[Congenital bilateral perisylvian syndrome: a case report]

Area of Science:

• Neurology
• Medical Genetics

Background:

• Congenital bilateral perisylvian syndrome (CBPS) is a rare neurological disorder.
• This case report focuses on a patient from China, highlighting the rarity of CBPS in the region.

Observation:

• The patient presented with pseudobulbar palsy, cognitive deficits, and intractable epilepsy.
• MRI revealed bilateral cortical thickening around deep sylvian fissures and polymicrogyria.
• EEG showed abnormalities including slow spikes and theta waves in specific brain regions.

Findings:

• The patient's intractable epilepsy was poorly controlled by standard antiepileptic drugs.
• Surgical intervention, specifically section of the corpus callosum (callosotomy), was performed.
• Post-callosotomy, significant seizure reduction and notable improvement in intellectual development were observed.

Implications:

• CBPS is characterized by specific clinical and neuroimaging features.
• Callosotomy can be an effective therapeutic option for managing intractable epilepsy associated with CBPS.
• This case expands understanding of CBPS management, particularly in cases with severe epilepsy.