Functional MRI study of response inhibition in Myoclonus Dystonia.

Does myoclonus-dystonia results from a dysfunctional basal ganglia network causing insufficient inhibitory motor control? To test this hypothesis functional MRI (fMRI) was performed using a validated "Go/No go" task, in order to localize Blood-oxygen-level dependence (BOLD) effects corresponding to Response Inhibition (RI). The study demonstrated a distinct association of motor symptoms in MD with the ventral lateral nucleus of the thalamus. Cerebellar dysfunction distinguishes DYT11 positive from negative patients. We suggest that MD might be best considered as a disorder of the cortico-ponto-cerebello-thalamo-cortical system.