The
Sturge-Weber syndrome was recently subdivided into type I (facial and leptomeningeal
angioma, possible
glaucoma), type II (facial
angioma, without evident endocranial involvement), and type III (exclusive leptomeningeal
angioma). Thus far in the literature only 24 cases of
Sturge-Weber syndrome type III have been reported. This study presents a case of a 2-year 9-month-old child with normal psychomotor development and skin free (no angiomas), who presented repeated episodes of severe
headache, vertiginous symptoms,
vomiting, and drowsiness, separated by complete recovery. The cranial computed tomography and magnetic resonance imaging with
gadolinium revealed left occipital leptomeningeal
angiomatosis with calcifications, suggesting a diagnosis of
Sturge-Weber syndrome type III. Considering the normal psychomotor development, the improved electroencephalographic reports between the episodes, and the absence of hypoperfusion areas on single-photon emission computed tomography at 30 months of follow-up, the symptomatology appears an expression of
migraine-like symptoms resulting from vasomotor disturbances within and around the
angioma, more than an expression of
partial seizures arising through an epileptic focus in the ischemic region around the
vascular malformation.