Abstract
Sickle cell disease is the most common cause of stroke in childhood, both ischaemic and haemorrhagic, and it also affects adults with the condition. Without any screening or preventative treatment, the incidence appears to fall within the range 0.5 to 0.9 per 100 patient years of observation. Newborn screening with Penicillin prophylaxis and vaccination leading to reduced bacterial infection may have reduced the incidence, alongside increasing hydroxyurea prescription. Transcranial Doppler screening and prophylactic chronic transfusion for at least an initial year has reduced the incidence of stroke by up to 10-fold in children with time averaged mean of the maximum velocity >200 cm/s. Hydroxyurea also appears to reduce the incidence of first stroke to a similar extent in the same group but the optimal dose remains controversial. The prevention of haemorrhagic stroke at all ages and ischaemic stroke in adults has not yet received the same degree of attention. Although there are fewer studies, silent cerebral infarction on magnetic resonance imaging (MRI), and other neurological conditions, including headache, epilepsy and cognitive dysfunction, are also more prevalent in sickle cell disease compared with age matched controls. Clinical, neuropsychological and quantitative MRI screening may prove useful for understanding epidemiology and aetiology.
Highlights
There are fewer studies, silent cerebral infarction on magnetic resonance imaging (MRI), and other neurological conditions, including headache, epilepsy and cognitive dysfunction, are more prevalent in sickle cell disease compared with age matched controls
The sickle gene mutation is most common in equatorial Africa, it is found in the Mediterranean regions of Europe and Turkey, related to the distribution of malaria [1], and sickle cell disease (SCD) is distributed worldwide as a consequence of the slave trade and economic migration
Stroke is an important cause of death in SCD, accounting for 10% of cases in the autopsy series covering the years 1929–1996 [34], this reduced to 4% for the Howard University series from 1976 to 2001 [35]
Summary
The sickle gene mutation is most common in equatorial Africa, it is found in the Mediterranean regions of Europe and Turkey, related to the distribution of malaria [1], and sickle cell disease (SCD) is distributed worldwide as a consequence of the slave trade and economic migration. Acute neurological symptoms and signs are common in SCD and, as well as stroke, include transient ischaemic attack (TIA) [8], headaches [9,10], seizures [8,11,12] and coma [13]. In one large series of patients with SCD and ACS, 3% of patients had neurological symptoms at presentation, and these symptoms developed in a further 7–10% as a complication of ACS [20] These patients should be classified clinically as having had a cerebrovascular accident [2], this may not occur during coding with the International Classification of Diseases
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