JLE

Epileptic Disorders

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Hemiconvulsion-Hemiplegia-Epilepsy in a girl with cobalamin C deficiency Volume 20, issue 6, December 2018

Figure 1

EEG following status epilepticus presentation. On Day 1 of acute presentation, EEG showed periodic lateralized epileptiform discharges (PLEDs) over the left hemisphere (A). By Day 3, the PLEDs were more localized over the left temporal region, and higher amplitude slowing had developed over the right hemisphere (B). By Day 4, left-sided PLEDs had resolved, replaced with diffuse suppression, and high-amplitude polymorphic slowing continued over the right hemisphere (C).

Figure 2

Neuroimaging following status epilepticus presentation. Brain MRI on Day 2 of acute presentation showed diffuse cytotoxic oedema of the left hemisphere, demonstrated by hyperintensity on T2-weighted sequences (A), hypointensity on apparent diffuse coefficient mapping (B), and hyperintensity on diffusion weighted imaging sequences (C). Head CT on Day 5 demonstrated increased swelling of the left hemisphere, now with herniation causing mass effect on the right hemisphere (D). This pressure was partially alleviated by a left craniectomy (E). Repeat T2-weighted MRI, 39 days after initial presentation, showed resolution of the mass effect and early signs of left hemisphere atrophy (F).