Hemodynamic features of non-aneurysmal subarachnoid hemorrhage in a case of familial moyamoya disease. atranscranial doppler ultrasound study

A very rare clinical manifestation of Moyamoya Disease (MMD) is subarachnoid hemorrhage, which is not due to the ruptured intracranial aneurysms (NASAH). This is an extremely rare condition and to our knowledge only five other cases [1,2,3,4,5] have been described in the literature; out of them only one was familial [3]. Moreover, there is currently no study reporting the hemodynamic features of intra and extracranial circulation assessed with ultrasound examination. We present data from a one-year Transcranial Doppler ultrasound (TCD) follow up, in the case of a 57-year-old woman admitted to our hospital for a sudden onset of headache followed by a partial seizure. Family history revealed that two of her cousins had been diagnosed with MMD, one of which deceased at the age of 52 for a subarachnoid hemorrhage (SAH) of unknown cause. On admission, a CT scan revealed SAH over the right frontal cortex and parieto-occipital cortex bilaterally. Digital Subtraction Angiography showed a bilateral stenosis of the supraclinoid segment of the ICAs (for artery acronyms see table 1 legend), and an anomalous Willis circle with absence of the ACAs and of L-MCA and multiple tortuous vessels branching from the R-MCA (‘moyamoya vessels'). No aneurysms or vascular malformations were found (fig. 1).