Sturge-Weber syndrome type 3 manifesting as 'Status migrainosus'

Abstract

Sturge-Weber syndrome (SWS) is a rare neurocutaneous syndrome characterised by facial naevus and leptomeningeal angiomatosis resulting in neurological and ophthalmological complications. In its rare variant, SWS type 3, the clinical hallmark of facial naevus is absent which poses a diagnostic challenge. Here, we present an interesting case of SWS type 3 where a child presented twice with prolonged severe unilateral headache mimicking migraine status followed on both occasions with focal seizures. He developed a dense right-sided homonymous hemianopia, and an urgent brain MRI scan was performed which pointed towards the diagnosis of SWS type 3.

Figure 1

CT head showing focal areas of cortical/subcortical calcification in the left occipital lobe.

Figure 2

(A) MRI head—precontrast sagittal section. MRI head—postcontrast sagittal section (B) and postcontrast axial imaging (C)—showing leptomeningeal enhancement within subarachnoid spaces/sulci of occipital and parietal lobe that is consistent with pial angiomatosis.