Moyamoya Syndrome Associated with Optic Nerve Coloboma and Mental Retardation

448 Mario Siebler, MD Department of Neurology, Heinrich Heine University Moorenstrasse 5 DE–40225 Düsseldorf (Germany) Tel. +49 211 81 18464, Fax +49 211 81 18485 E-Mail [email protected] bral digital subtraction angiography revealed a bilateral occlusion of the internal carotid arteries and the right vertebral artery, with the retrograde right ophthalmic artery as the only vessel supplying the brain. Further MRI sequences showed, besides old left hemispheric infarcts, new ischemic lesions in the right centrum semiovale, pointing to a recent watershed infarct ( fig. 1 b). 1 HMRS revealed a lactate peak in these ischemic areas ( fig. 1 c, d). A 550 MBq 99m Tc-HMPAO brain perfusion scintigraphy showed hypoperfusion in both middle and anterior cerebral artery territories with severely impaired functional cerebrovascular reserve capacity. Because of the hemodynamic cause of these infarcts, early treatment consisted of antiplatelet agents and hypotension prevention. We decided to support the oxygen supply of the brain with HBOT in order to bridge the time until neurosurgical treatment with an extracranial to intracranial bypass. Hyperbaric oxygen treatment units were administered once daily for 8 days. Each treatment unit consisted of 90 min surplus pressure with 100% oxygen at 2.0 bar in a hyperbaric chamber (Sayers-Hebold, Germany). Pressure and duration of each HBOT session were chosen according to human and experimental animal data in cerebral ischemia [1–3, 6] . After 8 hyperbaric oxygen treatment units and 11 days, an MRI and 1 H-MRS detected neither new ischemic lesions nor lactate peaks in the same areas ( fig. 1 e, f) and the patient showed clinical improvement. The patient was then treated with an extracranial to intracranial bypass, which preserved the spectroscopic and clinical result.