A 15-year-old boy presented with 5 years of refractory focal seizures with 4–6 episodes per day. The seizure semiology was described as numbness of the left half of the body followed by tonic posturing and head version to left with loss of awareness lasting for 30 seconds. Additional symptoms included cognitive impairment with delayed and dysarthric speech. Neuropsychological assessment revealed impaired intellectual and cognitive ability. MRI of the brain demonstrated bilateral perisylvian polymicrogyria (Figure, A–C). BPP is a cortical development malformation1 characterized by widened Sylvian fissures with an increased number of abnormally small gyri and shallow sulci. Patients present with dysarthria and refractory seizures.2
Apparent thickening of polymicrogyric cortex and stippling and irregularity at the gray-white matter junction (arrowheads) are also seen compared with frontal polar gray matter (arrows).
Study Funding
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Appendix Authors
Footnotes
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Submitted and externally peer reviewed. The handling editor was Roy Strowd III, MD, Med, MS.
Teaching slides links.lww.com/WNL/B913
- Received November 4, 2021.
- Accepted in final form March 16, 2022.
- © 2022 American Academy of Neurology
References
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