HEMIDYSTONIA SYMPTOMATIC OF PRIMARY ANTIPHOSPHOLIPID SYNDROME IN CHILDHOOD

We report three children with hemidystonia in whom anti-cardiolipin (a CL) antibodies were demonstrated. Systemic lupus erythematosus was exc luded on the basis of both clinical and serological criteria, and the diagnosis of primary antiphospholipid syndrome (PAPS) was made. In two cases, aCL antibodies could be causally related to a presumed immune- mediated thrombotic event involving the basal ganglia as shown by magn etic resonance imaging (MRI). In the remaining patient the finding of white matter alteration on NMR might be due to cross-reactivity of ant i-phospholipid (aPL) antibodies with cerebral phospholipids, resulting in demyelination. We suggest that PAPS must always be considered when isolated or recurrent focal cerebral ischaemia, and particularly hemi dystonia, occur in childhood.