Intraparenchymal hemorrhage in a patient with osteogenesis imperfecta and plasminogen activator inhibitor-1 deficiency.

نویسندگان

  • Richard P Goddeau
  • Louis R Caplan
  • Adel A Alhazzani
چکیده

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. DESIGN Case report. SETTING Academic medical center. PATIENT 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. MAIN OUTCOME MEASURES 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. CONCLUSION Intraparenchymal hemorrhage may occur in the setting of a fibrinolytic inhibitory deficiency and osteogenesis imperfecta.

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عنوان ژورنال:
  • Archives of neurology

دوره 67 2  شماره 

صفحات  -

تاریخ انتشار 2010