case_images: PMC5832162_01
case_images: EEG studies.
case_images: CRICC2018-5053175.001.jpg
case_images: PMC5832162_01_CRICC2018-5053175.001.jpg
case_images: fig1
case_images: Initial EEG on his second hospital day showed focal seizures emanating from the left frontal region (Figure 1(a)) and he was started on oral levetiracetam (loading dose of 1 gram followed by 750 mg PO twice daily thereafter). His cognition and right upper extremity shaking/tremor rapidly worsened and, on hospital day #4, EEG was significant for persistent focal seizures in left hemisphere despite being on levetiracetam (Figure 1(a)). Continuous EEG revealed persistent epileptogenic activity with bilateral hemispheric discharges (left > right) (Figure 1(b)). EEG (Figure 1(c)) showed "diffuse epileptiform discharge suggestive of encephalopathic state with presence of continuous left frontal and sometimes synchronized bifrontal sharps spikes with a more generalized appearance, which was concerning for nonconvulsive status epilepticus." Perampanel was added to his antiseizure regimen at dose of 4 mg twice daily. Efficacy of perampanel has been established as an adjunct treatment for partial-onset seizures with or without secondary generalization and primary generalized tonic-clonic seizures in idiopathic generalized epilepsy as well as for treatment of refractory seizures. He remained in a persistent coma at this point and lost reflexes to even deep painful stimuli while cEEG showed continuous episodic sharp wave from left frontal and synchronized bifrontal discharge was obvious on lowering his sedation (Figure 1(c)). His neurological condition did not improve with EEG persistently showing frequent generalized epileptiform discharges (Figure 1(d)). EEG (Figure 1(d)) at that time showed a worsening seizure activity pattern which initially started as focal left frontal lobe diffuse spike-wave complexes but now had progressed into generalized epileptiform discharges with development of nonconvulsive status epilepticus (NCSE).
case_images: MRIs.
case_images: CRICC2018-5053175.002.jpg
case_images: PMC5832162_01_CRICC2018-5053175.002.jpg
case_images: fig2
case_images: Initial MRI of brain during this hospital encounter was performed which did not show any acute intracranial lesion except mild cerebral and cerebellar substance loss (Figure 2(a)). A repeat MRI brain with contrast on hospital day #5 showed developing cytotoxic edema in the left frontal and parietal lobe with punctate calcified lesions in right cortex (Figure 2(b)) which was considered likely due to persistent seizure activity. Repeat MRI brain (Figure 2(d)) showed extensive and persistent worse cortical ribboning particularly around the left hemisphere, and involving right frontal, and limited involvement of the basal ganglia without any acute infarct on diffusion weighted imaging (DWI) (suggestive of cytotoxic edema from prolonged seizure activity and early CJD).