The parents of a 9-month-old boy reported brief truncal jerks and abnormal eye movements in their child that increased gradually in frequency since birth. These episodes were ongoing despite treatment with multiple antiepileptic drugs (AED). He had regressed from previously acquired motor and language milestones. Cranial nerve examination was abnormal for eye tracking. He did not respond to visual stimuli and his eyes tended to deviate from one side to the other. He had decreased truncal and left leg tone. The rest of his neurological examination was unremarkable. MRI demonstrated left partial hemimegalencephaly particularly involving the temporal and occipital regions (Fig. 1). Video electro-encephalography monitoring recorded two predominant seizure types: (i) episodes of eye rolling, nystagmus and staring; and (ii) episodes of eye rolling followed by body spasms. These seizures electrographically localized to the left posterior quadrant. Both magnetoencephalography and positron emission tomography localized the greatest abnormality to the left temporo-occipital region.
展开▼