Moyamoya disease associated with positive findings for rheumatoid factor and myeloperoxidase-anti-neutrophil cytoplasmic antibody / La enfermedad de moyamoya asociada con hallazgos positivos en relación con el factor reumatoideo y los anticuerpos citoplasmáticos antineutrófilos mieloperoxidasa

A 39-year old female suddenly fell into a state of unconsciousness. She had no significantpast medical history. A computed tomography scan of the head demonstrated a massive left putaminal haemorrhage with a ventricular perforation, low density areas in the right frontal lobe, corona radiata and occipital lobe. A single emergency burr hole drainage of the haematoma was performed. Bilateral common carotid arteriograms showed stenosis of the right internal carotid artery and a complete obstruction of left internal carotid artery which were both accompanied by moyamoya vessels. The biochemical studies indicated serological positive findings for RF and MPO-ANCA. She was transferred to another hospital for nursing care in a vegetative state on the 163nd hospital day. This case indicates that immunological factors, inflammation or vasculitis might have possibly been associated with the development of either an obstruction or stenosis of the intracranial internal carotid arteries.

Posterior circulation abnormalities in moyamoya disease : a radiological study.

Moyamoya disease (MMD) is an uncommon entity outside Japan. Though the clinical and radiological features are well described, involvement of the posterior circulation has not been highlighted. Out of 10 patients of MMD studied, the posterior circulation was involved in 9 (3 bilateral, 6 unilateral). The P1 segment was most commonly affected. Interestingly, no infarcts were seen in the territory of the posterior circulation in any patient. Five patients showed recent haemorrhages on scan. It was thalamic haemorrhage in four and subarachnoid in one patient. The posterior circulation is frequently involved in MMD as evident on angiography. However, ischaemic events of the posterior circulation are not frequent, as the posterior circulation acts as collateral pathway for the diseased anterior circulation till later stages of the disease.