THROMBOPHILIA FOUND IN PATIENTS WITH MOYAMOYA-DISEASE

Sixteen patients with Moyamoya disease and foul-with quasi-Moyamoya di sease were investigated in order to elucidate the presence of thrombop hilia. The assay system for diagnosing thrombophilia consisted of asse ssing both the activity and antigen levels of antithrombin III, protei n C, protein S, fibrinogen and plasminogen as well as detecting lupus anticoagulants. The analysis revealed that one third (four definite ca ses and three quasi-cases) of the examined patients demonstrated eithe r congenital or acquired thrombotic tendency. Protein C deficiency was found in two definite cases and in two quasi-cases among whom one qua si-case was identified to have a hereditary type I Protein C deficienc y. Protein S deficiency was found in one definite case and in one quas i-case. Type II plasminogen deficiency was found in one quasi-case, an d lupus anticoagulant was present in one quasi-case. Based on these fi ndings, an evaluation of thrombophilia should thus be performed when b oth diagnosing and treating suspected cases of Moyamoya disease. (C) 1 997 Elsevier Science B.V.