PMC4389202_01
A and B. EEG findings: A: EEG revealed recurrent focal seizures with left hemispheric onset and evolution, fulfilling the criteria for focal status epilepticus. B: Interictal left posterior quadrant sharp waves.
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Electroencephalography revealed recurrent focal seizures with left hemispheric onset and evolution, fulfilling the criteria for focal SE each time (Fig. 1).
Imaging: Brain MRI repeatedly showed extensive abnormalities in the periventricular and deep white matter, subcortical white matter, and bilateral basal ganglia.
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Brain MRI repeatedly showed extensive abnormalities in the periventricular and deep white matter, subcortical white matter, and bilateral basal ganglia (Fig. 2).
Skin biopsy findings: Electron microscopic images showed striking gaps and discontinuities in the perivascular smooth muscle layer, vacuolization within the smooth muscle, and numerous deposits of granular osmiophilic material (red circles) : much of which, because of the degree of vascular wall breakdown, was free-floating.
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Skin biopsy was obtained and returned positive; electron microscopic images showed gaps and discontinuities in the perivascular smooth muscle layer with numerous deposits of granular osmiophilic material (Fig. 3).