ABSTRACT
Objective To explore the clinical manifestations, electroencephalographic characteristics and therapeutic effect of drugs in children with Jeavons syndrome. Methods The clinical and electroencephalographic characteristics and thera-peutic effect of drugs were analyzed in 4 children with Jeavons syndrome. Results Among the four children there were 3 female and 1 male. The age at the onset of the disease was from 1 to 6 years. The typical clinical manifestations of this disease were brief, fast and repeated eyelid myoclonia (EM) with or without absence seizure. The typical electroencephalography (EEG) in two patients showed 3-6 Hz generalized spike and waves and polyspikes burst, and the eye closure and intermittent photic stimu-lation helped to induce discharges and clinical events. The typictal EEG in the other two patients showed 3.0-3.5 Hz generalizedδslow wave rhythm burst. The drugs of choice for treatment was sodium valproate monotherapy in two cases, levetiracetam in one case, sodium valproate combined with levetiracetam in one case. During the follow-up, seizures were controlled in one case, decreased in frequency in two cases and were still frequent in one case. Conclusions Jeavons syndrome is one of the idiopathic and generalized epileptic syndromes and characterized by EM with or without absence seizure. Video EEG monitoring plays an important role in the diagnosis of this disease. Sodium valproate and levetiracetam were effective for this disease.
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.
Subject(s)
Humans , Electroencephalography , Epilepsy, Absence , Epilepsy, Generalized , Eye , Eyelids , Magnetic Resonance Imaging , Seizures , Thalamus , Tomography, Emission-Computed, Single-PhotonABSTRACT
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.