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Adults With Sickle Cell Disease Have Significant Cognitive Impairment: Presented at ASH

By Sophie Bainbridge

ATLANTA, GA -- December 11, 2007 -- Individuals with sickle cell disease who manage to make it into their 20s and beyond have been found to have significant neuropsychological dysfunction and brain injury, a researcher said here at the 49th American Society of Hematology (ASH) Annual Meeting and Exposition.

A study that evaluated 138 adults with sickle cell anemia and 37 controls with a battery of cognitive tests found that neurocognitive dysfunction, undetected brain injury, or both affected most of the sickle cell subjects.

Particularly affected were areas of executive functioning, reading, and mathematical ability, said Elliott Vichinsky, MD, Director, Department of Hematology/Oncology, Children's Hospital Oakland, Oakland, and Professor of Pediatrics, University of California, San Francisco, California.

Sickle cell disease is becoming an adult disease as childhood treatments improve, and most patients are living into adulthood. However, it is a disease that is plagued by chronic organ dysfunction that decreases quality of life and results in major problems with assimilation into society and difficulty in reaching one's maximum potential, noted Dr. Vichinsky.

The results of this study are proof, he said. Of the sickle cell disease patients studied, 63% had neuropsychological dysfunction or abnormal findings on magnetic resonance imaging, 38% had imaging abnormalities, including evidence of silent infarcts and hippocampal atrophy, and 32% scored below 86 on the Wechsler Adult Intelligence Scale (WAIS) IQ scale. In contrast, 15% of the normal adult population scores below 86 on the WAIS scale, Dr. Vichinsky said.

After controlling for age, gender, and education, sickle cell patients did significantly worse than controls on the WAIS Processing Speed Index score and the Woodcock-Johnson score, which are tests of reading, math, and ability to follow directions. In addition, subtests of the Test of Everyday Attention, which measures flexibility of thought and attention, were significantly decreased in sickle cell patients, particularly as they aged. The same was not true for controls.

"The WAIS Processing Speed Index is an assessment of attention, scanning, and discrimination of visual information, and it requires the ability to plan and coordinate motor activities. It is decreased in patients who have known hypoxic injury and white matter injury," Dr. Vichinsky said.

Hemoglobin level was a strong predictor of these abnormalities. The more anemic the patients were, the more likely they were to have significant neurocognitive abnormalities.

Hippocampal volume was lower in sickle cell disease patients than in controls, and this volume decreased with age. No loss occurred in the age-matched controls.

"This was a very young group, in their 20s to early 30s, and they had progressive loss of brain hippocampal volume. This suggests, as we follow these patients out longer, they are going to have premature and progressive volume loss greater than we currently see," he said.

Studies to determine if transfusions will correct the neurocognitive dysfunction are now underway, he said. "We are very excited to see the results and are hopeful that this will be a good strategy for these patients," Dr. Vichinsky said.


[Presentation title: Neuropsychological (NP) Dysfunction and Neuroimaging Abnormalities in Neurologically Intact Adult Patients With Sickle Cell Disease. Abstract 428]

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