Epileptic spasms were defined by the International League Against Epilepsy Task Force on Classification and Terminology in 2001 as a specific seizure type. Epileptic spasms without hypsarrhythmia ...have been described in some series of patients, occurring either in infancy or childhood. More prolonged epileptic spasms without hypsarrhythmia were previously defined as a different seizure type, and referred to as “tonic spasm seizures”. Here, we present a 5‐year‐old boy who started having epileptic spasms without hypsarrhythmia at 8 months of age, effectively treated with oxcarbazepine. With the withdrawal of medication, epileptic spasms returned. Video‐EEG monitoring revealed high‐voltage slow waves superimposed by low‐voltage fast activity, followed by an electrodecremental phase and a burst of asymmetric fast activity, time‐locked to clinical tonic spasm seizures. Brain MRI showed left temporal atrophy with temporal pole grey/white matter junction blurring and ictal PET‐CT showed left basal frontal hypermetabolism. Seizures were refractory to several AEDs and vigabatrin was introduced with seizure cessation. Despite efforts to classify epileptic spasms, these are still considered as part of the group of unknown seizure types. In some cases, a focal origin has been suggested, leading to the term “periodic spasms” and “focal spasms”. In this case, epileptic spasms without hypsarrhythmia, associated with tonic spasms, may be a variant of focal spasms and might be considered as an epileptic syndrome. Published with video sequence
Evidence suggests increased prevalence of cluster B personality disorders (PD) among patients with juvenile myoclonic epilepsy (JME), which has been associated with worse seizure control and more ...psychosocial dysfunctions. A preliminary voxel-based morphometry study demonstrated corpus callosum (CC) volume reduction in patients with JME and cluster B PD, particularly in the posterior midbody and isthmus. In this study we aimed to follow up these results with region of interest analysis. Sixteen JME patients with cluster B PD, 38 JME patients without any psychiatric disorder, and 30 demographically matched healthy controls submitted to a psychiatric evaluation and a magnetic resonance imaging scan. The total and regional callosal areas were obtained from the midsagittal slice using a semi-automated program. Psychiatric evaluation was performed through SCID-I and -II. Significant reductions in the posterior region of the CC were observed in the JME with PD group relative to the other groups. These data support previous findings of callosal reductions in cluster B PD, as well as a possible involvement of CC in patients with JME and such personality characteristics.
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