PMID- 19118553 OWN - NLM STAT- MEDLINE DCOM- 20090415 LR - 20090320 IS - 1090-2430 (Electronic) IS - 0014-4886 (Linking) VI - 216 IP - 2 DP - 2009 Apr TI - Normal cortical excitability in Myoclonus-Dystonia--a TMS study. PG - 300-5 LID - 10.1016/j.expneurol.2008.12.001 [doi] AB - 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. FAU - van der Salm, S M A AU - van der Salm SM AD - Department of Neurology and Clinical Neurophysiology of the Academic Medical Center, University of Amsterdam, The Netherlands. FAU - van Rootselaar, A F AU - van Rootselaar AF FAU - Foncke, E M J AU - Foncke EM FAU - Koelman, J H T M AU - Koelman JH FAU - Bour, L J AU - Bour LJ FAU - Bhatia, K P AU - Bhatia KP FAU - Rothwell, J C AU - Rothwell JC FAU - Tijssen, M A J AU - Tijssen MA LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20081211 PL - United States TA - Exp Neurol JT - Experimental neurology JID - 0370712 SB - IM MH - Adult MH - Case-Control Studies MH - Cerebral Cortex/*physiopathology MH - Dystonic Disorders/complications/*physiopathology MH - Electric Stimulation/methods MH - Electromyography MH - Evoked Potentials, Motor/*physiology MH - Female MH - Functional Laterality MH - Humans MH - Male MH - Middle Aged MH - Myoclonus/complications/*physiopathology MH - Neural Inhibition/physiology MH - Peripheral Nerves/physiopathology MH - Reaction Time MH - Statistics, Nonparametric MH - *Transcranial Magnetic Stimulation MH - Young Adult EDAT- 2009/01/03 09:00 MHDA- 2009/04/16 09:00 CRDT- 2009/01/03 09:00 PHST- 2008/07/13 00:00 [received] PHST- 2008/10/28 00:00 [revised] PHST- 2008/12/03 00:00 [accepted] PHST- 2009/01/03 09:00 [entrez] PHST- 2009/01/03 09:00 [pubmed] PHST- 2009/04/16 09:00 [medline] AID - S0014-4886(08)00470-6 [pii] AID - 10.1016/j.expneurol.2008.12.001 [doi] PST - ppublish SO - Exp Neurol. 2009 Apr;216(2):300-5. doi: 10.1016/j.expneurol.2008.12.001. Epub 2008 Dec 11.