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Neonatology 2010; 98: 84–90. Diagnostic tools of metabolic and structural brain disturbances in neonatal non-ketotic hyperglycinemia Demet Terek, 1 Ozge Altun Koroglu, 1 Sezgin Gunes, 1 Mehmet Yalaz, 1 Mete Akisu, 1 Sema Kalkan Uçar, 2 Sarenur Gokben, 3 Mahmut Çoker 2 and Nilgün Kultursay 1 Divisions of 1 Neonatology, 2 Metabolism and Nutrition and 3 Neurology, Department of Pediatrics, Ege University Faculty of Medicine, Bornova, Izmir, Turkey Abstract Non-ketotic hyperglycinemia (NKH) is a rare autosomal recessive disorder of glycine metabolism. We report a newborn case of NKH and discuss the effects of this rare disease on brain metabolism and structure together with amplitude- integrated electroencephalography, cranial magnetic resonance and magnetic resonance spectroscopy findings which are very rarely reported together so far. Key words amplitude integrated electroencephalography, glycin encephalopathy, magnetic resonance spectroscopy, metabolic disorder, newborn. Non-ketotic hyperglycinemia (NKH) is a rare autosomal reces- sive disorder affecting glycine metabolism with a reported preva- lence of 1:60.000–100.000. The biochemical defect is in the glycine cleavage system which consists of a mitochondrial enzyme complex. There are four forms of glycine encephalopa- thy: neonatal; infantile; transient; and late. 1 The enzyme level is close to zero in the neonatal form which is the most common and severe form. The clinical manifestations of poor sucking, hypo- tonicity, lethargy, hiccups and seizures develop within 6 h to 8 days of birth of an otherwise healthy newborn. Acute neurologi- cal deterioration rapidly progresses to coma and often leads to death in a short period. If patients survive the ventilator- dependent period, they will suffer from severe mental and devel- opmental retardation and seizures. We report a case of newborn NKH and discuss the effects of this rare disease on brain metabolism and structure, we also look at using amplitude-integrated electroencephalography (aEEG), cranial magnetic resonance imaging (MRI) and magnetic reso- nance (MR) spectroscopy findings, which have been very rarely reported together previously. Case report A female infant was born at term (39 weeks gestation) with a birth weight of 3200 g, to a gravida 3 para 0 mother, (second- degree consanguineous parents had an unremarkable family history) after a pregnancy complicated with chronic hyperten- sion. The infants Apgar scores were 7 and 9 at 5 and 10 min, respectively. On day 2, the infant was admitted to the neonatal intensive care unit because of decreased movements, shallow breathing and poor sucking. The physical findings were hypotonicity, leth- argy, decreased newborn reflexes and pes equinovarus. The patient was intubated and mechanical ventilation was initiated because of respiratory acidosis. Routine biochemical and hematological tests including blood glucose and complete blood count were unremarkable. Since neonatal sepsis could not be ruled out, ampicillin and netilmicin antibiotherapy was initiated. Blood, cerebrospinal fluid and urine cultures were negative. On her 4 th day of life the patient began to have persistent hiccups and myoclonic seizures unresponsive to treatment. A continuous burst suppression pattern indicating severe–moderate brain damage was observed on aEEG (Fig. 1). Metabolic disorders were investigated because of parental con- sanguinity, and clinical and aEEG findings. Blood lactate, pyru- vate and ammonia levels were normal. Initially, conventional electroencephalography (EEG) showed low-voltage baseline rhythm. However, a later EEG performed after the onset of Correspondence: Demet Terek, MD, Ege University Faculty of Medi- cine, Children’s Hospital, Bornova 35100, Izmir, Turkey. Email: demet.terek@yahoo.com Received 14 June 2011; revised 21 December 2011; accepted 26 January 2012. doi: 10.1111/j.1442-200X.2012.03591.x Refeeding syndrome in a SGA micro-preemie 717 © 2012 The Authors Pediatrics International © 2012 Japan Pediatric Society