Objective: To describe a novel motor cortex (M1) electroencephalogram (EEG) abnormality distinct to celiac disease (CD)-related cortical myoclonus and to explore its relationship with cerebellar-M1 connectivity.

Background: CD is associated with cortical hyperexcitability [1] and a number of neurological manifestations including cortical myoclonus, with or without ataxia [2]. The mechanism underlying cortical hyperexcitability in CD is unknown. A variety of non-specific EEG abnormalities have been described but no distinct CD-related EEG pattern has been reported [3].

Method: We describe the clinical and neurophysiological characteristics of 3 cases of CD-associated cortical myoclonus including EEG, magnetoencephalography (MEG), and transcranial magnetic stimulation (TMS).

Results: EEG in all cases demonstrated low amplitude (20-40 µV), electropositive fast beta frequency (20-40 Hz) oscillatory polyspike and polyspike and slow wave activity over the central head region, corresponding to M1 contralateral to the myoclonic limb [Figure 1]. Jerk-locked back-averaging demonstrated a preceding EEG-MEG cortical potential; MEG source localization (dipole mapping and beamformer analysis) revealed an identical cortical generator situated in the posterior wall of the precentral gyrus for both the back-averaged potential and the electropositive phase of the spontaneous oscillatory abnormality. In one patient, cerebellar inhibition of M1 was physiologically normal and did not change after cerebellar intermittent theta burst stimulation. Transection of the localized cortex in one patient resulted in cessation of myoclonus.

Conclusion: The finding of oscillatory, low amplitude, electropositive high frequency EEG polyspike activity over the central head region corresponding to M1 may be pathognomonic of CD-related cortical myoclonus and its presence may serve as an important diagnostic clue for this condition. These oscillatory rhythms are consistent with prior descriptions of CD-related cortical hyperexcitability, which may not necessarily result from direct cerebellar disinhibition of the contralateral M1.

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