E. Mercuri et al., NEUROLOGICAL SOFT SIGNS MAY IDENTIFY CHILDREN WITH SICKLE-CELL DISEASE WHO ARE AT RISK FOR STROKE, European journal of pediatrics, 154(2), 1995, pp. 150-156
Stroke is one of the most frequent complications of sickle cell diseas
e (HbSS), occurring in 7-17% of children. Recent studies recognized mo
re minor lesions on MRI, not associated with clinical signs on standar
d neurological examination, which however have been found to be a risk
factor for developing stroke later. The aim of this study was to eval
uate whether minor lesions observed on imaging could be associated wit
h 'soft' neurological signs not detectable on conventional neurologica
l examination. Fourteen children with HbSS were assessed with MRT, sta
ndard neurological examination and evaluation of 'soft' signs (Zurich
Neuromotor Test) and motor function (Movement ABC). Eight of the 14 ch
ildren scanned showed lesions on MRI but only 3 of the full cohort wer
e abnormal on standard neurological examination. However, all of the e
ight 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 th
e 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 ev
en minor MRI lesions, allow the evaluation of the global incidence of
major and minor neurological signs and may also help to identify the p
opulation at risk for developing strokes. This population could then b
e closely monitored and benefit from early intervention.