Fatal hemorrhagic stroke in a Caucasian girl with moyamoya disease

Moyamoya disease (MMD) is an extremely rare neurovascular disorder in Caucasian children. To the best of our knowledge, the aggressive variant including hemorrhagic malignant stroke and consecutive global ischemia has not been reported for this population before. We present the case of an 11-year-old girl with sudden neurological deterioration due to intracerebral hemorrhage with early irruption into the ventricular system. MMD with extensive neovascularization was diagnosed by means of computed tomography and magnetic resonance imaging. Despite immediate ventricular drainage, intracranial pressure increased above the mean arterial pressure resulting in malignant bi-hemispheric ischemia. The girl died within 53 h after admission to hospital. Intracerebral hemorrhage in young patients is often attributed to vascular malformation. This case shows that MMD may constitute a potential diagnosis in the case of sudden neurological deterioration and loss of consciousness, even in previously healthy children.