Bleeding diathesis and hemophilias.

Patients with hemophilia and other congenital bleeding disorders are at risk for development of central nervous system (CNS) hemorrhage and can present with acute or chronic neurologic symptoms. These disorders are generally caused by qualitative or quantitative deficiency of components of hemostasis such as coagulation proteins, von Willebrand factor, or platelets. Rapid diagnosis and specific medical management such as coagulation factor replacement therapy are mandatory to minimize the morbidity and mortality of CNS bleeding. Therefore, the objective of this chapter is to introduce neurologists to the physiology of hemostasis and to provide an overview of the clinical presentation, and management of inherited bleeding disorders that can potentially present with CNS bleeding. Since hemophilia is the most common bleeding disorder encountered in clinical practice, more emphasis is placed on management of hemophilia. Additionally, neurologic manifestations related to the bleeding diathesis in patients with hemophilia are elaborated.