Double cortex syndrome (subcortical band heterotopia): A case report

Area of Science:

• Neurology
• Genetics
• Radiology

Background:

• Double cortex syndrome is a rare X-linked dominant disorder.
• It typically presents with developmental delay and seizures.

Observation:

• A 14-year-old girl experienced severe tonic-clonic seizures and loss of consciousness.
• Her developmental milestones declined around age 3, affecting speech, vision, and learning.
• Standard laboratory tests were unremarkable.

Findings:

• Electroencephalogram indicated epilepsy.
• Brain MRI revealed a continuous band of gray matter paralleling the cortex in both hemispheres, characteristic of band heterotopia or double cortex syndrome.

Implications:

• This case underscores the importance of neuroimaging in diagnosing rare genetic neurological disorders.
• Early diagnosis and management with antiepileptic drugs are crucial for patients with double cortex syndrome.
• Further research into X-linked dominant inheritance patterns and therapeutic strategies is warranted.