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Background: Celiac disease is associated with motor cortex hyperexcitability and neurological manifestations including cortical myoclonus. Electroencephalography abnormalities have been described, but no distinct pattern has been reported., Methods: We describe the neurophysiological characteristics of 3 patients with celiac-associated cortical myoclonus using electroencephalography, magnetoencephalography, and transcranial magnetic stimulation., Results: Electroencephalography in all cases demonstrated lateralized low-amplitude, electropositive beta-frequency polyspike activity over the central head region, corresponding to motor cortex contralateral to the myoclonic limb. Jerk-locked back-averaging demonstrated a preceding cortical potential; magnetoencephalography source localization revealed a cortical generator in the posterior wall of the precentral gyrus for the back-averaged potential and oscillatory abnormality. In 1 patient, cerebellar inhibition of the motor cortex was physiologically normal., Conclusions: Central head oscillatory, low-amplitude, electropositive electroencephalography polyspike activity may be a distinct marker of celiac-related cortical myoclonus and is consistent with celiac-related motor cortex hyperexcitability, which may not necessarily result from cerebellar disinhibition. © 2020 International Parkinson and Movement Disorder Society., (© 2020 International Parkinson and Movement Disorder Society.)