Vascular lesions in the central nervous system in sickle cell disease (neuropathology)

The incidence of serious neurologic complications in sickle cell disease ranges from 6% to 30% in various series. Our experience indicates that the incidence in adults is approximately 15%. The clinical and neuropathologic correlations at autopsy in 20 sickle cell disease patients are discussed in this paper. Some patients had massive infarcts and/or hemorrhages in the cerebral hemispheres as the terminal event. In some, both infarction and hemorrhage occurred simultaneously; others had thrombosis of major branches of the circle of Willis and in the internal carotid artery on one or both sides. Prominent large arterial occlusive disease in the terminal intracranial portions of the internal carotid arteries and proximal segments of their main branches was frequently observed. The pathology of the brain showed diffuse chronic gliotic scars of microinfarcts in the deep white matter and cerebral gray matter, even when no past neurologic history was recorded. We hypothesize that the vague, nonspecific complaints, including chronic alteration of intellectual and emotional function, of some sickle cell disease patients prior to their death, may constitute the clinical counterpart of disseminated microscopic lesions resulting from small-vessel disease. The pathogenesis of vaso-occlusive disease includes altered cerebral blood flow, platelet response to endothelial injury, and sickled erythrocyte adhesion to endothelium.