Thrombotic Thrombocytopenic Purpura Presenting as a Sudden Headache With Focal Neurologic Findings☆,☆☆,★
Section snippets
INTRODUCTION
Thrombotic thrombocytopenic purpura (TTP) was first described in 1923 by Moschowitz, but was not formally named until 1954.1 TTP is one of the endothelioses, a spectrum of disease including hemolytic uremic syndrome and postpartum kidney failure. It occurs in approximately 5 to 10 cases per 50,000 hospital admissions. No single age group is more heavily affected, but a female predominance of approximately 10 to 1 has been noted.1 Untreated, TTP has a mortality rate of 90%.2 Neurologic and
CASE REPORT
A 49-year-old black woman with a history of borderline diabetes and hypertension was referred to the University of Maryland Medical Center emergency department for evaluation of a bifrontal headache that had begun earlier in the day. The referring physician had noted drowsiness and slurred speech, in addition to a blood pressure of 180/120 mm Hg. The headache occurred suddenly, with maximal intensity at onset, and the patient had associated nausea and vomiting. She had experienced intermittent
DISCUSSION
The pathophysiology underlying TTP has yet to be completely understood. Endothelial cell damage with hyaline thrombus formation is the hallmark histologic finding in TTP. Proposed mechanisms for the endothelial cell wall injury include abnormal antibody formation, immune complexes, and alterations in the coagulation and fibrinolytic systems. No platelet-aggregating factor, prostacyclin inhibitor, or abnormal antibody has consistently been identified. Discussion continues as to whether the
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Should intravenous immunoglobulin G be first-line treatment for acute thrombotic thrombocytopenic purpura? Case report and review of the literature
Am J Kidney Dis
(1991) Thrombotic thrombocytopenic purpura
JAMA
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Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura
N Engl J Med
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Thrombotic thrombocytopenic purpura: Report of 25 cases and review of the literature
Medicine
(1981)
Cited by (7)
Thrombotic thrombocytopenic purpura (TTP) presenting as pancreatitis
2003, Journal of Emergency MedicineA systematic review of causes of sudden and severe headache (Thunderclap Headache): should lists be evidence based?
2014, Journal of Headache and PainThrombotic thrombocytopenic purpura
2008, Uncommon Causes of Stroke, 2nd EditionPsychiatric symptoms in thrombotic thrombocytopenic purpura
2000, Actas Espanolas de Psiquiatria
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From the Division of Emergency Medicine, Department of Surgery, University of Maryland Medical Center, Baltimore, Maryland.
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Address for reprints: Michael Witting, MD, 419 West Redwood Street, Suite 280, Baltimore, Maryland 21201, 410-328-8025, Fax 410-987-3263
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Reprint no. 47/1/69694