Studies in Sickle Cell Anemia

Neurologic manifestations are frequent in patients who have sickle cell disease. These manifestations may be the earliest presenting signs and symptoms and they are so variable that the patient may be erroneously diagnosed as having conditions such as meningitis, poliomyelitis, subdural hematoma, neoplasm, subarachnoid hemorrhage, lead encephalitis, subacute bacterial endocarditis, and congenital malformations of the brain. Furthermore, development of these manifestations cannot be predicted on the basis of the type of crisis involved. Prognosis following neurological involvement is unpredictable, but recurrent episodes, together with abnormal electroencephalographic readings suggest a poor outcome. Such patients die or are the victims of rather severe neurological deficits. Neurological examination of these patients suggests diffuse involvement of the cerebral hemispheres. Examination of the pathological material, however, often fails to reveal thromboses. The striking findings are infarcts in the white matter and perivascular hemorrhages. The thesis that the abnormal neurological findings in these patients are actually due to sickle cell disease is supported by the fact that all such symptoms appear in association with clinical circumstances known to induce sickling. These include surgery and anesthesia, fever and infection.