Normal cortical excitability in Myoclonus-Dystonia - A TMS study

S.M.A. van der Salm, A.F. van Rootselaar, E.M.J. Foncke, J.H.T.M. Koelman, L.J. Bour, K.P. Bhatia, J.C. Rothwell, M.A.J. Tijssen*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

28 Citations (Scopus)


Objective: The aim of the present study is to investigate cortical excitability in patients with DYT 11 positive Myoclonus-Dystonia (M-D), using transcranial magnetic stimulation (TMS).

Methods: Silent period, motor evoked potential (MEP) recruitment curve, short interval intracortical inhibition (SICI), intracortical facilitation (ICF) and short interval intracortical facilitation (SICF), with short interstimulus intervals (ISIs) ranging from 1.2 to 3.2 ms, were studied in 15 DYT 11-positive M-D patients and their matched controls. In four patients and matched controls peripheral double pulse electrical nerve stimulation was performed.

Results: All TMS parameters of cortical excitability were normal compared to healthy controls. In the SICF protocol we observed more variable and polyphasic MEPs in M-D patients. Cross-covariance analysis of MEP area revealed a significant correlation difference at ISI 2.2 and 2.8 ms. This increased variability was not seen in other TMS protocols or with peripheral nerve stimulation.

Conclusions: In contrast with other types of dystonia, no changes in cortical excitability were found in DYT 11 patients. Our findings suggest that M-D is both clinically and pathophysiologically a separate entity from other dystonic disorders. Polyphasic MEPs during the SICF protocol in M-D patients could reflect central neuron membrane instability. Application of the SICF protocol in other patient groups has to prove its value in movement disorders.

Original languageEnglish
Pages (from-to)300-305
Number of pages6
JournalExperimental neurology
Issue number2
Publication statusPublished - 1 Apr 2009
Externally publishedYes


  • Myoclonus-Dystonia
  • Polyphasic MEP
  • Transcranial magnetic stimulation


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