Neonatal glycine encephalopathy: biochemical and neuropathologic findings.

Title

Neonatal glycine encephalopathy: biochemical and neuropathologic findings.

Creator

Agamanolis D P; Potter J L; Lundgren D W

Publisher

Pediatric neurology

Date

1993
1993-04

Description

A patient with neonatal glycine encephalopathy who had severe neurologic retardation, spasticity, and seizures died at 17 years of age. Glycine concentration was markedly elevated in brain tissue, especially in the cerebellum. Neuropathologic study revealed spongy myelinopathy throughout the central nervous system and calcium oxalate crystals in the cerebellum, which are probably derived from degradation of glycine. Myelinopathy appeared to be static compared to neonatal patients. The neurologic manifestations of neonatal glycine encephalopathy are probably due to neurotransmitter abnormalities, not to myelin damage.

Subject

Adolescent; Amino Acid Metabolism; Amino Acids/blood; Brain Diseases; Brain/pathology; Calcium Oxalate/blood; Child; Crystallization; Follow-Up Studies; Glycine/*blood; Humans; Inborn Errors/genetics/*pathology; Infant; Male; Metabolic/genetics/*pathology; Myelin Sheath/*pathology; Newborn; Preschool; Spinal Cord/pathology

Rights

Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).

Pages

140–143

Issue

2

Volume

9

Citation

Agamanolis D P; Potter J L; Lundgren D W, “Neonatal glycine encephalopathy: biochemical and neuropathologic findings.,” NEOMED Bibliography Database, accessed December 2, 2021, https://neomed.omeka.net/items/show/3390.

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