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Neurological Correlates and Clinical Implications

Steven Kugler, MD; Brenda Anderson, RNC; Dewitt Cross, MD; Zafar Sharif, MD; Mary Sano, PhD; Rita Haggerty, PhD; Isak Prohovnik, PhD; Anne Hurlet-Jensen, MD; Sadek Hilal, MD, PhD; Jay P. Mohr, MD; Darryl C. De Vivo, MD

Arch Neurol. 1993;50(6):629-635.

Abstract
• Objective.
—Eight asymptomatic patients with sickle-cell disease (SCD) with magnetic resonance imaging (MRI) abnormalities consistent with cerebral infarcts (group 1) and eight asymptomatic patients with SCD with normal MRI scans (group 2) were followed up to assess the neurological correlates and the clinical outcome.

Design.
—Patients in the two cohorts underwent clinical evaluations and xenon 133 regional cerebral blood flow (rCBF) studies within 1 month of the entry MRI. This study sequence was repeated up to 5 years later. Neuropsychological studies also were performed in six group 1 patients and eight group 2 patients at the end of the study.

Setting.
—The patients were recruited from the Comprehensive Sickle Cell Center at Columbia University, New York, NY.

Patients.
—All patients had SCD, hemoglobin SS, and normal findings on clinical evaluation at entry. The group 1 cohort had clinically silent MRI abnormalities consistent with cerebral infarction. The group 2 cohort was age matched to group 1 and had normal MRI studies.

Interventions.
—None.

Main Outcome Measure.
—The natural history of MRI abnormalities and the neurological correlates were assessed to determine the predictive value of subclinical MRI lesions as a risk factor for clinically apparent stroke.

Results.
—The mean duration of MRI follow-up was 3.7 years. In group 1, four patients (50%) demonstrated progressive MRI abnormalities and three patients (38%) became clinically symptomatic. In group 2, findings for all patients remained normal on clinical and radiological examination. Both groups had markedly elevated rCBF values. Individual rCBF differences correlated with the specific MRI abnormalities. The psychometric study results were similar in the two cohorts. Eighty-three percent of group 1 and 88% of group 2 patients had defective scores in one or more areas of cognitive functioning. Three patients met cognitive criteria for dementia.

Conclusions.
—Cranial MRI abnormalities have important prognostic implications even when detected in clinically asymptomatic patients. Cognitive abnormalities exist in patients with SCD even in the absence of MRI abnormalities or clinical stroke.

Author Affiliations
From the Departments of Neurology (Drs Kugler, Mohr, and De Vivo and Ms Anderson), Pediatrics (Drs Kugler, Hurlet-Jensen, and De Vivo and Ms Anderson), Radiology (Drs Cross and Hilal), Psychiatry (Drs Sharif and Prohovnik), and Psychology (Drs Sano and Haggerty), College of Physicians and Surgeons of Columbia University and the Presbyterian Hospital, New York, NY.

Footnotes
Accepted for publication October 6, 1992.

Presented in part at the 20th Child Neurology Society Meeting, Portland, Ore, October 1991.

Reprint requests to The New York Neurological Institute, 710W 168th St, New York, NY 10032 (Dr De Vivo).

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