Neurologic complications of sickle cell disease

Handbook of Clinical Neurology
Akila Venkataraman, Robert J Adams

Abstract

Sickle cell disease (SCD) is a group of genetic blood disorders that vary in severity, but the most severe forms, primarily homozygous sickle cell anemia, are associated with neurologic complications. Over the last 90 years it has become established that some patients will develop severe arterial disease of the intracranial brain arteries and suffer brain infarction. Smaller infarctions and brain atrophy may also be seen and over time there appear to be negative cognitive effects in some patients, with or without abnormal brain imaging. Focal mononeuropathies and pneumococcal meningitis are also more common in these patients. Brain infarction in children can largely be prevented screening children beginning at age 2 years and instituting regular blood transfusion when the Doppler indicates high stroke risk (>200cm/sec). Iron overload and the uncertain duration of transfusion are disadvantages but overall this approach, tested in a randomized clinical trial, reduced first stroke by over 90%. Secondary stroke prevention has not been subjected to a randomized controlled trial except for one recently stopped comparison of regular transfusions compared to hydroxuyrea (results favored transfusion). The usual stroke prevention agents ...Continue Reading

Citations

Jul 17, 2018·Somatosensory & Motor Research·Priscila Oliveira SilvaAgnaldo José Lopes
Sep 3, 2017·Neurology·Jean Jacques NoubiapJoseph Kamtchum-Tatuene
Mar 2, 2019·Current Neurology and Neuroscience Reports·Shama Farooq, Fernando D Testai
Sep 24, 2020·Experimental Biology and Medicine·Raven A HardyHyacinth I Hyacinth
Sep 12, 2019·Einstein·Emanuel Almeida Moreira de OliveiraLeonardo Meneghin Mendonça

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