This paper is only available as a PDF. To read, Please Download here.
Cerebral infarction is a rare pathology among children and its etiology can be identified in almost two-thirds of cases. The remaining one-third are considered idiopathic. Recently, inherited disorders of blood coagulation predisposing to thrombosis have been taken into account as a possible cause of childhood stroke. We describe here a case of a 6-year-old child presenting with ischemic stroke and protein S (PS) defect. The family study suggested inheritance of the defect. The immunological characterization of PS in the affected family members was consistent with a defect mainly in the free form of PS. In the case here reported no associated predisposing condition to stroke could be identified but familial PS defect was found. No therapy was administered. Nevertheless symptoms disappeared spontaneously and there were no recurrences at the 1 year follow-up. Diagnostic imaging techniques demonstrated that a reduction in the cerebral ischemic area had occurred 2 months after the stroke.
To read this article in full you will need to make a payment
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:Subscribe to Brain and Development
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- The protein C anticoagulant pathway.Arteriosclerosis Thrombosis. 1992; 12: 135-145
- Familial protein S deficiency is associated with recurrent thrombosis.J Clin Invest. 1984; 74: 2082-2088
- Hereditary protein S deficiency: clinical manifestations.Ann Intern Med. 1987; 106: 677-682
- Occurrence of arterial thrombosis in a cohort of patients with hereditary deficiency of clotting inhibitors.J. Med. 1992; 23: 61-74
- Hereditary protein S deficiency in young adults with arterial occlusive disease.Thromb Haemost. 1990; 64: 206-210
- Arterial thrombosis and protein S deficiency.Thromb Haemost. 1989; 62: 1040
- Severe arterial cerebral thrombosis in a patient with protein S deficiency (moderately reduced total and markedly reduced free protein S): a family study.Thromb Haemost. 1989; 61: 144-147
- Stroke and familial protein S deficiency.Stroke. 1992; 23: 299
- Cerebral infarction and familial protein S deficiency.Stroke. 1990; 21: 1760-1761
- Childhood stroke associated with protein C or deficiency.J. Pediatr. 1987; 111: 562-564
- Inherited hypercoagulable states in children.Am J Pediatr Hemat Oncol. 1989; 11: 170-173
- Further studies on the abnormal factor X (Factor X Friuli) coagulation disorder. A report of another family.Blood. 1979; 37: 534-541
- A family with abnormal protein C (Protein C Padua) and a thrombotic tendency.Haematologia. 1993; 25: 25-33
- Combined inherited protein S and heparin co-factor II deficiency in a patient with upper limb thrombosis: a family study.Thromb Res. 1992; 67: 23-30
- Quantitative estimation of proteins by electrophoresis of human serum proteins.Anal Biochem. 1966; 15: 45-52
- Familial thrombophilia due to a previously unrecognized mechanism characterized by poor anticoagulant response to activated protein C: prediction of a cofactor to activated protein C.in: 5th edn. Proc Natl Acad Sci USA. 90. 1993: 1004-1008
- Stroke in children. Clinical characteristics and prognosis.Acta Paediatr Scand. 1983; 72: 391-395
- Cerebrovascular disease in infants and children. a study of incidence, clinical features, and survival.Neurology. 1978; 28: 763-768
- Coagulopathies and vasculopathies.in: Raimondi AJ Choux M Di Rocco C Cerebrovascular diseases in children. Springer, New York1992: 188-205
- The antiphospholipid syndrome: ten years on.Lancet. 1993; 342: 341-344
- Childhood stroke associated with protein C or S deficiency and primary antiphospholipid syndrome.Pediatr Neurol. 1993; 9: 67-70
- Antiphospholipid antibodies and stroke in an infant.Pediatrics. 1991; 87: 933-935
- Role of protein C in childhood cerebrovascular occlusive accidents.Eur J Pediatr. 1989; 149: 216-218
- The hereditary thrombosis.in: Neri Serneri GG Gensini GF Abbate R Prisco D Thrombosis: An update. Scientific, Florence1992: 89-106
Accepted: January 15, 1994
Received: September 21, 1993
© 1994 Elsevier Science B.V. All rights reserved. Published by Elsevier Inc.