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Because few families with congenital <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content>s and no other neurological signs have been reported, the underlying mechanisms of <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content>s are still not entirely clear. We report on the clinical, genetic, neurophysiological and neuroimaging findings of 10 of 26 living members of a novel four\u2010generation family with congenital <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content>s. <jats:italic><jats:styled-content style=\"fixed-case\">DCC<\/jats:styled-content><\/jats:italic> and <jats:italic><jats:styled-content style=\"fixed-case\">RAD<\/jats:styled-content>51<\/jats:italic> were sequenced in affected members of the family. Five of the ten subjects with <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content>s underwent neurophysiological and neuroimaging evaluations. The neurophysiological evaluation consisted of electromyographic (<jats:styled-content style=\"fixed-case\">EMG)<\/jats:styled-content> mirror recordings, investigations of corticospinal excitability, and analysis of interhemispheric inhibition using transcranial magnetic stimulation techniques. The neuroimaging evaluation included functional <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> during finger movements. Eight (all females) of the ten members examined presented <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content>s of varying degrees at the clinical assessment. Transmission of <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content>s appears to have occurred according to an autosomal\u2010dominant fashion with variable expression. No mutation in <jats:italic><jats:styled-content style=\"fixed-case\">DCC<\/jats:styled-content><\/jats:italic> or <jats:italic><jats:styled-content style=\"fixed-case\">RAD<\/jats:styled-content>51<\/jats:italic> was identified. <jats:styled-content style=\"fixed-case\">EMG<\/jats:styled-content> mirror activity was higher in <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content> subjects than in healthy controls. Short\u2010latency interhemispheric inhibition was reduced in <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content> subjects. Ipsilateral motor\u2010evoked potentials were detectable in the most severe case. The neuroimaging evaluation did not disclose any significant abnormalities in <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content> subjects. The variability of the clinical features of this family, and the lack of known genetic abnormalities, suggests that <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content>s are heterogeneous disorders. The pathophysiological mechanisms of <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content>s include abnormalities of transcallosal inhibition and corticospinal decussation.<\/jats:p>","DOI":"10.1002\/mdc3.12066","type":"journal-article","created":{"date-parts":[[2014,7,28]],"date-time":"2014-07-28T06:40:59Z","timestamp":1406529659000},"page":"180-187","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Congenital Mirror Movements in a New Italian Family"],"prefix":"10.1002","volume":"1","author":[{"given":"Alfonso","family":"Fasano","sequence":"first","affiliation":[{"name":"Movement Disorders Center TWH, UHN, Division of Neurology University of Toronto Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matteo","family":"Bologna","sequence":"additional","affiliation":[{"name":"Neuromed Institute IRCCS Pozzilli (IS) 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