[A novel homozygous mutation in PLA2G6 gene causes infantile neuroaxonal dystrophy in a case]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2016 Feb;33(1):64-7. doi: 10.3760/cma.j.issn.1003-9406.2016.01.016.
[Article in Chinese]

Abstract

Objective: To investigate the clinical symptoms and potential mutations in the PLA2G6 gene for a child with infantile neuroaxonal dystrophy.

Methods: Clinical data of the patient was collected. The coding regions of PLA2G6 gene was subjected to Sanger sequencing using blood DNA from the patient and her parents.

Results: The patient has presented with psychomotor regression and hypotonia, followed by development of tetraparesis. A novel homozygous mutation G68A in the PLA2G6 gene was found by DNA sequencing, while her parents were both heterozygous carriers.

Conclusion: The psychomotor regression and tetraparesis of the patient was caused by infantile neuroaxonal dystrophy due to a novel homozygous mutation in the PLA2G6 gene, which was inherited from her parents.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Base Sequence
  • Brain / diagnostic imaging
  • Child, Preschool
  • DNA Mutational Analysis
  • Female
  • Group VI Phospholipases A2 / genetics*
  • Homozygote
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Molecular Sequence Data
  • Mutation*
  • Neuroaxonal Dystrophies / diagnostic imaging
  • Neuroaxonal Dystrophies / genetics*
  • Radiography

Substances

  • Group VI Phospholipases A2
  • PLA2G6 protein, human