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Thrombotic thrombocytopenic purpura in the brain: possible prognostic impact of von Willebrand factor physiology
Author(s) -
Vieregge P.,
Neugebauer C.,
Reusche E.,
Hallermann W.,
Budde U.,
Heide W.,
Otto V.,
Mentzel M.,
Hütteroth T.
Publication year - 1997
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.1997.tb00412.x
Subject(s) - medicine , von willebrand factor , autopsy , thrombotic thrombocytopenic purpura , pathology , vincristine , cortex (anatomy) , cyclophosphamide , purpura (gastropod) , cardiology , platelet , chemotherapy , neuroscience , ecology , biology
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.