Glutaminase deficiency is characterized by refractory seizures, respiratory failure, brain abnormalities and death in the neonatal period, though milder cases with spastic ataxia-dysarthria have also been reported. This condition is caused by mutations in the glutaminase (GLS) gene. [ http://www.ncbi.nlm.nih.gov/pubmed/30575854 http://www.ncbi.nlm.nih.gov/pubmed/30970188 ]