Highlight
- •Study included 29 patients (16 DM1 and 13 DM2).
- •Executive and naming dysfunction were found.
- •FDG-PET showed glucose hypometabolism in prefrontal, temporal, and pericentral regions.
- •PET findings corresponded well with the results of neuropsychological testing.
Abstract
Aim
To determine regions of reduced brain metabolism in patients with myotonic dystrophy
type 1 (DM1) and type 2 (DM2) using 18F-fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET), and to analyse their potential association
with cognitive deficit.
Method
Study included 29 patients (16 DM1 and 13 DM2). FDG-PET and detailed neuropsychological
testing were performed in both groups.
Results
The most common cognitive findings were executive, visuospatial, and naming dysfunction
in DM1, and executive and naming dysfunction in DM2. FDG-PET showed the most prominent
glucose hypometabolism in prefrontal, temporal, and pericentral regions in both DM1
and DM2 patients, with additional affection of insula and subcortical grey matter
in DM2. In DM1 patients, we found association between right frontotemporal hypometabolism
and executive dysfunction (p < 0.05). In DM2 patients attention deficit was in association with prefrontal, insular,
and striatal hypometabolism, as well as right frontotemporal hypometabolism (p < 0.05). Executive dysfunction in DM2 was more common in patients with prefrontal and
insular hypometabolism, right parietotemporal and frontotemporal hypometabolism, as
well as left striatal hypometabolism (p < 0.05). Patients with parietotemporal defect on FDG-PET were more likely to have naming
dysfunction (p < 0.01).
Conclusion
FDG-PET findings corresponded well with the results of neuropsychological testing.
FDG-PET may be a good biomarker of central nervous system involvement in DM1 and DM2,
but this hypothesis will have to be more strongly supported by larger studies.
Keywords
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Article info
Publication history
Published online: May 10, 2017
Accepted:
May 9,
2017
Received in revised form:
April 24,
2017
Received:
February 26,
2017
Identification
Copyright
© 2017 Elsevier B.V. All rights reserved.
