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Neuropsychological aspects of pediatric sickle cell disease
M C Kral1, R T Brown, G W Hynd
1Department of Pediatrics, Medical University of South Carolina, Charleston 29425, USA. kralm@musc.edu
Insights
Sickle cell disease (SCD) causes progressive cerebrovascular issues. Children with SCD experience widespread neurocognitive impairments, with subtle deficits linked to silent brain injuries.
Area of Science:
- Pediatric Neuropsychology
- Neurology
- Genetics
Background:
- Sickle cell disease (SCD) is a genetic disorder causing abnormal red blood cells and progressive cerebrovascular disease.
- Neurocognitive sequelae are common in children with SCD, impacting various cognitive domains.
Purpose of the Study:
- To investigate the neurocognitive impairments associated with sickle cell disease in children.
- To explore the relationship between cerebrovascular events (clinically apparent and silent) and neurocognitive deficits in pediatric SCD.
- To propose a disease-specific model of neuropsychological impairment in SCD.
Main Methods:
- Review of clinical data and neurocognitive assessments in children with SCD.
- Analysis of the impact of clinically apparent cerebrovascular accidents (CVAs) on cognitive function.
- Investigation of the association between silent infarcts and subtle neurocognitive deficits.
Main Results:
- Clinically apparent CVAs in children with SCD lead to pervasive neurocognitive impairments affecting intellectual functioning, language, memory, and academics.
- Subtle neurocognitive deficits in attention, executive function, and visual-motor speed are linked to silent infarcts not detected by physical examination.
- A disease-specific model of neuropsychological impairment is suggested by the disease course and associated sequelae.
Conclusions:
- Sickle cell disease significantly impacts cognitive function in children, with distinct patterns of impairment related to cerebrovascular events.
- Silent infarcts contribute to subtle neurocognitive deficits, highlighting the need for comprehensive neuropsychological evaluation in pediatric SCD.
- Further clinical and research efforts in pediatric neuropsychology are recommended for SCD management.
Abstract:
Sickle cell disease (SCD), a class of genetic disorders characterized by abnormal, sickled red blood cells, is a chronic illness that results in progressive cerebrovascular disease. Neurocognitive sequelae of clinically apparent cerebrovascular accidents in children with SCD are characterized by pervasive impairments, including decrements in general intellectual functioning, language and verbal abilities, visual-motor and visual-spatial processing, memory, academic achievement, and processing of subtle prosodic information. In contrast, subtle neurocognitive deficits in the areas of attention and concentration, executive function, and visual-motor speed and coordination appear to be associated with silent infarcts that are not necessarily detected on physical examination. Investigation of the disease course and associated neurocognitive sequelae suggest a disease-specific model of neuropsychological impairment. Recommendations are made for clinical and research efforts in the field of pediatric neuropsychology.
