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Autosomal dominant cortical tremor, myoclonus and epilepsy Volume 18, supplement 2, September 2016

Figure 1

Electroclinical and MRI features of familial cortical tremor, myoclonus and epilepsy. (A) free-hand drawing (Archimedes’ spiral and ladder) showing the differences between essential (left) and cortical (right) tremors. The cortical tremor is fairly irregular, and sudden, brisk jerks cause disruption to the drawing. (B) EEG of a patient during photic stimulation with eyes closed, showing the photomyoclonic response consisting of increasing, mainly anteriorly, myogenic potentials related to each flash stimuli. (C) EMG recording of bursts between agonist and antagonist muscles (EMG1: right wrist extensor; EMG2: right wrist flexor) with extended arms; irregular, high-frequency, short EMG bursts without the regular alternating pattern typically found in tremors. (D) Jerk-locked averaging analysis shows a positive-negative potential, recognizable over the left centroparietal electrodes, preceding myoclonus by about 30 ms (right wrist extensor muscle; number of triggers = 100).

(E) 1H-MR Spectroscopy using a PRESS sequence (TR 1,500 ms; TE 144 ms) showing abnormal spectral peak areas at 3.22 ppm, corresponding to choline (location of the 8 cm3 voxel: right cerebellar hemisphere). Reproduced from Striano and Zara (2010), with permission.

Figure 2

Free-hand drawing, Archimede's spiral hands (left) and basal EEG of patient C/3 obtained at the age of 59 (A), 70 (B), and 80 years (C), showing worsening of myoclonus and progressive slowing of EEG background activity.

Reproduced from Coppola et al. (2011), with permission.