Choudhury, TarakKundu,SoumiDas, G.C.Dasgupta, Malay Kumar2026-02-232026-02-232016https://hdl.handle.net/20.500.14572/4876Tarak Choudhury RG Kar Medical College, Kolkata, West Bengal Soumi Kundu RG Kar Medical College, Kolkata, West Bengal G.C. Das RG Kar Medical College, Kolkata, West Bengal Malay Kumar Dasgupta RG Kar Medical College, Kolkata, West BengalAbstract: In encephalopathic infants, cerebrospinal fluid hyperglycinemia and elevated cerebrospinal fluid to plasma glycine ratio are considered pathognomonic of Non-ketotic hyperglycinemia (NKH). It is due to a defect of the glycine cleavage system and has poor prognosis. We present a case of transient NKH presented to us with hypotonia, recurrent apnea and seizure. Increased ratio of cerebrospinal fluid to plasma glycine concentrations of 0.16 was seen as a strong diagnostic indicator of Non-ketotic hyperglycinemia.en-USNKHapneaseizureshypotoniaglycineCSFplasmaArticle