A 9-year-old boy with facial port-wine stain and seizures undergoes non-contrast CT head, which shows curvilinear gyriform calcification in the left parieto-occipital cortex with underlying cerebral hemiatrophy and thickened calvarium on the same side. What is the most likely diagnosis?
- A Sturge-Weber syndrome ✓
- B Tuberous sclerosis
- C Neurofibromatosis type 1
- D Von Hippel-Lindau disease
Correct answer: A. Sturge-Weber syndrome
Explanation
Gyriform 'tram-track' cortical calcification in the parieto-occipital region, ipsilateral cerebral hemiatrophy and compensatory calvarial thickening are classic findings of Sturge-Weber syndrome, caused by a persistent primordial leptomeningeal venous plexus producing chronic venous congestion and cortical hypoxia. The port-wine trigeminal distribution clinches it. Tuberous sclerosis produces subependymal calcified nodules rather than gyriform calcification, and neither NF1 nor VHL causes cortical tram-tracking.
Reference: Osborn's Brain, 2nd ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.