Journal of Neurology

, Volume 255, Issue 12, pp 1904–1909 | Cite as

Grey and white matter loss along cerebral midline structures in myotonic dystrophy type 2

  • Martina MinneropEmail author
  • Eileen Luders
  • Karsten Specht
  • Jürgen Ruhlmann
  • Christiane Schneider-Gold
  • Rolf Schröder
  • Paul M. Thompson
  • Arthur W. Toga
  • Thomas Klockgether
  • Cornelia Kornblum


Myotonic dystrophy type 2 (DM2) is an autosomal dominantly inherited multisystemic disorder and a common cause of muscular dystrophy in adults. Although neuromuscular symptoms predominate, there is clinical and imaging evidence of cerebral involvement. We used voxel-based morphometry (VBM) based on T1-weighted magnetic resonance images to investigate brain morphology in 13 DM2 patients in comparison to 13 sex- and age-matched controls. Further, we employed novel computational surface-based methods that specifically assess callosal thickness. We found grey and white matter loss along cerebral midline structures in our patient group. Grey matter reductions were present in brainstem and adjacent hypothalamic and thalamic regions, while white matter was mainly reduced in corpus callosum. The reduced callosal size was highly significant and independently confirmed by different methods. Our data provide first evidence for grey and white matter loss along brain midline structures in DM2 patients. The reduced size of the corpus callosum further extends the spectrum of white matter changes in DM2 and may represent the morphological substrate of neuropsychological abnormalities previously described in this disorder.

Key words

DM2 brainstem corpus callosum VBM morphometry 


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Copyright information

© Steinkopff-Verlag 2008

Authors and Affiliations

  • Martina Minnerop
    • 1
    • 2
    Email author
  • Eileen Luders
    • 3
  • Karsten Specht
    • 4
  • Jürgen Ruhlmann
    • 5
  • Christiane Schneider-Gold
    • 6
  • Rolf Schröder
    • 7
  • Paul M. Thompson
    • 3
  • Arthur W. Toga
    • 3
  • Thomas Klockgether
    • 1
  • Cornelia Kornblum
    • 1
  1. 1.Dept. of NeurologyUniversity Hospital of BonnBonnGermany
  2. 2.Brain Imaging Center West (BICW)JülichGermany
  3. 3.Laboratory of NeuroImaging, Dept. of NeurologyUCLA School of MedicineLos AngelesUSA
  4. 4.Dept. of Biological and Medical PsychologyUniversity of Bergen & Dept. of Medical Engineering, Haukeland University HospitalBergenNorway
  5. 5.Medical Center BonnBonnGermany
  6. 6.Dept. of NeurologyUniversity of GöttingenGöttingenGermany
  7. 7.Dept. of NeuropathologyUniversity of ErlangenErlangenGermany

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