Headache in Sturge-Weber syndrome: a case report and review of the Literature

Sturge–Weber syndrome, defined also as encephalotrigeminal or leptomeningeal angiomatosis, is a nonhereditary congenital neurocutaneous disorder, characterized by a facial vascular nevus (port-wine stain) that follows the distribution of the trigeminal nerve, associated with an ipsilateral leptomeningeal angioma. Neurological manifestations include seizures (in 80% to 90% of cases), mental retardation (in 40% to 50% of cases) and focal signs, such as hemiplegia, aphasia or hemianopia, that are often fluctuating (1, 2). In previous isolated studies the association of the syndrome with headache, in particular migraine with aura, was reported, but poorly documented (3). In a series of 23 epileptic patients with Sturge–Weber syndrome it was noted that 7 cases had episodes of throbbing unilateral headache with autonomic symptoms and transient hemiparesis. None of these episodes was preceded or accompanied by loss of consciousness or ictal phenomena such as clonic jerks. It was suggested that these attacks were most likely episodes of hemiplegic migraine rather than epileptic seizures (2). The prevalence of migraine in Sturge– Weber syndrome was seen to be higher than in normal population (4). A patient with this syndrome, free of epileptic seizures for 10 years, started suffering from hemiplegic migraine attacks, without a family history for this disorder (5). Conversely, no case of headache was reported in a long-term outcome study regarding 52 adults with Sturge–Weber syndrome (1). The headache secondary to encephalotrigeminal angiomatosis was not included in the first edition of the International Headache Society (IHS) classification. In the second edition this headache was finally classified in group 6 (section 6.3.5), which includes the secondary headaches attributed to cranial or cervical vascular disorders (6).