalencephaly, a brain malformation causing hemisphere enlargement, often leads to severe epilepsy. This case study found active brain inflammation, suggesting a link between neuroinflammation and worsening seizures in affected children.

Area of Science:

  • Neurology
  • Neuroscience
  • Pediatric Neurology

Background:

  • Hemimegalencephaly is a congenital brain malformation characterized by unilateral hemisphere enlargement.
  • It is frequently associated with intractable epilepsy, developmental delay, and neurological deficits.
  • The underlying mechanisms contributing to epilepsy in hemimegalencephaly are not fully understood.

Purpose of the Study:

  • To investigate the presence and extent of neuroinflammation in a pediatric patient with hemimegalencephaly and intractable epilepsy.
  • To explore the potential role of neuroinflammation in seizure exacerbation and epileptic encephalopathy.

Main Methods:

  • Case report of a 12-month-old girl with hemimegalencephaly.
  • Surgical intervention: hemispherectomy due to escalating seizures, developmental arrest, and encephalopathy.
  • Histopathological examination of resected brain tissue using immunohistochemistry.

Main Results:

  • Immunohistochemical analysis revealed abundant activated CD68-positive microglia (indicating microgliosis).
  • Evidence of reactive astrogliosis was also detected.
  • These findings indicate active inflammatory changes within the affected brain hemisphere.

Conclusions:

  • The presence of significant neuroinflammation in hemimegalencephaly associated with intractable epilepsy is demonstrated.
  • Ongoing brain inflammation may contribute to seizure exacerbation and the development of epileptic encephalopathy.
  • This suggests that anti-inflammatory strategies could be a potential therapeutic avenue for such cases.