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

Wintzen, A.R.; Broekmans, A.W.; Bertina, Rogier M.; Briët, E.; Briët, P E; Zecha, A; Vielvoye, G J; Bots, G T

doi:10.1136/bmj.290.6465.350

Cited by 63 publications

(16 citation statements)

References 13 publications

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“…The haemostatic disorder and the genesis of ischaemic stroke have been linked in a number of cases of inherited coagulation deficiencies: AT-II1 [16]. protein C [17] and protein S [18]. Furthermore, several acquired disorders such as antiphospholipid antibodies [19] and lupus anticoagulants [20] have been associated with isch aemic stroke.…”

Section: Discussionmentioning

confidence: 99%

Haemostasis and Fibrinolysis after Recent Stroke

Franke¹,

Buscher²,

Wersch

1992

Cerebrovasc Dis

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Several measures of haemostasis and fibrinolysis were tested in 34 patients with cerebral haemorrhage and 47 patients with cerebral infarction within 11 days of the event. No significant differences were found between the two groups, but in comparison with a control group the following values were significantly elevated as the result of coagulation and fibrinolysis: fibrin monomers, thrombin-antithrombin-III, D-dimers and tissue plasminogen activator activity. In approximately 11 % of the patients the fibrin monomer levels were elevated to such an extent (> 30 mmol/1) that they were compatible with activation of the coagulation system. It is not possible to differentiate between cerebral infarction and cerebral haemorrhage by means of coagulation or fibrinolysis parameters. In the fibrin-monomer-negative group 22 % of the patients died, compared with 56% in the fibrin-monomer-positive group. Thus a state of hypercoagulability may have a negative effect on prognosis. Follow-up of 4 patients with cerebral haemorrhage showed that the abnormalities of coagulation and fibrinolysis parameters could last as long as 4 weeks.

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Section: Discussionmentioning

confidence: 99%

Haemostasis and Fibrinolysis after Recent Stroke

Franke¹,

Buscher²,

Wersch

1992

Cerebrovasc Dis

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“…All 3 female patients had a history of venous thromboembolism and there was no evidence for arterial thrombo embolism as cause for the cerebral incident. In 1 patient the available angiogram showed cerebral venous thrombosis [24], Cerebral venous thrombosis has also been reported in a type II protein C-deficient patient [23].…”

Section: Cerebral Haemorrhagic Infarction Due To Venous Thrombosismentioning

confidence: 99%

“…Three Dutch female patients with a type I protein C deficiency suffered at a relatively young age (27, 34 and 38 years of age, respec tively) from a cerebral event attributed to cerebral venous thrombosis [24], In all cases the cerebral event was preceded by a period of prodromal headache. A cerebral haemor rhagic infarction was documented by CT scan in 2 patients and at autopsy in the 1 pa tient who died.…”

Section: Cerebral Haemorrhagic Infarction Due To Venous Thrombosismentioning

confidence: 99%

Hereditary Protein C Deficiency

Broekmans¹

1985

Pathophysiol Haemos Thromb

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Hereditary protein C deficiency, which is inherited as an autosomal-dominant trait, predisposes to venous thrombotic disease. Heterozygotes are at risk for superficial thrombophlebitis, deep venous thrombosis and/or pulmonary embolism, which may occur without apparent cause at a young age. Other manifestations are cerebral venous thrombosis and mesenteric vein thrombosis. In severe, often homozygous, protein C deficiency, a purpura fulminans syndrome may occur shortly after birth, resulting in death due to extensive thrombosis, if it is not adequately treated. The thrombotic manifestations in heterzygotes are effectively prevented by coumarin therapy. However, in the initial phase of oral anticoagulant therapy, the patients have an increased risk for the development of coumarin-induced haemorrhagic skin necrosis. The purpura fulminans syndrome can be treated with either replacement therapy or with coumarin therapy. Heparin appears to be ineffective in the prevention of both the purpura fulminans syndrome and the coumarin-induced skin necrosis.

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“…A few instances of cerebral hemorrhagic infarction due to spontaneous cerebral venous thrombosis have been identified in patients with protein C deficiency. 12 Stroke has been reported in women taking oral contraceptives, 13 -14 a setting in which free protein S levels have been found to be reduced. 15 Free protein S deficiency was not found in a cohort of young adults with stroke in whom the blood test was performed a mean of 36 months after the stroke.…”

mentioning

confidence: 99%

Free protein S deficiency: a possible association with cerebrovascular occlusion.

Sacco

Owen

Mohr

et al. 1989

Stroke

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A deficiency of free protein S, known to increase the risk of peripheral venous thrombosis, has not been well described in patients with cerebrovascular disease. In a pilot study of 35 patients with symptomatic cerebrovascular disease, using a qualitative crossed immunoelectrophoresis assay we found eight patients with a free protein S deficiency. A Laurell immunoelectrophoresis assay was used to quantify the percentage of free protein S after removal of the inactive protein S C4b-binding protein complex by precipitation with polyethylene glycol 8000. In a quantitative study of 103 patients with cerebrovascular disease, 21 had a free protein S that was <20% of the average normal total protein S concentration (normal range 20-40%); 19 had suffered cerebral infarction and the other two had suffered intracranial hemorrhage. The frequency of free protein S deficiency in this group of stroke patients was far higher than the expected prevalence in the general population. (Stroke 1989;20:1657-1661

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

Abstract: Case report

Cited by 63 publications

References 13 publications

Haemostasis and Fibrinolysis after Recent Stroke

Haemostasis and Fibrinolysis after Recent Stroke

Hereditary Protein C Deficiency

Free protein S deficiency: a possible association with cerebrovascular occlusion.

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