PP06.5 – 2643: Clinical profile and radiological characteristics of children with dyskinetic cerebral palsy – An observational study

Abstract

Objective To study the clinical profile, etiology, radiological characteristics, motor functional ability and severity of dystonia in children with dyskinetic CP. Methods This was an observational study. Sixty-five consecutive children with dyskinetic CP seen between January 2013 and December 2013 were evaluated. For all patients, neuroimaging was done; gross motor function was evaluated using Gross Motor Function Classification System (GMFCS). Video-recordings of each child were made to evaluate resting and task-based dyskinesia using Burke–Fahn–Marsden Scale (BFMS). Results Majority of the dyskinetic children was termborn (80%), weighed >2500 g (72%), age-group 1–2 years (55.4%) and were boys (77%). Dystonia was predominant (91%) as compared to choreo-athetosis (9%). Commonest neonatal problems were neonatal jaundice (79%), sepsis (40%) and birth asphyxia (26%). Main parental concerns were developmental delay (97%), dystonia (60%) and speech problems (26%). 83% were severely disabled and non-ambulatory (level V and IV GMFCS); only onetenth achieved ambulation (Level I and II GMFCS). Mean BFMS score was 45.8+19. Nearly 90% of dystonic children were at level IV and V GMFCS; 67% of choreo-athetotic children were ambulatory. Etiologically, hyperbilirubinemia (66%) and perinatal asphyxia (30%) were predominant. In children with hyperbilirubinemia, mean serum bilirubin was 31+9.4 mg/dL, exchange transfusion done in 60.7%, and 21.5% were G-6-PD deficient. Radiologically, this predominantly involved globus pallidum, substantia nigra and subthalamus; normal scans were seen in 2 children. Perinatal asphyxia led to periventricular-white-matter, putaminal and thalamic changes. The mean dystonia scales were higher in the hyperbilirubinemia group as compared to perinatal asphyxia group. Conclusion Hyperbilirubinemia contributes significantly to severely disabling dyskinetic CP in developing countries. Distinctive radiological patterns of injury help identify the nature of insult. Significant motor impairments and dystonia impair functional abilities of these dyskinetic children.