Intraparenchymal hemorrhage in a patient with osteogenesis imperfecta and plasminogen activator inhibitor-1 deficiency.
نویسندگان
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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ورودعنوان ژورنال:
- Archives of neurology
دوره 67 2 شماره
صفحات -
تاریخ انتشار 2010