[Moyamoya disease associated with persistent primitive trigeminal artery--a case report and review of literature]

A case of moyamoya disease associated with the persistent primitive trigeminal artery (PTA) is reported. 5 such cases reported in literature are reviewed. A 16-year-old female patient was admitted to our clinic, having complained of right hemiparesis for 8 years. CT scan revealed multiple low density areas in the left frontal, temporal and parietal lobes. In left carotid angiogram, the internal carotid artery was occluded at the C3 portion and basal moyamoya (stage IV) was found. In right carotid angiogram, the internal carotid artery was occluded at the C3 portion and basal moyamoya (stage V) was found. Furthermore the persistent primitive trigeminal artery was seen with marked moyamoya vessels from the posterior cerebral artery. In vertebral angiogram, the posterior cerebral artery was seen bilaterally with moyamoya vessels. In 133Xe-rCBF study, compared with the mean flow in each hemisphere, the flow decreased at the temporal lobe, but, in contrast, increased at the parieto-occipital lobes. Reviewing the literature, the following conclusions are suggested. 1. Persistent primitive trigeminal artery will promote the stage of moyamoya disease. 2. It will protect the brain from hemorrhage and/or ischemia in moyamoya disease.