MRS in Early and Presymptomatic Carriers of a Novel Octapeptide Repeat Insertion in the Prion Protein Gene

Research output: Contribution to journalArticle

Abstract

To evaluate the proton magnetic resonance (MR) spectroscopy (1H MRS) changes in carriers of a novel octapeptide repeat insertion in the prion protein gene (PRNP) and family history of frontotemporal dementia with ataxia. Four at-risk mutation carriers and 13 controls were compared using single voxel, short TE, 1H MRS from the posterior cingulate gyrus. The mutation carriers had an increased choline/creatine, P= .003 and increased myoinositol/creatine ratio, P= .003. 1H MRS identified differences in markers of glial activity and choline metabolism in pre- and early-symptomatic carriers of a novel PRNP gene octapeptide insertion. These findings expand the possible diagnostic utility of 1H MRS in familial prion disorders.

Original languageEnglish (US)
Pages (from-to)409-413
Number of pages5
JournalJournal of Neuroimaging
Volume23
Issue number3
DOIs
StatePublished - Jul 1 2013

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Keywords

  • Familial prion disorders
  • Frontotemporal dementia
  • MRI
  • MRS

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology

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