Ischemic strokes in the structure of neurological disorders in children with linear scleroderma of the head

Abstract

Objective. The aim of the study: analysis of the structure of neurological disorders in children with linear scleroderma of the head.Methods. Clinical and neurological observation, MRI, EEG, examination of genetic thrombophilia markers.Results. A total of 60 patients with linear scleroderma of the face and head (LSF) were examined: 23 boys (38.33%) and 37 girls (61.67%) aged between 3 and 16 years. The average age of disease onset was 6.64 ± 0.53 years. Neurological disorders were found in half of the patients (n = 32; 51.67%). Among them, disease onset in 26 children (81.3%) was characterized by typical skin changes, while in 8 (25%) it was characterized by neurological signs. Neurological disorders included headaches (in 43.8% of children), epileptic seizures (in 25%), cranial nerve involvement, acute cerebrovascular disorders (9.4%), and tics (9.4%). MRI of the brain revealed changes in 27 out of 32 children with neurological disorders (84%): white matter lesions (63%) and less commonly gliotic changes in the periventricular area (31%). EEG monitoring detected abnormalities in 21 children (65%), but a typical pattern of epileptiform activity was only found in 14 children (24%), including 6 children (10% of cases) with epileptiform activity without clinical seizures. Among the three children with acute cerebrovascular disorders, all were diagnosed with genetic thrombophilia, two had cerebral vascular malformations, and one had antiphospholipid syndrome.Conclusion. The pathogenesis of strokes in children with LSF involves sclerodermic vasculopathy, cerebral vascular anomalies, and mutations in blood coagulation genes leading to hypercoagulability.

Keywords

• linear scleroderma of the head
• children
• neurological disorders
• headaches
• seizure syndrome
• cranial nerve involvement
• ischemic stroke
• mri and eeg changes
• genetic thrombophilia