An Apparently Classical Case Report of Sturge-Weber Syndrome.
Sturge-Weber syndrome is a rare, sporadic, congenital neurocutaneous syndrome, likely due to abnormal development of the cephalic microvasculature. Symptoms and signs depend on the extent and location of the venous dysplasia. We describe a case of a 33-year-old woman presenting with drug-resistant epilepsy, chronic headache, and recurring nonepileptic seizures. Computed tomography and magnetic resonance imaging scans showed severe frontoparietal right hemisphere atrophy, prevalent right frontoparietal leptomeningeal enhancement, circumscribed angioma of the left rolandic sulcus, and prominent deep venous system. We report an apparently classical Sturge-Weber syndrome and hypothesize a shared pathophysiologic mechanism for clinical symptoms. We speculate that all the main symptoms observed in our patient could be the expression of a functional imbalance between the atrophic right hemisphere and the hyperexcitable left cortex.
Institute of Neurology, Catholic University, Rome, Italy nadia.giannantoni@gmail.com.
Nadia Mariagrazia
NM
Giannantoni
Institute of Neurology, Catholic University, Rome, Italy.
Giacomo
G
Della Marca
Institute of Neurology, Catholic University, Rome, Italy.
Catello
C
Vollono
D000328:Adult; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D013341:Sturge-Weber Syndrome / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed
Sturge–Weber syndrome; epilepsy; migraine with aura; nonepileptic seizures