Richard P Goddeau1, Louis R Caplan, Adel A Alhazzani. 1. Department of Neurology, Dartmouth Hitchcock Medical Center, 1 Medical Center Drive, Lebanon, NH 03756, USA. richard.p.goddeau@hitchcock.org
Abstract
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.
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.