[Cerebral venous thrombosis and hereditary protein C deficiency]

J Massons1, A Arboix, M Oliveres

  • 1Servicio de Neurología, Hospital Central de La Alianza, Barcelona.

Insights

Protein C deficiency, a risk factor for venous thrombosis, can lead to rare cases of cerebral venous thrombosis. This case highlights the importance of considering this deficiency in young adults with cerebral venous thrombosis.

Area of Science:

  • Hematology
  • Neurology
  • Genetics

Background:

  • Protein C, protein S, and antithrombin III are key plasma inhibitors of coagulation.
  • Protein C deficiency is a significant risk factor for venous thrombosis.
  • Cerebral venous thrombosis (CVT) is an uncommon manifestation of protein C deficiency.

Observation:

  • A 32-year-old patient presented with symptoms of endocraneal hypertension.
  • CT and MR imaging revealed multiple hemorrhagic cerebral infarctions.
  • Arteriography confirmed vertebral venous thrombosis.

Findings:

  • The patient was diagnosed with protein C deficiency.
  • Literature review found only six documented cases of CVT due to protein C deficiency.
  • Coadjuvant factors predisposing to thromboembolic disease were present in most reported cases.

Implications:

  • This case underscores the necessity of including protein C deficiency in the differential diagnosis of cerebral venous thrombosis in young adults.
  • Early diagnosis and management of protein C deficiency can prevent severe thrombotic events.
  • Further research into the genetic and environmental factors contributing to CVT in protein C deficient individuals is warranted.

Related Concept Videos

Anticoagulant Drugs: Low-Molecular-Weight Heparins01:30

Anticoagulant Drugs: Low-Molecular-Weight Heparins

Hemostasis is a crucial process that prevents excessive blood loss from damaged blood vessels. It involves various mechanisms such as vasoconstriction, platelet adhesion and activation, and fibrin formation. The importance of each mechanism depends on the type of vessel injury. In contrast, thrombosis is the abnormal formation of a blood clot within the blood vessels, leading to potential complications if the clot obstructs blood flow. Thrombosis can be caused by increased coagulability of the...
Disorders of Hemostasis01:24

Disorders of Hemostasis

Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
Venous Thrombosis I: Introduction01:30

Venous Thrombosis I: Introduction

Venous thrombosis, the most common disorder of the veins, involves the formation of a thrombus or blood clot associated with vein inflammation. It can be classified as either superficial vein thrombosis or deep vein thrombosis.Superficial Vein Thrombosis: This involves the formation of a thrombus in a superficial vein, usually the greater or lesser saphenous vein. Though less severe than deep vein thrombosis (DVT), SVT can lead to complications if untreated.Deep Vein Thrombosis (DVT): This...
Venous Thrombosis III: Interprofessional Care01:29

Venous Thrombosis III: Interprofessional Care

Venous thrombosis requires effective prevention and treatment strategies to improve patient outcomes and reduce potential complications.Prevention StrategiesHealthcare providers must prioritize preventing venous thromboembolism (VTE) for all adult patients upon admission. Interventions depend on bleeding and thrombosis risk, medical history, current medications, diagnoses, planned procedures, and patient preferences. Patients on bed rest should change positions every two hours and, if not...
Cerebral Edema l: Introduction01:19

Cerebral Edema l: Introduction

Cerebral edema is a pathological increase in brain water content that disrupts intracranial pressure regulation and impairs neurological function. Because the cranial vault is rigid, even modest increases in tissue volume can compromise cerebral perfusion, distort neural structures, and initiate secondary injury. Cerebral edema develops through four principal mechanisms: vasogenic, cytotoxic, interstitial, and ionic.Vasogenic EdemaVasogenic edema arises from disruption of the blood–brain...
Cerebral Edema ll: Pathophysiology01:22

Cerebral Edema ll: Pathophysiology

Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...