Clin Pediatr Hematol Oncol.  2008 Oct;15(2):127-133.

Cerebral Venous Sinus Thrombosis Associated with Protein S Deficiency in a Child

• Oh YM
• Kim JS
• Jeong JY
• Choi SH
• Park SK

Abstract

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.

Keyword

Venous sinus thrombosis; Protein S deficiency; Child

MeSH Terms

Adolescent
Brain
Child
Colon, Sigmoid
Diagnosis, Differential
Female
Follow-Up Studies
Headache
Humans
Infarction
Korea
Lower Extremity
Neurologic Manifestations
Paresthesia
Phlebography
Plasma
Protein S
Protein S Deficiency
Risk Factors
Sinus Thrombosis, Intracranial
Stroke
Superior Sagittal Sinus
Temporal Lobe
Protein S