Ketogenic diet in early myoclonic encephalopathy due to non ketotic hyperglycinemia.

@article{Cusmai2012KetogenicDI,
  title={Ketogenic diet in early myoclonic encephalopathy due to non ketotic hyperglycinemia.},
  author={R Cusmai and Diego Martinelli and Romina Moavero and Carlo Dionisi Vici and Federico Vigevano and Cinzia Castana and Mirella Elia and Silvia M Bernabei and Elsa Bevivino},
  journal={European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society},
  year={2012},
  volume={16 5},
  pages={509-13}
}
Non ketotic hyperglycinemia is a rare inborn error of glycine metabolism due to deficient activity of glycine cleavage system, a multienzymatic complex consisting of four protein subunits: the P-protein, the H-protein, the T-protein and the L-protein. The neonatal form of non ketotic hyperglycinemia presents in the first days of life with encephalopathy, seizures, multifocal myoclonus and characteristic "hiccups". Rapid progression may lead to intractable seizures, coma and respiratory failure… CONTINUE READING