Type III Sturge‐Weber Syndrome With Migraine‐Like Attacks Associated With Prolonged Visual Aura

Abstract

Sturge-Weber syndrome is known to be associated with migraine attacks and prolong aura even without cerebral infarction. We report the case of a 36-year-old woman with type III Sturge-Weber syndrome developing with prolonged left homonymous hemianopsia after an intractable migraine-like headache and becoming a permanent visual field defect at 18-month follow up. By adopting a multimodality imaging study, we suggested that the underlying mechanism of prolonged visual field defect was due to blood flow disturbance and vasogenic leakage under the leptomeningeal angioma combining with atrophy and the damaged integrity of white matter in right occipital lobe.

Sturge-Weber syndrome type 3 manifesting as ‘Status migrainosus’

“…Huang et al 11 described a case of SWS type 3 in a 36-year-old who had been experiencing migraine-like attacks with visual aura and a permanent visual field defect since the age of 10, but unlike our case did not have these symptoms preceding seizure activity. They propose the mechanism for migrainous attacks in SWS to be chronic focal ischaemia and alteration to the venous drainage leading to compromised neuronal function, which may lead to a tendency for migrainous episodes 11.…”

“…They propose the mechanism for migrainous attacks in SWS to be chronic focal ischaemia and alteration to the venous drainage leading to compromised neuronal function, which may lead to a tendency for migrainous episodes 11. Another similarity was the persistence of the visual field defect after the migraine had resolved.…”

Migraine and stroke: In search of shared mechanisms

“…Interestingly, in hereditary hemorrhagic telangiectasia patients without a pulmonary arteriovenous malformation, prevalence of migraine without aura was also higher in one study (52). Outside of hereditary hemorrhagic telangiectasia, an association between migraine and occipital arteriovenous malformations has been noted (53), and migraine is a recognized accompaniment of Sturge-Weber syndrome (54), and has been noted in association with moyamoya disease (55), underscoring the link between vascular structural abnormalities and migraine.…”

Symptomatic migraine: A systematic review to establish a clinically important diagnostic entity

“…. 12,15,16,19,[21][22][23]25,30,31,[38][39][40][41]44,[48][49][50][51][52][53][54][55][56][57] In 21 of these cases, symptoms were side-locked and contralateral to the side of the lesion. Types of lesions were arteriovenous malformations (AVMs) (frontal, n = 1; parietal, n = 1; occipito-parietal, n = 1; occipital, n = 5); occipital astrocytomas (n = 2); brainstem cavernous angiomas (n = 2); carotid artery dissection (n = 2), stenosis (n = 1) and aneurysm (n = 1); hemorrhage due to cerebral amyloid angiopathy (n = 1); unspecified occipital tumor (n = 1); parietal metastasis (n = 1); parietooccipital leptomeningeal angioma (n = 1); and occipital brain infarction (n = 1).…”