Double Cortex Syndrome (Subcortical Band Heterotopia): A Case Report

Area of Science:

• Pediatric Neurology
• Neuroimaging
• Genetics

Background:

• Developmental delay affects 5-10% of preschool children, often requiring diagnostic brain Magnetic Resonance Imaging (MRI).
• Neuronal migration disorders, such as subcortical band heterotopia (double cortex syndrome), are rare causes of developmental delay and epilepsy.
• Double cortex syndrome typically exhibits sex-linked inheritance, making sporadic cases unusual.

Observation:

• A 4.5-year-old girl presented with status epilepticus, developmental delay, and a history of recurrent seizures since infancy.
• Physical examination was unremarkable, but electroencephalography (EEG) showed generalized poly spike-wave discharges.
• Brain MRI revealed corpus callosal dysplasia, generalized band heterotopia, and polymicrogyria.

Findings:

• The patient was diagnosed with double cortex syndrome, a rare form of neuronal migration disorder.
• This represents the first reported case in Iran, specifically from Ahvaz city.
• The continuous or generalized form of band heterotopia is particularly rare.

Implications:

• Brain MRI is crucial for diagnosing neuronal migration disorders in developmentally delayed and epileptic children.
• Accurate diagnosis is essential for appropriate therapeutic management, prognosis, and genetic counseling.
• Early and precise diagnosis facilitates prenatal diagnosis and prevention strategies for affected families.