PROGRESSIVE INTRACRANIAL OCCLUSIVE DISEASE-ASSOCIATED WITH DEFICIENCYOF PROTEIN-S - REPORT OF 2 CASES

Background and Purpose: Deficiency of the free fraction of protein S h as been associated with arterial or venous stroke. The pathogenesis of vascular occlusion in patients with protein S deficiency is not known . We present two cases of cerebral infarction and deficiency of protei n S in which the subjects had progressive intracranial occlusions. Cas e Description: A 16-year-old girl was admitted because of left brain s tem infarction and protein S deficiency. Cerebral angiography disclose d stenosis of the right intracranial vertebral artery and occlusion of the left posterior cerebral artery. A second angiogram performed 18 m onths later disclosed occlusion of the right vertebral intracranial ar tery. In the second case, a 17-year-old girl was admitted because of l eft hemispheric cerebral infarction and protein S deficiency. Cerebral angiography showed stenosis of the left anterior cerebral artery, lef t supraclinoid internal artery, and left middle cerebral artery. A sec ond cerebral angiogram performed 5 months later disclosed occlusion of the left anterior cerebral artery and poor hemispheric perfusion thro ugh the left middle cerebral artery. Conclusions: Based on our cases, we postulate that some patients with prothrombotic states may develop progressive intracranial arterial occlusions, possibly secondary to a permanent thrombogenic stimulus. We suggest routinely searching for pr othrombotic states in young patients with intracranial occlusion, espe cially if the occlusion is progressive and other causes are not obviou s.