Intraparenchymal hemorrhage in a patient with osteogenesis imperfecta and plasminogen activator inhibitor-1 deficiency.
BACKGROUND
Osteogenesis imperfecta is associated with susceptibility to connective tissue damage, including intracranial but usually extra-axial hemorrhage. Plasminogen activator inhibitor-1 deficiency is a rare fibrinolytic cause of systemic bleeding diathesis.


OBJECTIVE
To describe a case of a brainstem intraparenchymal hemorrhage associated with connective tissue and coagulation disorders.


METHODS
Case report.


METHODS
Academic medical center.


METHODS
A 36-year-old woman with a history of osteogenesis imperfecta presented to the emergency department after an argument, during which she developed left ear pain and right eye esotropia followed by quadriparesis and somnolence. Neuroimaging showed a tegmental mesencephalic hemorrhage.


METHODS
Results of computerized tomography, magnetic resonance angiography, and parenchymal imaging; and serum hematologic markers.


RESULTS
No underlying vascular abnormality or mass lesion was found. Among coagulopathic serum markers, only plasminogen activator inhibitor-1 activity level was abnormally low.


CONCLUSIONS
Intraparenchymal hemorrhage may occur in the setting of a fibrinolytic inhibitory deficiency and osteogenesis imperfecta.
Department of Neurology, Dartmouth Hitchcock Medical Center, 1 Medical Center Drive, Lebanon, NH 03756, USA. richard.p.goddeau@hitchcock.org
Richard P
RP
Goddeau
Louis R
LR
Caplan
Adel A
AA
Alhazzani
D000328:Adult; D002543:Cerebral Hemorrhage / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D003238:Connective Tissue / Q000473:pathology*; D005260:Female; D006801:Humans; D018810:Magnetic Resonance Angiography; D010013:Osteogenesis Imperfecta / Q000150:complications*; D017395:Plasminogen Activator Inhibitor 1 / Q000172:deficiency*; D014057:Tomography, X-Ray Computed; D014568:Urokinase-Type Plasminogen Activator / Q000627:therapeutic use
D017395:Plasminogen Activator Inhibitor 1; D014568:Urokinase-Type Plasminogen Activator