ABSTRACT
Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy characterized by the production of structurally abnormal hemoglobin, causing a pathologic sickle shape in the erythrocyte. The clinical manifestations of SCD had been recognized for centuries in Africa before the etiology of this disease was known1,2. In 1910, Herrick published the first clinical case of an African-American with severe anemia associated with the occurrence of a ‘large number of thin, elongated, sickle-shaped and crescent-shaped forms’ in stained and wet smears of blood (Figure 1)3. He stated ‘Whether the blood picture represents merely a freakish poikilocytosis or is dependent on some peculiar physical or chemical condition of the blood, or is characteristic of some particular disease, I cannot at present answer’. Five years later Cook and Myers considered this sickling disorder of the blood to be a familial clinical entity4. It was not until 1922 that Mason used the term sickle cell anemia for the first time5. In 1923, Sydenstricker and colleagues published the first description of hemiplegia and seizure secondary to SCD in a five-year-old boy2. Since that time SCD has been a well-recognized risk factor for neurologic complications of a wide clinical spectrum, ranging from cognitive deficits to stroke. However, among the complications of SCD it is stroke that represents the most catastrophic sequela and the leading cause of death in both children6 and adults7 with the disease.
