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KMID : 0387820080150020127
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Clinical Pediatric Hematology-Oncology
2008 Volume.15 No. 2 p.127 ~ p.133
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Cerebral Venous Sinus Thrombosis Associated with Protein S Deficiency in a Child
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Oh Yeon-Mi
Choi Seong-Hoon
Park Sang-Kyu
Jung Jin-Young
Kim Joon-Sung
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Abstract
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Cerebral venous sinus thrombosis (VST) is a disease with multiple etiologies that present with a wide spectrum of clinical signs and symptoms. Cerebral VST associated with protein S deficiency in children has not yet been reported in Korea. A 15-year-old female with out any known risk factors for stroke was presented with severe headache and paresthesia of the left lower extremity. Brain MRI with venography revealed multiple lesions of cerebral VST on the left transverse sinus, sigmoid sinus, and superior sagittal sinus. It also showed a venous hemorrhagic infarction in the left temporal lobe. The initial plasma levels of protein S antigen, free protein S, and protein S activity were 34% (normal, 60∼150%), 32% (normal, 70∼130%), and 25% (normal, 60∼140%), respectively. Anticoagulation therapy was performed for 4 months, and consequently the patient showed a remarkable improvement of neurologic symptoms. Follow-up plasma levels of protein S antigen, free protein S, and protein S activity were still low as 45%, 27%, and 44%, respectively. Protein S deficiency should be included in the differential diagnosis of cerebral VST in children.
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KEYWORD
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Venous sinus thrombosis, Protein S deficiency, Child
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