Skip to main content
British Medical Journal (Clinical Research Ed.) logoLink to British Medical Journal (Clinical Research Ed.)
. 1985 Feb 2;290(6465):350–352. doi: 10.1136/bmj.290.6465.350

Cerebral haemorrhagic infarction in young patients with hereditary protein C deficiency: evidence for "spontaneous" cerebral venous thrombosis.

A R Wintzen, A W Broekmans, R M Bertina, E Briët, P E Briët, A Zecha, G J Vielvoye, G T Bots
PMCID: PMC1417351  PMID: 3917815

Abstract

Among 53 patients with hereditary protein C deficiency belonging to 20 families three women were encountered who, aged 27, 34, and 38 respectively, had had cerebral haemorrhagic infarction, probably due to intracranial venous thrombosis. All three had also had venous thrombosis of the leg and pulmonary embolism either before or after their cerebral infarction. One patient sustained cerebral infarction while receiving an oral contraceptive, but infarction in the two others occurred "spontaneously." One patient also had an intraventricular and subarachnoid haemorrhage during the induction phase of coumarin treatment, which was assumed to have resulted from haemorrhagic infarction of the chorioid plexus, analogous to coumarin provoked haemorrhagic skin necrosis in protein C deficiency. Hereditary protein C deficiency should be considered in young patients with acute or subacute cerebral symptoms, especially if they have a family or personal history of venous thromboembolism.

Full text

PDF
350

Images in this article

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ambruso D. R., Jacobson L. J., Hathaway W. E. Inherited antithrombin III deficiency and cerebral thrombosis in a child. Pediatrics. 1980 Jan;65(1):125–131. [PubMed] [Google Scholar]
  2. Broekmans A. W., Veltkamp J. J., Bertina R. M. Congenital protein C deficiency and venous thromboembolism. A study of three Dutch families. N Engl J Med. 1983 Aug 11;309(6):340–344. doi: 10.1056/NEJM198308113090604. [DOI] [PubMed] [Google Scholar]
  3. Comp P. C., Esmon C. T. Generation of fibrinolytic activity by infusion of activated protein C into dogs. J Clin Invest. 1981 Nov;68(5):1221–1228. doi: 10.1172/JCI110368. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Dunsker S. B., Torres-Reyes E., Peden J. C., Jr Pseudotumor cerebri associated with idiopathic cryofibrinogenemia. Report of a case. Arch Neurol. 1970 Aug;23(2):120–127. doi: 10.1001/archneur.1970.00480260026004. [DOI] [PubMed] [Google Scholar]
  5. Filippa G., Regli F., Yaşargil M. G. Beitrag zur Diagnostik der inneren Hirnvenenthrombose. Klinische Diagnostik und Therapie. Dtsch Med Wochenschr. 1966 Jun 3;91(22):1025–1034. doi: 10.1055/s-0028-1110696. [DOI] [PubMed] [Google Scholar]
  6. Girolami A., Rotilio A., Gerosa M., Patrassi G. Further studies on clotting changes in patients with cerebral sinus thrombosis. A case with thrombosis of right transverse sinus. Folia Haematol Int Mag Klin Morphol Blutforsch. 1981;108(4):597–604. [PubMed] [Google Scholar]
  7. Griffin J. H., Evatt B., Zimmerman T. S., Kleiss A. J., Wideman C. Deficiency of protein C in congenital thrombotic disease. J Clin Invest. 1981 Nov;68(5):1370–1373. doi: 10.1172/JCI110385. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Kobayashi S., Hino H., Hirasawa Y., Tazaki Y. [Superior sagittal sinus thrombosis due to familial antithrombin III deficiency: case report of two families (author's transl)]. Rinsho Shinkeigaku. 1980 Nov;20(11):904–910. [PubMed] [Google Scholar]
  9. Marlar R. A., Kleiss A. J., Griffin J. H. Mechanism of action of human activated protein C, a thrombin-dependent anticoagulant enzyme. Blood. 1982 May;59(5):1067–1072. [PubMed] [Google Scholar]
  10. McGehee W. G., Klotz T. A., Epstein D. J., Rapaport S. I. Coumarin necrosis associated with hereditary protein C deficiency. Ann Intern Med. 1984 Jul;101(1):59–60. doi: 10.7326/0003-4819-101-1-59. [DOI] [PubMed] [Google Scholar]
  11. Owen W. G. The control of hemostasis. Role of endothelium in the regulation of inhibitory and catabolic pathways. Arch Pathol Lab Med. 1982 May;106(5):209–213. [PubMed] [Google Scholar]
  12. Pabinger-Fasching I., Bertina R. M., Lechner K., Niessner H., Korninger C. Protein C deficiency in two Austrian families. Thromb Haemost. 1983 Dec 30;50(4):810–813. [PubMed] [Google Scholar]
  13. Samama M., Horellou M. H., Soria J., Conard J., Nicolas G. Successful progressive anticoagulation in a severe protein C deficiency and previous skin necrosis at the initiation of oral anticoagulant treatment. Thromb Haemost. 1984 Feb 28;51(1):132–133. [PubMed] [Google Scholar]

Articles from British Medical Journal (Clinical research ed.) are provided here courtesy of BMJ Publishing Group

RESOURCES