Presentation
Seizures
Patient Data
Age: 30 years
Gender: Male
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Diminished volume with mild atrophic changes are noted the left cerebral hemisphere compared to the right side showing relatively dilated lateral ventricle and prominent cortical sulci, associated with :
- coarse cortical tram-track calcification involving the parieto-occipital region, evident on SWI and confirmed by a recent CT (not available)
- prominent leptomeningeal enhancement along the left cerebral hemisphere notably the frontal and partieto-occipital regions with prominent related cortical vessels
- left sphenoid wing dysplasia
- left anterior temporal arachnoid cyst is noted averaging 4.5 x 4 cm in axial dimensions, indenting the underlying temporal gyri
- thickened areas of the scalp over the left frontal and parietal regions, refer to associated cutaneous lesions
Case Discussion
Overall features are suggestive of Sturge-Weber syndrome with pial angiomas. The patient has in addition ipsilateral sphenoid wing dysplasia and temporal arachnoid cyst which are uncommonly reported associations 1.
Sphenoid wing dysplasia is a characteristic but not pathognomonic feature of neurofibromatosis type 1 (NF1), also a rare association with Sturge-weber syndrome could occur 2.
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