Sickle cell disease

Kirkham, Fenella J. (2014) Sickle cell disease. In, Rosenberg's Molecular and Genetic Basis of Neurological and Psychiatric Disease: Fifth Edition. 5 ed. Elsevier Inc., pp. 1237-1249. (doi:10.1016/B978-0-12-410529-4.00102-9).

Abstract

People with sickle cell disease, a chronic hemolytic anemia, present with a wide variety of neurological syndromes, including ischemic and hemorrhagic stroke, anterior and posterior territory transient ischemic attacks (TIAs), "soft neurological signs," seizures, headache, coma, visual loss, and altered mental status. There is a peak for ischemic stroke in childhood, typically associated with stenosis or occlusion of the distal internal carotid and proximal middle cerebral arteries diagnosable using magnetic resonance angiography or transcranial Doppler ultrasound (TCD). For hemorrhagic stroke, the peak age is early adulthood, when aneurysms are common. Covert infarction may be detected on magnetic resonance imaging in around 25%. Cognitive difficulties, characteristically affecting attention, executive function, memory, arithmetic and processing speed, are also common. Indefinite transfusion is standard care for secondary prevention (and primary in those with TCD velocities. >. 200. cm per second). In addition to the possibility of cure from transplantation, hydroxyurea, aspirin and overnight respiratory support are under investigation as strategies to prevent neurological complications.

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Contributors

Author: Fenella J. Kirkham

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