| Abstract [eng] |
Moyamoya angiopathy is a rare chronic cerebral arteriopathy characterised by progressive steno-occlusive changes of the internal carotid arteries and their major branches as well as pathological compensatory collateral network formation. Usually it presents with ischemic and haemorrhagic events, and headaches. It is classified into Moyamoya disease (presents only as an intracranial angiopathy) and Moyamoya syndrome (caused by acquired or genetic disorders). This paper presents two cases – one disease, one syndrome. First, a 22-year-old male diagnosed with the disease at age 7–8, presented with strong, pulsating headaches. Magnetic resonance imaging revealed stenosis bilaterally in the middle cerebral arteries and in the right anterior cerebral artery as well as multiple lenticulostriate and leptomeningeal collaterals; transcranial colour-coded duplex sonography – characteristics of Moyamoya angiopathy. Second, a 51-year-old male diagnosed with the syndrome after a transient ischaemic attack in the right middle cerebral artery, presented with episodic numbness of his left arm and face, aphasia, and disorientation. Magnetic resonance imaging revealed stenosis in both internal carotid arteries as well as the right anterior and middle cerebral arteries; transcranial colour-coded duplex sonography – reverse flow in the anterior cerebral artery, the same stenosis, and collateralization through the posterior communicating artery. Generally, diagnostic testing searches for signs of Moyamoya disease or syndrome. Due to its accuracy, digital subtraction angiography is the gold standard for Moyamoya, but it is invasive. High resolution magnetic resonance vessel wall imaging is especially promising, although not easily accessible. Transcranial colour-coded duplex sonography could be used for screening and follow-up. |
