From the Research
Brainstem hypoplasia, arachnoid cyst, and cortical thickening syndrome is a rare neurological condition that requires a multidisciplinary approach for management, focusing on treating symptoms rather than the underlying condition, as there is no cure. This condition typically presents in early childhood with symptoms including developmental delays, motor difficulties, seizures, and cognitive impairments. The most recent and highest quality study on arachnoid cysts, which are a key component of this syndrome, was published in 2020 1, highlighting the importance of considering psychiatric disorders associated with arachnoid cysts, which are rare but can include hypomania symptoms and marked cognitive impairment. Management may include anticonvulsant medications such as levetiracetam (Keppra) at 10-20 mg/kg twice daily or valproic acid (Depakote) at 10-15 mg/kg twice daily for seizure control, as well as physical, occupational, and speech therapy to improve motor skills and communication. Regular monitoring with MRI scans every 6-12 months is recommended to track any changes in brain structure, particularly the arachnoid cysts which may occasionally require surgical intervention if they enlarge and cause increased intracranial pressure, as discussed in a study from 2012 2. A study from 2016 3 also highlights the association of arachnoid cysts with other syndromes, such as Kallmann syndrome, emphasizing the need for a comprehensive approach to diagnosis and treatment. Key aspects of management include:
- Treating symptoms rather than the underlying condition
- Multidisciplinary approach involving neurologists, neurosurgeons, developmental pediatricians, and therapists
- Regular monitoring with MRI scans
- Consideration of surgical intervention for arachnoid cysts that cause increased intracranial pressure
- Physical, occupational, and speech therapy to improve motor skills and communication.