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Cerebral hyperemia, stroke, and transfusion in sickle cell disease

Department of Psychiatry, the Neurological institute, Columbia-Presbyterian Medical Center, New York, NY.(Dr. Prohovnik)
Department of Neurology, the Neurological institute, Columbia-Presbyterian Medical Center, New York, NY. (Drs. Prohovnik, Pavlakis, Mohr, and De Vivo)
Department of Pediatrics, the Neurological institute, Columbia-Presbyterian Medical Center, New York, NY. (Drs. Pavlakis, Piomelli, and De Vivo)
Department of Radiology, College of Physicians and Surgeons, Columbia University, The New York State Psychiatric Institute, and the Neurological institute, Columbia-Presbyterian Medical Center, New York, NY. (Drs. Prohovnik, Bello, and Hilal)

To investigate cerebral hemodynamics in sickle cell disease (SCD), we used the ¹³³Xenon inhalation technique of quantifying cerebral blood flow (CBF) in 67 patients. Clinical examinations and cerebral magnetic resonance imaging also were performed in all patients. Compared with age-matched healthy controls, CBF was elevated by 68% in patients, and inversely related to hematocrit. An experimental index of cerebral blood volume, pr₄, was also elevated in the patients in a similar manner. Cerebral blood volume was positively correlated to CBF in SCD patients but not in controls. History of stroke and current neurologic symptoms were associated with lower flow and higher cerebral blood volume. Transfusion therapy reduced the hyperemia, the reduction being greater than expected by hematocrit elevation alone. These findings document a vasodilatory hyperemia in SCD. This dilatation may be a risk factor for ischemic distal-field infarctions, as visualized by MRI, due to a limitation of cerebrovascular reserve capacity.

Address correspondence and reprint requests to Dr. Prohovnik, Mailbox 72, Psychiatric Institute, 722 West 168th Street, New York, NY 10032.

Supported in part by PHS grant HB 57010, Sickle Cell Center Grant HL 28381, the Jean and Louis Dreyfus Foundation, and the Colleen Giblin Foundation for Pediatric Neurology Research.

Presented in part at the Fifteenth Annual Child Neurology Society Meeting, Boston, October 1986, and the 13th Annual meeting of the Society of Cerebral Blood Flow and Metabolism, Montreal, June 1987.

Received May 18, 1988. Accepted for publication in final form September 6, 1988.

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