JLE

Epileptic Disorders

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ITPA-associated developmental and epileptic encephalopathy: characteristic neuroradiological features with novel clinical and biochemical findings Volume 24, issue 3, June 2022

Figure 1.

A prolonged EEG recording (sensitivity: 10 mV; low filter: 1 Hz; high filter: 70 Hz; paper speed: 30 mm/sec). (A) Normal awake interictal background with a mix of diffuse posterior 2-3-Hz high-amplitude delta activity with faster 4-5-Hz theta intermixed more anterior activity, with no persistent asymmetries. (B) Ictal onset from the right hemisphere associated with a change in background to rhythmic fast 4-4.5-Hz sharp-wave activity in the right mid-temporal region, which later slows down (C, D). (E) Ictal onset of a rhythmic sharp-wave activity at 4-5 Hz seen from the left hemisphere while slower activity is seen on the right hemisphere. (F) Slower sharp-wave activity at a frequency of 3-4 Hz seen in the left temporal region, which later resolves in the same epoch while the right hemisphere shows normal baseline activity.

Figure 2.

(A-D) Patient 1: T2 axial and diffusion-weighted images showing T2 hyperintensity in the posterior limb of the internal capsule (arrow [A]) with diffusion-restriction (arrow [B]) of the same region. At 18 months of age, brain MRI in the same patient shows diffuse cortical atrophy on T2 axial image (C) and resolution of diffusion-restriction in the previously involved area (D). C-H) Patient 2: T2 axial and diffusionweighted images showing T2 hyperintensity in the posterior limb of the internal capsule (E) and dentate nucleus (G) with diffusion-restriction in the same areas (F, H).