[Focal chronic ischemia and concomitant migraine: an atypical form of Sturge-Weber angiomatosis?]. / Ischémie chronique focale et migraine accompagnée: forme atypique d'une angiomatose de Sturge-Weber?

A young man with a left hemifacial hemangioma had during a six months period about forty left hemispheric neurologic attacks suggestive of classic migraine. The neurologic examination was normal during the attack-free period. The CT scan (fig. 1) and the M.R.I. study (fig. 2) only showed a moderate interhemispheric asymmetry. The left internal carotid angiogram showed subtle anomalies of the venous system (fig. 3). All the neurologic manifestations ceased as soon as therapy by aspirin was initiated. A Positron Emission Tomography (PET) study with the oxygen 15 continuous inhalation technique was performed 7 months after the last attack in order to measure the regional Cerebral Blood Flow (rCBF), Oxygen Extraction Fraction (rOEF) and Oxygen Consumption (rCMRO2). Striking, statistically significant, alterations were observed in the left temporo-parieto-occipital area (fig. 4) consisting of a "misery perfusion" syndrome (rCBF = 28-38 ml/100 g/mn; rOEF = 0.64-0.80), without alteration in the rCMRO2 (Table). A repeated PET study 12 months later was unchanged. The association of local chronic oligemia and ipsilateral facial hemangioma, ipsilateral cerebral hypotrophy and venous anomalies suggested the diagnosis of atypical leptomeningeal angiomatosis of the Sturge-Weber type. The importance and persistence of the hemodynamic alterations suggest that chronic oligemia and, hence, tissue hypoxia may participate in the pathogenesis of the migraine-like attacks. Moreover, local circulatory stasis with thrombotic events may be implicated, as suggested by the apparent efficacy of aspirin.

Cilio-optic vein associated with phakomatosis.

This presentation demonstrates the fluorescein angiographic characteristics of the cilio-optic vein. These congenitally enlarged vessels appear at the disc edge and dip into the optic nerve to anastomose with branches of the central retinal vein. Fluorescein angiography shows lamellar filling of the vessels in the early choroidal phase. In one patient, these findings are demonstrated bilaterally with the use of bilateral simultaneous angiography. A second type of communication is presented, a retinociliary vein which drains the retina into the choroidal circulation. In both types presented, a branching hypofluorescent pattern extends from the disc vessel into the choroid. Our patients have evidence of a phakomatosis: neurofibromatosis or Sturge-Weber syndrome. This anomalous disc vessel should not be confused with optociliary shunt, disc neovascularization, cilioretinal artery, or arteriovenous shunt.