PMC7674959_01
Spontaneous EEG recording of Case No. 1 patient (8-years old male Beagle): Polyspike-slow wave complexes of varying amplitude are seen on all derivations, accompanied by sudden myoclonic contraction of the neck and forelimbs. Low-cut filter: 0.5 Hz; high-cut filter: 50 Hz; speed 10 sec/page; channel C: ECG, bipolar on the chest; channel B: empty; channel D: bipolar, right brachiocephalic muscle. Figure shown at 20 muV/mm. EEG, electroencephalogram; ECG, electrocardiography.
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F1
Spontaneous EEG recording revealed solitary spikes (50-250 muV), solitary spike-slow wave complexes, and generalized polyspike-slow wave complexes every 20-40 s (Figure 1).
IPS-induced EEG recording of Case No. 1 patient (8-year old male Beagle): IPS at 13 Hz generates polyspikes-slow wave complexes visible on all derivations, accompanied by sudden generalized myoclonic contractions. Low-cut filter: 0.5 Hz; high-cut filter: 50 Hz; speed 5 s/page; channel C: ECG, bipolar on the chest; channel B: empty; channel D: bipolar, right brachiocephalic muscle; MKT channel: photic stimulation marker. Figure shown at 20 muV/mm. IPS, intermittent photic stimulation; EEG, electroencephalogram; ECG, electrocardiography.
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F2
PMC7674959_02
Spontaneous EEG recording of Case No. 2 patient (7-year old male Beagle): In the fifth second, a generalized spike, visible on all derivations, is followed by slow waves. A muscle artifact can be seen after the discharge on T3. Low-cut filter: 0.5 Hz; high-cut filter: 50 Hz; speed 10 sec/page; channel C: ECG: bipolar on the chest, channel B: bipolar, left brachiocephalic muscle; channel D: bipolar, right brachioradialis muscle. EEG, electroencephalogram; ECG, electrocardiogram.
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F3
Left temporal electrode T3 shows a movement artifact after the discharge (Figure 3).
IPS-induced EEG recording of Case No. 2 patient: IPS at 11 Hz generates after 120 ms generalized polyspike-slow wave complexes, followed immediately by the myoclonic contraction on the EMG channel. Low-cut filter: 0.5 Hz; high-cut filter: 50 Hz, speed 5 sec/page; channel C: ECG, bipolar on the chest, channel B: bipolar, left brachiocephalic muscle; channel D: bipolar, right brachioradialis muscle; MKT channel: photic stimulation marker. Figure shown at 20 muV/mm. IPS, intermittent photic stimulation; EEG, electroencephalogram; ECG, electrocardiography; EMG, electromyography.
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F4
After 250-600 ms, IPS was followed by cortical polyspikes on EEG, which later on, after 100-150 ms, were correlated with the contraction of the thoracic limb (Figure 4). The left occipital electrode O1 showed a movement artifact after the discharge (Figure 4).
Transverse section of muscle showing many PAS-positive intracytoplasmic inclusions (arrows), occasionally forming subsarcolemmal agglomerations. PAS stain, image (A)-ob x 40 (scale bar = 75 mum), image (B)-ob x 100 (scale bar = 30 mum). PAS, periodic acid-Schiff.
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F5
Inclusions were absent within the sweat glands (Figure 5).