Volume 49, Issue 6 pp. 1092-1101

Cognitive deficits after recovery from thrombotic thrombocytopenic purpura

April S. Kennedy

April S. Kennedy

From the Department of Medicine and Department of Psychiatry & Behavioral Sciences, College of Medicine, and the Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and the Department of Hematology and Central Hematology Laboratory, Inselspital, Berne University Hospital and University of Berne, Berne, Switzerland.

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Qurana F. Lewis

Qurana F. Lewis

From the Department of Medicine and Department of Psychiatry & Behavioral Sciences, College of Medicine, and the Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and the Department of Hematology and Central Hematology Laboratory, Inselspital, Berne University Hospital and University of Berne, Berne, Switzerland.

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James G. Scott

James G. Scott

From the Department of Medicine and Department of Psychiatry & Behavioral Sciences, College of Medicine, and the Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and the Department of Hematology and Central Hematology Laboratory, Inselspital, Berne University Hospital and University of Berne, Berne, Switzerland.

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Johanna A. Kremer Hovinga

Johanna A. Kremer Hovinga

From the Department of Medicine and Department of Psychiatry & Behavioral Sciences, College of Medicine, and the Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and the Department of Hematology and Central Hematology Laboratory, Inselspital, Berne University Hospital and University of Berne, Berne, Switzerland.

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Bernhard Lämmle

Bernhard Lämmle

From the Department of Medicine and Department of Psychiatry & Behavioral Sciences, College of Medicine, and the Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and the Department of Hematology and Central Hematology Laboratory, Inselspital, Berne University Hospital and University of Berne, Berne, Switzerland.

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Deirdra R. Terrell

Deirdra R. Terrell

From the Department of Medicine and Department of Psychiatry & Behavioral Sciences, College of Medicine, and the Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and the Department of Hematology and Central Hematology Laboratory, Inselspital, Berne University Hospital and University of Berne, Berne, Switzerland.

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Sara K. Vesely

Sara K. Vesely

From the Department of Medicine and Department of Psychiatry & Behavioral Sciences, College of Medicine, and the Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and the Department of Hematology and Central Hematology Laboratory, Inselspital, Berne University Hospital and University of Berne, Berne, Switzerland.

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James N. George

James N. George

From the Department of Medicine and Department of Psychiatry & Behavioral Sciences, College of Medicine, and the Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and the Department of Hematology and Central Hematology Laboratory, Inselspital, Berne University Hospital and University of Berne, Berne, Switzerland.

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First published: 01 June 2009
Citations: 70
James N. George, MD, or Sara K. Vesely, PhD, Department of Biostatistics and Epidemiology, The University of Oklahoma Health Sciences Center, Room CHB 358, P.O. Box 26901, Oklahoma City, OK 73126-0901; e-mail: [email protected] or [email protected].

This project was supported by the Hematology Research Fund of the University of Oklahoma Health Sciences Center and the Swiss National Science Foundation (Grant 3200B0-108261).

Abstract

BACKGROUND: Patients with apparent complete recovery from thrombotic thrombocytopenic purpura (TTP) often complain of problems with memory, concentration, and fatigue.

STUDY DESIGN AND METHODS: Twenty-four patients who were enrolled in the Oklahoma TTP-HUS Registry for their initial episode of TTP, 1995-2006, and who had ADAMTS13 activity of less than 10 percent were evaluated for a broad range of cognitive functions 0.1 to 10.6 years (median, 4.0 years) after their most recent episode. At the time of their evaluation, they had normal physical and Mini-Mental State Examinations and no evidence of TTP.

RESULTS: The patients, as a group, performed significantly worse on 4 of the 11 cognitive domains tested than standardized US data from neurologically normal individuals adjusted for age, sex, and education (p < 0.05). These four domains measured complex attention and concentration skills, information processing speed, rapid language generation, and rote memorization. Twenty-one (88%) patients performed below expectations on at least 1 of the 11 domains. No clear patterns were observed between cognitive test results and patients' characteristics or features of the preceding TTP, including age, occurrence of severe neurologic abnormalities, multiple episodes, and interval from an acute episode.

CONCLUSION: Patients who have recovered from TTP may have persistent cognitive abnormalities. The abnormalities observed in these patients are characteristic of disorders associated with diffuse subcortical microvascular disease. Studies of larger patient groups will be required to confirm these preliminary observations and to determine patient characteristics that may contribute to persistent cognitive abnormalities.

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