OCCLUSIVE DISEASE OF INTRACRANIAL MAIN ARTERIES WITH COLLATERAL NETWORKS (MOYAMOYA DISEASE) IN ADULTS

Abstract

Three adult cases of occlusive disease of the intracranial main arteries with collateral networks (moyamoya disease) are reported. Each patient had a characteristic angiographic picture: occlusion of the internal carotid artery at the level of the siphon just above the origin of the posterior communicating artery, combined with typical collateral networks in the base of the brain and in the convexity. Transitory ischaemic attacks occurred in the initial phase of the disease. One female patient had her initial transitory symptoms during the use of contraseptive pills. Her first objective neurological deficits, spastic hemiparesis and dysphasia, occurred half a year later, some days after discontinuation of the pills. Later she had a spontaneous intracerebral and subdural haematoma; she was then treated conservatively. The other female patient also exhibited severe dysphasia combined with a slight hemisyndrome. Biopsy of the temporal artery showed marked intimal proliferation in this female case only. The male patient suffered mainly from neurotic and depressive symptoms. Brain scanning suggested cerebral infarction, which disappeared later. EEG findings varied from slight asymmetry to focal changes. At follow-up examination the male patient was able to work, but both females were unable to care for themselves. Several aetiological factors possibly operate in the background of the disease, which does not appear to be confined to any particular race.