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a) Awake EEG showing left central spike discharges. b) Seizure onset with head version to the right and posturing of the left arm.
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One seizure was captured with head turning to right and posturing of the left arm without a clear focus of onset (Fig. 1).
Nerve conduction studies. a) Presence of the C- reflex after stimulation of the left and (b) right median nerves c) Giant SEPs.
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Short duration (20-30 ms) jerks, a rostrocaudal pattern of muscle activation, negative myoclonus and giant cortical somatosensory evoked potentials (SEPs) suggested a cortical origin of jerks, and a syndrome of progressive myoclonic epilepsy was suspected (Fig. 2).
a) and b) FLAIR sequences showing T2 hyperintense lesions in the right cerebral hemisphere. c), d) and e) Magnetic resonance angiogram sequences showing stenoses of distal internal carotid arteries (white arrow) and proximal middle cerebral arteries with collateral vessel formation (red arrow).
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Imaging of the cord was normal (Fig. 3).