STROKE AS A LIFE-THREATENING COMPLICATION IN CHILDREN WITH LINEAR SCLERODERMA OF FACE.

Objective: To investigate the spectrum of neurological disorders in children with juvenile localized scleroderma (JLS) on face and JLS without plaques on face and head.

Materials and methods: 156 children with JLS were examined were with a neurological examination MRI, EEG, genetic thrombophilia markers detection.

Results: Neurological disorders (ND) were found in 56 from 114 (49%) of the patients with scleroderma of head and face (LSH)(group1) and in 30% (13 from 42) with JLS without plaques on face (Group 2). Headaches were detected in Group 1 in 43,8%, in Group 2-in30%. In Group 1 other disorders were detected: epileptic seizures (in 25%), cranial nerve involvement (12.5%), stroke (5.3%), tics (5.3%), none of named in Group 2. Out of 56 children with ND changes on brain magnetic resonance imaging (MRI) were detected in 47 (84%). white matter lesions and less commonly gliotic changes in the periventricular area. Electroencephalography (EEG) monitoring revealed typical patterns of epileptiform activity in the majority of cases. These three cases of ischemic stroke in children with LSH are the first to be presented. All patients with stroke were diagnosed with genetic thrombophilia, two had cerebral vascular malformations, and one had antiphospholipid syndrome.

Conclusion: Our data suggest that sclerodermic vasculopathy, cerebral vascular anomalies and genetic thrombophilia are rick factors for stroke in children with LSH. Mandatory MRI angiography and screening for genetic thrombophilia could identify risk group for stroke in children with LSH.