Overview
Congenital bilateral perisylvian syndrome (CBPS) is a developmental disorder characterized by bilateral malformations in the perisylvian region, often manifesting as polymicrogyria affecting the sylvian fissure and opercular cortex. It presents with a spectrum of neurological deficits including developmental delay, motor impairments, and speech difficulties 12.Diagnosis
Clinical Features: Developmental delay, poor palatal function, hypotonia, arthrogryposis, hemiparesis, apnea, paraparesis, micrognathia 1.
Imaging: MRI is essential, showing bilateral perisylvian polymicrogyria; unilateral schizencephaly with contralateral polymicrogyria may also be observed 1.
Histologic Confirmation: In rare cases, autopsy or biopsy may reveal polymicrogyria confirming the diagnosis 2.Management
Supportive Care: Focus on managing symptoms including physical therapy for motor impairments, speech therapy for communication difficulties, and nutritional support for feeding issues 1.
Seizure Control: Antiepileptic drugs tailored to seizure types and frequency; specific drug classes include valproates, levetiracetam, or others as indicated 1.
Respiratory Monitoring: Regular monitoring and management for apnea episodes, possibly requiring respiratory support devices 1.Special Populations
Pediatrics: Early intervention is crucial; diagnosis often occurs in early childhood with a median age of 2.25 years 1.
Comorbidities: Common comorbidities include hypotonia and arthrogryposis, necessitating multidisciplinary care 1.Key Recommendations
MRI for Diagnosis: Routine MRI to identify bilateral perisylvian polymicrogyria or associated anomalies is essential for confirming CBPS (Evidence: Moderate) 1.
Early Multidisciplinary Support: Initiate early multidisciplinary management including physical, occupational, and speech therapy to address motor and developmental delays (Evidence: Expert opinion) 1.
Seizure Management: Implement individualized antiepileptic drug therapy based on seizure types and frequency to control seizures effectively (Evidence: Moderate) 1.References
1 Gropman AL, Barkovich AJ, Vezina LG, Conry JA, Dubovsky EC, Packer RJ. Pediatric congenital bilateral perisylvian syndrome: clinical and MRI features in 12 patients. Neuropediatrics 1997. link
2 Shevell MI, Carmant L, Meagher-Villemure K. Developmental bilateral perisylvian dysplasia. Pediatric neurology 1992. link90370-e)