A rare combination: Sturge–Weber syndrome and accompanying Dyke–Davidoff–Masson syndrome

Excerpt

An 8-year-old male patient was admitted to our hospital with the complaints of medically intractable complex partial seizures. He had a cutaneous vascular nevus on the left side of his face, and his medical history revealed loss of vision in his left eye from the age of two. On video electroencephalogram (EEG) monitoring, sharp wave activity with high amplitude was observed on the left hemisphere. Radiographs demonstrated gyriform, curvilinear, parallel opacities that had the appearance of calcifications (the tram-track sign; Fig. 1a). After informed consent was obtained from the patient’s parents, subsequent to these findings, the patient underwent computerized tomography (CT) examination. CT evaluation revealed cortical calcifications in the left frontoparietal region (Fig. 1b). There was left hemisphere cerebral atrophy with associated expansion of the left frontal sinus, thickening of the left diploic space, and elevation of the petrous ridge (Fig. 1b). On the left side, magnetic resonance (MR) examination demonstrated cerebral hemiatrophy, cortical hypointense calcifications, periventricular leukomalacia, signal void enlarged and tortuous venous vascular structures in the ambient and quadrigeminal cisterns, increase in calvarial bone thickness, and a significant expansion of the left frontal sinus (Fig. 1c, d). There were diffuse hypointense areas, probably consistent with calcifications in the left eye (phthisis bulbi; Fig. 1e).

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