[Afibrinogenemia: description of a case with recurrent intracranial bleeding].
BACKGROUND
Congenital afibrinogenemia is a very rare autosomal recessive disorder of the coagulation. The lack of fibrinogen makes the blood inclotting and patients suffer from bleeding at a minimal trauma. It is usually noticed within the first few days of life because of the umbilical cord bleeding. Intracerebral hematoma is infrequent.


METHODS
46-year-old woman who suffers from congenital afibrinogenemia and has five episodes of intracerebral bleeding, all of them in the posterior circulation. As a complication, she also presents an epilepticus status and the therapy with concentrated fibrinogen caused pulmonary thromboembolism.


CONCLUSIONS
After analysing the cases described in literature, there are evidences that the majority of the intracerebral hematoma are located in the posterior circulation. A few patients developed major thomboemolisms following infusion with concentrated fibrinogen and there are only isolated descriptions of beneficial prophylactic effects. So, it is would necessary studies more extensive research to achieve effectiveness in the prevention of the bleeding.
Jiménez Caballero
P E
PE
Sección de Neurología, Hospital Virgen de la Salud, Toledo, Spain. pjimenez1010j@yahoo.es
Serviá Candela
M
M
Marsal Alonso
C
C
D000328:Adult; D000347:Afibrinogenemia / Q000150:complications* / Q000151:congenital / Q000188:drug therapy; D002560:Cerebrovascular Circulation; D005260:Female; D005340:Fibrinogen / Q000627:therapeutic use; D006801:Humans; D020300:Intracranial Hemorrhages / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D008875:Middle Aged; D012008:Recurrence; D012196:Review Literature as Topic; D013226:Status Epilepticus / Q000503:physiopathology
D005340:Fibrinogen