Neuroimaging in Sickle Cell Disease : A Review

© 2020 The Authors. Journal of Neuroimaging published by Wiley Periodicals LLC on behalf of American Society of Neuroimaging..

Sickle cell disease is the most common hereditary hemoglobinopathy, which results in abnormally shaped and rigid red blood cells. These sickle-shaped red blood cells cause vaso-occlusion and ischemic phenomena that can affect any organ in the body. As a common cause of disability, the neurological manifestations of sickle cell disease are particularly important. Neuroimaging has a crucial role in the diagnosis, management, and prevention of the complications of sickle cell disease. These complications can affect the brain parenchyma, vasculature, and skull and can be ascribed directly or indirectly to a vasculopathy of small and large vessels. Vaso-occlusion can cause ischemic stroke. Ischemic damage in the absence of an acute neurological deficit, and therefore only apparent on neuroimaging, is termed silent cerebral ischemia. Weakening of the arterial walls can cause aneurysms. In its most severe form, a vasculopathy of the terminal internal carotid arteries can progress to moyamoya syndrome, characterized by steno-occlusive disease and the formation of friable collateral arteries. Rupture of aneurysms or friable collateral arteries is a potential cause of intracranial hemorrhage. The skull and vertebrae may be affected by extra-medullary hematopoiesis, due to severe anemia, or iron deposition, due to chronic red blood cell transfusion. Impaired blood supply to bone is associated with osteomyelitis and osteonecrosis. Fat embolization syndrome is a rare complication of osteonecrosis, which may cause devastating neurological impairment. Awareness and early recognition of the diverse manifestations of sickle cell disease on neuroimaging is crucial to ensure optimal treatment in a complex patient cohort.

Medienart:

E-Artikel

Erscheinungsjahr:

2020

Erschienen:

2020

Enthalten in:

Zur Gesamtaufnahme - volume:30

Enthalten in:

Journal of neuroimaging : official journal of the American Society of Neuroimaging - 30(2020), 6 vom: 16. Nov., Seite 725-735

Sprache:

Englisch

Beteiligte Personen:

Mallon, Dermot [VerfasserIn]
Doig, David [VerfasserIn]
Dixon, Luke [VerfasserIn]
Gontsarova, Anastasia [VerfasserIn]
Jan, Wajanat [VerfasserIn]
Tona, Francesca [VerfasserIn]

Links:

Volltext

Themen:

Fat embolization syndrome
Journal Article
Moyamoya syndrome
Pediatric stroke
Research Support, Non-U.S. Gov't
Review
Sickle cell disease
Vasculopathy

Anmerkungen:

Date Completed 14.06.2021

Date Revised 14.06.2021

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1111/jon.12766

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM320263541