Congenital Perisylvian Syndrome: Magnetic Resonance Imaging Findings in Six Cases

Abstract

Background: Congenital perisylvian syndrome is an extremely rare congenital neurological disorder associated with distinctive imaging findings. Herein we describe the clinical features and imaging appearance of congenital perisylvian syndrome on Magnetic Resonance Imaging (MRI). Material and Methods: Six patients with clinical history, neurological examination and MRI findings suggestive of perisylvian syndrome were included in this review. Results: Six patients were found to have perisylvian syndrome on MRI findings. Five patients presented with pseudobulbar palsy while seizures were present in 3 patients. The most common MRI findings were vertically oriented sylvian fissures that were continuous with the central or post-central gyrus and perisylvian polymicrogyria. The findings were bilateral in 4 cases and unilateral in 2 cases. Conclusion: Perisylvian syndrome is a rare neurological disorder which can be diagnosed with certainty on MRI findings along with relevant clinical history.

Keywords

• Perisylvian syndrome
• Magnetic Resonance Imaging
• Pseudobulbar palsy
• Polymicrogyria
• Seizures