SPECT Findings in Association with Interictal and Ictal Discharges in a Patient with Jeavons Syndrome

ABSTRACT

Jeavons syndrome is one of the underrecognized epileptic syndromes, characterized by eyelid myoclonia with or without absence seizures, eye closure-induced seizures, electroencephalography (EEG) paroxysms, and photosensitivity. This syndrome is considered to be among idiopathic generalized epilepsies, but the underlying pathophysiology is unknown. Recent studies using functional MRI and EEG have suggested an important role of both thalamus and occipital cortex in the fundamental pathophysiology underlying Jeavons syndrome. We described here a patient with typical Jeavons syndrome, in whom SPCET studies performed ictally and interictally revealed ictal hyperperfusion mainly confined to the both occipital and parietal cortices and ictal hypoperfusion in the diffuse frontal and temporal cortices. Our SPECT findings of ictal hyperperfusion in occipital and parietal cortices and ictal hypoperfusion in widespread cortices are, to certain degree, in line with previous EEG and fMRI studies, suggesting that the interactions between occipital and other cortical areas might be implicated in generalized spike-waves generation and a photosensitivity in Jeavons syndrome.