JOURNAL: European Journal of Neurology
VOLUME: 04
ISSUE:
06
PAGES: 0601-0605
AUTHOR: P. Vieregge, Neugebauer, E. Reusche, W. Hallermann, U. Budde, W. Heide, V. Otto, M. Mentzel, T. Hutteroth
ADDRESS: Department of Neurology, Medical University of Lubeck , Lubeck , Germany. Division of Neuropathology, Medical University of Lubeck , Lubeck , Germany. Department of Internal Medicine, Medical University of Lubeck , Lubeck , Germany. Central Coagulation Laboratory, General Hospital of Hamburg-Harburg , Hamburg , Germany. Department of Neurology, Municipal Hospital of Braunschweig , Braunschweig , Germany. Department of Internal Medicine, Municipal Hospital Sud , Lubeck , Germany
ABSTRACT: We report three patients with CNS involvement in thrombotic thrombocytopenic purpura (TTP). The first two cases presented with fluctuating levels of consciousness and lateralized neurologic deficits. Repeated brain CT scans were normal in case 1, and showed small parietal lucencies in case 2. Both patients recovered to only minor residues after combined treatment of plasma exchange, corticosteroids, immunoglobulins, vincristine (case 1), and cyclophosphamide (case 2), respectively. Case 3 with multiple infarctions in brain CT died of heart failure. Autopsy showed myocardial infarction and multiple hemorrhages in various organs including cerebral cortex. Excess deposition of von Willebrand factor antigen within hyaline thrombi in small and medium-sized arteries of the cerebral cortex is shown for the first time immunohistologically.
KEYWORDS: Thrombotic thrombocytopenic purpura Moschcowitz's disease von Willebrand factor Cerebrovascular disease, Thrombotisch thrombozytopenische Purpura
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