Related Experiment Video

Updated: Aug 30, 2026

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry
08:23

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry

Published on: November 5, 2019

Brain injury in children with sickle cell disease: prevalence and etiology

R Grant Steen1, Xiaoping Xiong, James W Langston

  • 1Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, TN, USA. Grant_Steen@med.unc.edu

Annals of Neurology
|November 5, 2003
PubMed

Insights

In children with sickle cell disease (hemoglobin SS), brain injury is common and closely linked to blood vessel disease (vasculopathy). Vasculopathy often appears before brain damage, suggesting it is a precursor to injury.

Area of Science:

  • Pediatric Neurology
  • Hematology
  • Radiology

Background:

  • Sickle cell disease (hemoglobin SS) is a severe inherited blood disorder.
  • Brain injury is a significant complication in children with hemoglobin SS.
  • The relationship between brain imaging findings and blood vessel abnormalities is not fully understood.

Purpose of the Study:

  • To investigate the association between brain injury (MRI) and vasculopathy (MRA) in children with hemoglobin SS.
  • To determine if vasculopathy precedes or accompanies brain injury in this population.

Main Methods:

  • Retrospective review of neuroimaging (MRI and MRA) for 146 children with hemoglobin SS.
  • Standardized criteria used to assess brain injury (infarction, leukoencephalopathy, encephalomalacia, atrophy) and vasculopathy (tortuosity, stenosis, occlusion).

Main Results:

  • 46% of patients showed signs of brain injury, and 64% had vasculopathy by age 10.
  • Only 28% of patients were normal on both MRI and MRA.
  • Abnormal MRA findings frequently correlated with abnormal MRI findings (p < 0.00001).
  • Cystic infarction was associated with limited vasculopathy, while encephalomalacia correlated with stenosis/occlusion (p < 0.0001).
  • Large arteries were affected in 31% of brain injury patients; small arteries were inferred to be abnormal in up to 69%.

Conclusions:

  • Vasculopathy is highly prevalent and closely associated with brain injury in children with hemoglobin SS.
  • The findings suggest vasculopathy is a prodromal event for most forms of brain injury in this condition.
  • Early detection and management of vasculopathy may be crucial for preventing brain damage in sickle cell disease.

More Related Videos

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload05:23

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload

Published on: March 14, 2017

Related Concept Videos

Hemorrhagic Stroke ll: Pathophysiology01:29

Hemorrhagic Stroke ll: Pathophysiology

A hemorrhagic stroke develops when a cerebral blood vessel ruptures, allowing blood to escape into the surrounding brain tissue, as in intracerebral hemorrhage (ICH), or into the subarachnoid space, as in subarachnoid hemorrhage (SAH). Because the skull is a rigid compartment, the sudden presence of extravascular blood rapidly increases intracranial pressure and compresses adjacent neural structures, leading to immediate tissue injury and impaired cerebral perfusion.Mass Effect and Primary...
Secondary Spinal Cord Injury llI: Pathophysiology01:25

Secondary Spinal Cord Injury llI: Pathophysiology

Early Ischemia and Ionic ImbalanceWithin minutes of spinal cord injury, a secondary cascade begins, progressing over hours to weeks. Vascular damage reduces blood flow, causing ischemia and mitochondrial dysfunction. ATP depletion leads to ion pump failure, membrane depolarization, sodium influx, potassium efflux, and water accumulation, resulting in cellular swelling. Increased intracellular calcium further disrupts mitochondria and accelerates cellular injury.Excitotoxicity and Neuronal...
Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Spinal Cord Injury ll: Pathophysiology01:14

Spinal Cord Injury ll: Pathophysiology

Spinal cord injury progresses through two interconnected phases: primary injury and secondary injury.Primary InjuryPrimary injury happens at the moment of trauma and involves immediate mechanical damage to the spinal cord.Compression happens when broken vertebrae, herniated discs, or accumulating blood (such as a hematoma) press directly against the spinal cord, distorting its normal shape and function. In cases of contusion, the cord is bruised by a blunt force (like penetrating injuries or...
Disorders of Erythrocytes01:27

Disorders of Erythrocytes

Disorders of erythrocytes, or red blood cells (RBCs), include a range of conditions affecting their number, shape, or function.
Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
On the other...
Hemorrhagic Stroke l: Introduction01:17

Hemorrhagic Stroke l: Introduction

A hemorrhagic stroke is an acute neurological event that occurs when a weakened cerebral blood vessel ruptures, allowing blood to accumulate within or around the brain. The sudden release of blood forms a focal hematoma that increases intracranial pressure, displaces neural tissue, and can obstruct cerebrospinal fluid pathways. These effects may be compounded by intraventricular extension of the hemorrhage, cerebral edema, or compression of adjacent structures, all of which contribute to...