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Pitfalls in measuring cerebrospinal fluid glycine levels in infants with encephalopathy
Samah Aburahma1, Mohammad Khassawneh, May Griebel
1Jordan University of Science and Technology, Irbid, Jordan. samahk72@yahoo.com
Insights
Elevated cerebrospinal fluid glycine levels in infants can occur in various conditions, not just nonketotic hyperglycinemia. This finding highlights the need for careful diagnosis in encephalopathic infants.
Area of Science:
- Neurology
- Biochemistry
- Pediatrics
Background:
- Cerebrospinal fluid (CSF) hyperglycinemia and elevated CSF to plasma glycine ratio are typically pathognomonic for nonketotic hyperglycinemia.
- The diagnostic significance of these findings in acutely encephalopathic infants requires further evaluation.
Purpose of the Study:
- To assess the significance of CSF hyperglycinemia and elevated CSF to plasma glycine ratio in acutely encephalopathic infants.
- To determine the range of clinical conditions associated with elevated CSF glycine levels.
Main Methods:
- Retrospective chart review of infants with isolated elevated CSF glycine levels.
- Data collected from January 1995 to December 2000 at Arkansas Children's Hospital.
- Analysis of 22 patient cases, including diagnoses and glycine ratio trends.
Main Results:
- Hypoxic ischemic encephalopathy was the most common diagnosis (n=8).
- Nine patients exhibited an elevated CSF to plasma glycine ratio.
- The elevated ratio was transient in 7 of these 9 patients.
Conclusions:
- Elevated CSF to plasma glycine ratio can be observed in diverse clinical scenarios beyond nonketotic hyperglycinemia.
- The transient nature of this elevation suggests it may not always indicate a permanent metabolic disorder.
- Further research is warranted to understand glycine's role in ischemic neuronal injury and its implications in infant encephalopathy.
Abstract:
In encephalopathic infants, cerebrospinal fluid hyperglycinemia and elevated cerebrospinal fluid to plasma glycine ratio are considered pathognomonic of nonketotic hyperglycinemia. To evaluate the significance of cerebrospinal fluid hyperglycinemia and elevated cerebrospinal fluid to plasma glycine ratio in acutely encephalopathic infants, a retrospective chart review of all cases of isolated elevation of cerebrospinal fluid glycine levels at Arkansas Children's Hospital from January 1995 to December 2000 was performed. Twenty-two patients (14 males) were included. The most common diagnosis was hypoxic ischemic encephalopathy (n = 8). Nine patients had elevated cerebrospinal fluid to plasma glycine ratio, which was transient in 7 patients. This study shows that elevated cerebrospinal fluid to plasma glycine ratio can be encountered in a variety of clinical conditions. The significance of this observation in light of the poor prognosis of nonketotic hyperglycinemia and the possible role of glycine in the mechanism of ischemic neuronal injury is addressed.
