ABSTRACT
Children with glycogen storage disease [GSD] suffer from hypoglycemia that is life long and needs frequent feedings and nocturnal intragastric infusions of solutions containing glucose or cornstarch, a regimen that is not possible for most of our followed up patients. The objective of this study was the evaluation of permanent EEG changes in children suffering from liver glycogenoses with attacks of hypoglycemia, hypoglycemic convulsions and those with euglycemia. In a cross-sectional study, 25 children suffering from liver GSD [with no current neurological symptoms] and 20 age and sex matched clinically free children [control group] underwent EEG studies. The results showed that 11 [44%] of the studied children were found to have nocturnal convulsions. Abnormal EEG studies were found in 79 children [76%]. One had a left temporal epileptic focus, 1 had increased slow activity, 7 had abnormal background symmetry and 16 [64%] had absent alpha rhythm in alert state EEG recording. The focal findings did not prove to correlate to hypoglycemia but rather to the level of direct bilirubin above 0.3mg% and total bilirubin above 1mg% [P=0.018%]. Colchicine intake and its prolonged use [>36 months] were associated with abnormal discharge [P=0.004 and respectively P=0.008].The absence of alpha rhythm was related to positive consanguinity [P=0.003] and was detected in children with other affected sib [P=0.000]
Conclusion: liver glycogenosis are associated with EEG changes. These changes were found to be attributable to familial, genetic factors and factors affecting the liver capacity to maintain direct bilirubin within a tight normal range of normal population