Pharmacoresistent Partial Epilepsy Secondary to Progressive Inflammatory Poliodystrophy.

Area of Science:

• Neurology
• Neuroinflammation
• Pediatric Epilepsy

Background:

• Epilepsy with progressive cortical volume loss is often linked to energy failure (e.g., mitochondrial disorders) or inflammation, typically in the temporal lobe.
• However, postmortem studies indicate seizures alone do not universally cause cortical volume loss.

Observation:

• Two children presented with extratemporal, pharmacoresistant epilepsy and gradual gray matter volume reduction over years.
• They also showed signs of central nervous system (CNS) inflammation.

Findings:

• Brain imaging and antibody profiles in these patients did not align with known antibody-mediated CNS syndromes like N-methyl-D-aspartate receptor encephalitis.
• This suggests a distinct inflammatory process.

Implications:

• These cases highlight a novel presentation of subacute CNS inflammation associated with epilepsy and progressive cortical volume loss.
• The findings underscore the importance of serial brain imaging in evaluating children with epilepsy and unexplained cortical volume changes.