A rare case of glycine encephalopathy unveiled by valproate therapy

Velusamy Subramanian; Pramila Kadiyala; Praveen Hariharan; E. Neeraj

doi:10.4103/1817-1745.159200

A rare case of glycine encephalopathy unveiled by valproate therapy

Velusamy Subramanian, Pramila Kadiyala, Praveen Hariharan, E. Neeraj

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6 Scopus citations

Abstract

Glycine encephalopathy (GE) or nonketotic hyperglycinemia is an autosomal recessive disorder due to a primary defect in glycine cleavage enzyme system. It is characterized by elevated levels of glycine in plasma and cerebrospinal fluid usually presenting with seizures, hypotonia, and developmental delay. In our case, paradoxical increase in seizure frequency on starting sodium valproate led us to diagnose GE.

Original language	English (US)
Pages (from-to)	143-145
Number of pages	3
Journal	Journal of Pediatric Neurosciences
Volume	10
Issue number	2
DOIs	https://doi.org/10.4103/1817-1745.159200
State	Published - Apr 1 2015
Externally published	Yes

Keywords

Glycine encephalopathy
nonketotic hyperglycinemia
organic acidemia
recurrent seizures
valproate

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10.4103/1817-1745.159200

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AU - Kadiyala, Pramila

AU - Hariharan, Praveen

AU - Neeraj, E.

PY - 2015/4/1

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AB - Glycine encephalopathy (GE) or nonketotic hyperglycinemia is an autosomal recessive disorder due to a primary defect in glycine cleavage enzyme system. It is characterized by elevated levels of glycine in plasma and cerebrospinal fluid usually presenting with seizures, hypotonia, and developmental delay. In our case, paradoxical increase in seizure frequency on starting sodium valproate led us to diagnose GE.

KW - Glycine encephalopathy

KW - nonketotic hyperglycinemia

KW - organic acidemia

KW - recurrent seizures

KW - valproate

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ER -

A rare case of glycine encephalopathy unveiled by valproate therapy

Abstract

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