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Neurological 'soft' signs may identify children with sickle cell disease who are at risk for stroke
E Mercuri1, J C Faundez, I Roberts
1Department of Paediatrics, Hammersmith Hospital, London, UK.
Insights
Soft neurological signs detected by the Zurich Neuromotor Test and Movement ABC can identify minor MRI lesions in children with sickle cell disease (HbSS), aiding in stroke risk assessment.
Area of Science:
- Pediatric Neurology
- Hematology
- Neuroimaging
Background:
- Sickle cell disease (HbSS) frequently leads to stroke in children.
- Minor MRI lesions, often clinically silent, are stroke risk factors.
- Conventional neurological exams may miss subtle neurological deficits.
Purpose of the Study:
- To determine if 'soft' neurological signs correlate with minor MRI lesions in children with HbSS.
- To assess the diagnostic value of specific neuromotor and motor function tests.
Main Methods:
- 14 children with HbSS underwent MRI, standard neurological examination, and 'soft' sign assessments (Zurich Neuromotor Test, Movement ABC).
- Data were analyzed to compare MRI findings with clinical and test results.
Main Results:
- 8 of 14 children had MRI lesions; only 3 showed abnormalities on standard neurological exams.
- All 8 children with MRI lesions exhibited abnormal 'soft' signs.
- The combined sensitivity of the Zurich and Movement ABC tests for detecting MRI lesions was 100%.
Conclusions:
- 'Soft' neurological signs effectively identify subtle MRI lesions in pediatric HbSS.
- These tests aid in evaluating neurological signs and identifying at-risk populations for stroke.
- Early identification allows for closer monitoring and timely intervention to prevent strokes.
Unlabelled:
Stroke is one of the most frequent complications of sickle cell disease (HbSS), occurring in 7-17% of children. Recent studies recognized more minor lesions on MRI, not associated with clinical signs on standard neurological examination, which however have been found to be a risk factor for developing stroke later. The aim of this study was to evaluate whether minor lesions observed on imaging could be associated with 'soft' neurological signs not detectable on conventional neurological examination. Fourteen children with HbSS were assessed with MRI, standard neurological examination and evaluation of 'soft' signs (Zurich Neuromotor Test) and motor function (Movement ABC). Eight of the 14 children scanned showed lesions on MRI but only 3 of the full cohort were abnormal on standard neurological examination. However, all of the eight children with MRI lesions also showed abnormal signs on at least one of the two tests (Zurich and Movement ABC). All the children with normal MRI were normal on all the tests performed. The sensitivity of Zurich Neuromotor Test and Movement ABC in the group of children with MRI lesions is 0.88 and 0.75, respectively, and increases to 1 when the two tests are used together. The specificity of both tests is 1 even when the tests are used separately.
Conclusion:
Although the number of cases is small, 'soft' signs may reliably identify the presence of even minor MRI lesions, allow the evaluation of the global incidence of major and minor neurological signs and may also help to identify the population at risk for developing strokes. This population could then be closely monitored and benefit from early intervention.