Neuropsychological Dysfunction and Neuroimaging Abnormalities in Neurologically Intact Adults With Sickle Cell Disease
Overview
- Phase
- Not Applicable
- Intervention
- Not specified
- Conditions
- Anemia, Sickle Cell
- Sponsor
- UCSF Benioff Children's Hospital Oakland
- Enrollment
- 212
- Locations
- 12
- Primary Endpoint
- Wechsler Adult Intelligence Scale (WAIS)-III Performance IQ
- Status
- Completed
- Last Updated
- 9 years ago
Overview
Brief Summary
Sickle cell disease (SCD), also known as sickle cell anemia, is an inherited blood disease that can cause intense pain episodes and may lead to organ failure. Preliminary studies have shown that adults with SCD may have brain abnormalities that contribute to problems with cognitive functioning, including attention and memory difficulties. This study will use brain magnetic resonance imaging (MRI) and neuropsychological testing to examine the differences in cognitive functioning in adults with SCD and adults without SCD.
212 subjects participated in this cross-sectional study consisting of screening questionnaires, a neuropsychological testing battery, and MRI testing. Enrollment into this study ended in May 2008.
Detailed Description
SCD is an inherited blood disorder. Symptoms include anemia, infections, organ damage, and intense episodes of pain, which are called "sickle cell crises." In the past, SCD was considered a fatal disease, and many people with SCD died at a young age. Due to advances in medical care, people with SCD are now living longer lives; however, they often experience a deterioration in quality of life due to progressive organ failure. Past research has suggested that children with SCD commonly have frontal lobe dysfunction syndrome, which is a brain disorder that can affect cognitive functioning in areas such as attention, concentration, information processing, and decision making. Often times, however, neurocognitive and brain disorders are not diagnosed or treated in people with SCD. In preliminary brain imaging studies, at least half of adult participants with SCD had cognitive dysfunction that could be seen in images of the brain, while participants without SCD rarely had visible changes in the brain. Brain dysfunction may be one of the most important and least-studied problems affecting adults with SCD. The purpose of this study is to evaluate the extent of cognitive functioning problems in adults with SCD. The study will also determine if there is a connection between cognitive functioning problems and abnormalities seen on MRI brain images of adults with SCD. This study is an observational case/control study that will enroll adults with SCD and a control group of healthy adults who do not have SCD. At a study visit on Day 1, participants will undergo blood collection and will complete psychosocial questionnaires. Female participants will provide a urine sample for pregnancy testing. Study researchers will conduct a medical record review, a physical exam, and a neurological exam. They will also interview participants to collect medical history information. On Day 2, participants will undergo either a brain MRI or neuropsychological testing; on Day 3, the other procedure will be completed. On Day 4, study researchers will explain the study procedure results to participants. Participants will be asked if they are willing to take part in a second phase of the study in the future. Enrollment into this study ended in May 2008. A pilot interventional study follows this study, and is reported separately in ClinicalTrials.gov under NCT 00850018.
Investigators
Eligibility Criteria
Inclusion Criteria
- •Individuals who meet all of the following criteria are eligible for enrollment as cases into the study:
- •Adult between the ages of 21 and 55
- •African descent
- •Proficient/fluent in English
- •Hemoglobin electrophoresis confirming hemoglobin SS or SB0 (%A \<= 15)
- •Hemoglobin \<= 10 g/dL
- •Capable of giving informed consent for the protocol
- •Individuals who meet all of the following criteria are eligible for enrollment as community controls into the study:
- •Adult between the ages of 21 and 55
- •African descent
Exclusion Criteria
- •Individuals who meet any of the following criteria are disqualified from enrollment in the case group of the study:
- •Overt stroke
- •Previous evidence of an abnormal MRI or CT other than small peri-ventricular or watershed lesions
- •History of head injury that resulted in neurological symptoms or medical visit
- •Abnormal neurologic exam with focal findings
- •Mini-Mental Status Examination (MMSE) score of \< 20
- •Profile of Mood States (POMS) score on the Depression-Dejection Subscale suggestive of a clinical depression (score \> 40)
- •Alcohol consumption exceeding 14 drinks/week if female, 21 drinks/week if male
- •Drug abuse, defined as using non-prescribed medication
- •History of claustrophobia and/or presence of metallic implants such as pacemakers, surgical aneurysm clips, or known metal fragments embedded in the body
Outcomes
Primary Outcomes
Wechsler Adult Intelligence Scale (WAIS)-III Performance IQ
Time Frame: Within 2 months of signing informed consent.
Extent of neurocognitive dysfunction in neurologically asymptomatic adult patients with sickle cell disease as measured by WAIS-III performance IQ. This quotient is based on an average of 100, with a standard deviation of 15. The Wechsler intelligence scales are not considered adequate measures of extremely high and low intelligence (IQ scores above 160 and below 40, respectively). The performance IQ is derived from scores on seven subtests: picture completion, picture arrangement, block design, object assembly, digit symbol, matrix reasoning, and symbol search.
Secondary Outcomes
- Participants With Brain Lacunae as Measured by Clinical MRI(Within 2 months of informed consent)
- Volume of Total Cortical Gray Matter as Measured by Volumetric MRI.(Within 2 months of informed consent)