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G172 MILD SYSTEMIC HYPOTHERMIA IN THE TREATMENT OF NEONATAL SEIZURES IN HYPERAMMONAEMIC ENCEPHALOPATHY MONITORED BY CEREBRAL FUNCTION MONITOR

D. Kenny, A. Jain, P. Davis, M. Thoresen. Department of Clinical Science, South Bristol (Child Health)

Seizures may occur secondary to neurotoxicity associated with hyperammonaemic encephalopathy and studies suggest that seizures further damage a compromised brain. Glutamine has been implicated and hypothermia has been shown to reduce concentrations of glutamine in the post-hypoxic cerebral cortex of neonatal animals. Hypothermia has also been shown to reduce the duration of post-hypoxic seizures in encephalopathic infants.1 We describe an infant who presented aged 50 h with hyperammonaemic encephalopathy that was found to be a result of a urea cycle defect. Cerebral function monitoring (CFM), which is a single channel amplitude integrated EEG, displayed seizure activity unresponsive to the common anticonvulsants. Haemodialysis and alternative pathway metabolites were commenced and the patient was paralysed. Cooling has been shown to hasten the reduction of ammonia,2 hence the infant was simultaneously cooled to a rectal temperature of 34°C while dialysed. The seizure activity subsided despite a rapid reduction to sub therapeutic anticonvulsant drug levels, most likely due to increased drug removal during haemodialysis. After a seizure free period at normothermia and drug levels within the therapeutic range, ammonia levels rose to ~500 and non-convulsive seizures were noted on the CFM trace as well as EEG. Rapid cooling for the 12 h of haemofiltration stopped seizure activity while the ammonia levels changed little this time. As soon as rewarming commenced non-convulsive seizures reappeared on CFM despite therapeutic levels of anticonvulsants. In conclusion, continuous CFM is invaluable in monitoring seizure activity where paralysis may mask clinical seizures and other interventions such as haemodialysis lead to rapid changes in anticonvulsant levels. Mild hypothermia has anticonvulsant effect in itself and may also be neuroprotective in hyperammonaemic encephalopathy.

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G173 PROSPECTIVE DIETARY THERAPY IN A PATIENT WITH MOLYBDENUM COFACTOR DEFICIENCY

M. A. Kurian1, T. Randall2, P. …

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