[Unilateral moyamoya disease associated with acrofacial vitiligo in a 13-year-old patient--case report]

Cerebrovascular Moyamoya disease (MMD) is a clinical entity characterized by stenotic or occlusive lesions around the terminal portions of the internal carotid arteries (ICA) and the formation of abnormal vascular networks at the base or convexity. This condition is sporadic, and there are no pathological explanations for this cause. A case with unilateral juvenile MMD is reported. A 13-year-old girl with sudden episode of left limbs weakness, accompanied by transient dysphasia and seizures attack, was admitted to our hospital for further investigation. Computed tomography scanning showed an area of localized brain swelling in the right capsular-thalamic region. Angiographic findings demonstra-ted unilateral involvement. Cerebral panangiography reve-aled a high grade stenosis of the right supraclinoid ICA and a peculiar network (moyamoya phenomenon) formed by dilated basal collateral perforating arteries and produced a cloudy image resembling "a puff of smoke". Left carotid angiogram showed a collateral circulation through the anterior communicant artery and apportioned to the right cerebral hemisphere. The vertebrobasilar system was normal. An ultrasoud Doppler study demonstrated a severely decreased blood flow gradient (0.1 l/min) through the right hypoplastic ICA, while the left common and the left ICA showed remarkable increased blood flow, partly due to abondant collateral vessels. Acquired, non-progressive vitiligo with areas of complete pigment loss appeared on flexor wrists, extensor distal extremities, superior eyelids and especially on joints regions (elbow, knee and small joints of the hands and fret). This association has not been reported previosly.