Teaching Video NeuroImage: Severe Facioglossal Weakness and Dysarthria Due to Bilateral Perisylvian Polymicrogyria

A 20-year-old man with a history of spastic hemiparetic cerebral palsy, microcephaly, intellectual disability, and childhood-onset focal epilepsy presented to our Epilepsy Transition Program to establish care. Seizure semiology included staring spells and convulsions, which were well controlled on monotherapy with oxcarbazepine. Examination (Video 1) showed significant lower facial and tongue weakness leading to severe dysarthria and excessive drooling. MRI showed bilateral perisylvian polymicrogyria (BPP) (Figure). BPP is the most common form of polymicrogyria; its clinical manifestations include facio-pharyngeo-glosso-masticatory paresis, intellectual disability, and seizures.1,2 The facioglossal weakness in BPP is likely the result of the topographic proximity of the respective primary motor centers and the affected perisylvian area.