INCREASED PLASMA MALONDIALDEHYDE ASSOCIATED WITH CEREBELLAR STRUCTURAL DEFECTS

Background - Malondialdehyde (MDA) in plasma is regarded as an indicat or for increased lipid peroxidation. Method - Measurements of MDA conc entrations in plasma were compared among healthy children (n=31), pati ents with neurological disorders or epileptic syndromes (n=15), and ch ildren with pontocerebellar structural defects (n=31), where the cause or genetic defect remained unknown. Results - In healthy children the median MDA value was 5.86 nmol/ml (mean (SD) value: 6.25 (1.97), rang e: 3.76-11.19). For the group with various neurological disorders or e pilepsy, the values were similar with the median value at 5.66 nmol/ml (range 0.22-10.86). Compared with healthy controls and the neurologic al/epileptic group, the 31 children with pontocerebellar structural de fects had significantly increased MDA values with a median value at 11 .29 nmol/ml (mean (SD) value: 11.62 (3.27), range 3.65-19.22). Implica tion - These findings of increased plasma MDA in the majority of child ren with pontocerebellar structural defects of unknown origin raised t he question whether increased lipid peroxidation leads to prenatal and postnatal pontocerebellar maldevelopment or degeneration.