Two prospective, randomized studies using human recombinant lysosomal -galactosidase A found a reduction in microvascular deposits and reduced plasma levels of globotri- These studies had short follow-up periods, and no reduction in stroke incidence was found. Agalsidase- and agalsidase- given at the same dose of 0.2 mg/kg have similar short-term effects in reducing left ventricular mass.85,92 Many coagulopathies are inherited as autosomal-dominant These disorders, including protein C and S deficien- cies, the factor V Leiden mutation, and various other factor deficiencies, can lead to an increased risk of cerebral venous As discussed below, there has not been a strong association between several of these disorders and arterial events such as MI and ischemic stroke.98,99 Some apparently acquired coagulopathies such as the presence of a lupus anticoagulant or anticardiolipin antibody (aCL) can be familial in 10% of cases.100,101 Inherited disorders of various clotting factors (ie, factors V, VII, X, XI, and XIII) are auto- somal-recessive traits and can lead to cerebral hemorrhage in infancy and childhood.102 Arterial dissections, moyamoya syndrome, and fibromuscular dysplasia have a familial com- Intracranial aneurysms are a feature of certain mendelian disorders, including autosomal-dominant polycystic kidney disease and Ehlers-Danlos type IV syndrome (so-called vas- cular Ehlers-Danlos). Intracranial aneurysms occur in 8% of individuals with autosomal-dominant polycystic kidney disease and 7% with cervical fibromuscular dysplasia.105,106 Ehlers-Danlos type IV is associated with dissection of verte- bral and carotid arteries, carotid-cavernous fistulas, and intra- Loss-of-function mutations in KRIT1, malcavernin, and PDCD10 genes cause cerebral cavernous malformation syn- Mutations in can cause inherited cerebral amyloid angiopathy syndromes.109 Genetic Factors: Summary and Gaps The cause of ischemic stroke remains unclear in as many as 35% of patients. 