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Measurement & Analysis of the Temporal Discrimination Threshold Applied to Cervical Dystonia
10:05

Measurement & Analysis of the Temporal Discrimination Threshold Applied to Cervical Dystonia

Published on: January 27, 2018

Severity of dystonia is correlated with putaminal gray matter changes in myoclonus-dystonia

R J Beukers1, J N van der Meer, S M van der Salm

  • 1Department of Neurology, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands.

European Journal of Neurology
|January 12, 2011
PubMed
Abstract

Insights

In myoclonus-dystonia, increased gray matter volume in the putamen is linked to symptom severity. This finding highlights the putamen

Area of Science:

  • Neuroscience
  • Genetics
  • Radiology

Background:

  • Myoclonus-dystonia (M-D) is an inherited movement disorder with variable findings in previous brain structure studies.
  • Sensorimotor structures, particularly the basal ganglia, are implicated in dystonia pathophysiology.

Purpose of the Study:

  • To investigate gray matter (GM) volumes in sensorimotor brain regions in genetically homogeneous M-D using MRI.
  • To correlate GM volumes with clinical severity scores in M-D patients.

Main Methods:

  • Magnetic resonance imaging (MRI) was used to acquire brain scans from 25 M-D patients (DYT11 mutation carriers) and 25 controls.
  • Gray matter volumes were quantified in sensorimotor cortices and basal ganglia.
  • Clinical severity was assessed using the Burke-Fahn Marsden dystonia rating scale (BFMDRS) and unified myoclonus rating scale (UMRS).

Main Results:

  • No significant group differences in GM volume were observed between M-D patients and controls.
  • Dystonia severity in M-D patients strongly correlated with increased GM volume in the bilateral putamina.

Conclusions:

  • The putamen plays a crucial role in the pathophysiology of myoclonus-dystonia.
  • Putaminal volume changes are associated with the clinical severity of dystonia.

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