It is believed that a congenital malformation of the vascular tree is more likely than progressive occlusive disease, and certainly the great majority of cases have shown no clinical progression, though recurrent hemiplegic episodes may be followed by permanent motor deficit and epilepsy.
it seems likely that the onset of symptoms is due to a transient failure of the collateral supply rather than that the disease showed itself at that moment. Now F. Galligioni and his colleagues9 have reported a series of four similar cases from Italy, in wh:ch the e was apparent hypoplasia of the internal carotid artery together with what they term "cerebral pseudoangiomatosis." These were Europeans, and three of them were adults, though again all four were female. These authors believe that a congenital malformation of the vascular tree is more likely than progressive occlusive disease, and certainly the great majority of cases have shown no clinical progression, though recurrent hemiplegic episodes may be followed by permanent motor deficit and epilepsy.6 It is important to be aware of this condition, for it represents one of the rarer findings in cases of acute or recurrent hemiplegia in childhood (and now also in adults), and it is also important that the arteriographic appearances should not mistakenly be interpreted as representing an angioma. Whether these vessels serve a vital function is uncertain, but as they probably represent a collateral supply to a deficient area their presence may be beneficial rather than the potential danger that they might be considered were they part of an angioma.