Introduction. Sturge-Weber syndrome is a neurocutaneous disorder
characterized by neurological features such as headaches, developmental
delay, mental retardation and seizures, facial anomalies such as port-wine
stain and glaucoma which is the most common ocular manifestation. The main
neuroimaging findings in patients with Sturge-Weber syndrome are
leptomeningeal angiomatosis and corticopial calcifications associated with
underlying cortical atrophy. The purpose of this report is to present a rare
case of a patient with seizures whose magnetic resonance imaging findings
suggested a Sturge-Weber syndrome variant. Case Report. We report a case of
a 14-yearold boy with a two year history of well controlled generalized
tonic clonic seizures with visual aura, who was admitted to our institution
for neuroimaging examination. Neuropsychological testing showed normal
cognitive and psychomotor development. Electroencephalography revealed
unilateral runs of right occipital spikes with secondary generalization.
Neuroimaging findings showed focal cerebral leptomeningeal enhancement in
the right parasagittal occipital region associated with focal cortical
atrophy, whereas susceptibility weighted imaging showed hypointense
intracortical calcification and hyperplastic enhanced ipsilateral choroid
plexus. The computed tomography confirmed cortical calcifications. Also, an
overlying parietooccipital subcutaneous lipoma was found in the innervation
field of the ophthalmic nerve. Conclusion. Magnetic resonance imaging is the
key imaging modality that confirmed the clinical suspicion of Sturge-Weber
syndrome based on a physical and neurological examination. Neither magnetic
resonance imaging nor clinical examination is sufficient for a correct
diagnosis.
Type III roach scale Sturge-Weber syndrome associated with an extracranial lipoma: A case report
