Learn More
Encephalopathy with electrical status epilepticus during sleep or ESES is an age-dependent and self-limited syndrome whose distinctive features include a characteristic age of onset (with a peak around 4-5 years), heterogeneous seizures types (mostly partial motor or unilateral seizures during sleep and absences or falls while awake), a typical EEG pattern(More)
OBJECTIVES Little is known about the long term outcome of patients with periventricular nodular heterotopia (PNH) and epilepsy, particularly the course of seizures. This study investigated the electroclinical and prognostic features of 16 patients with PNH. METHODS Of 120 patients with epilepsy and malformations of cortical development, 16 had PNH. Of(More)
Few studies have focused on the clinical, neurophysiological and prognostic features of adult epileptic patients with malformation of cortical development. We reviewed the clinical data of a series of sixty adult epileptic patients with different types of malformation of cortical development, who had been followed at the Epilepsy Centre of the Department of(More)
PURPOSE To analyse the video-polygraphic features of two patients with adult epileptic spasms (ES). METHODS Two patients with epileptic encephalopathy related to different cortical malformation; epileptic spasms had been present since the age of 18 and 25 years respectively. The patients underwent a clinical and neurophysiological examination (awake and(More)
The purpose of our study was to describe the clinical characteristics of sporadic (S) cases of partial epilepsy with auditory features (PEAF) and pinpoint clinical, prognostic and genetic differences with respect to previously reported familial (F) cases of autosomal dominant partial epilepsy with auditory features (ADPEAF). We analysed 53 patients (24(More)
PURPOSE To investigate the neurophysiological features of photic reflex myoclonus (PRM) in patients with progressive myoclonus epilepsies (PMEs) of different types (Unverricht-Lundborg disease, Lafora's disease, cryptogenic). METHODS All patients underwent computerized video-polygraphic recordings, collecting electromyographic (EMG) activity from several(More)
Myoclonic jerks and myoclonic status (MS) are sometimes difficult to distinguish clinically from movement disorders such as hand stereotypies, tremor, and dystonia in Rett syndrome. We describe a rare and complete video-polygraphic study of a girl with Rett syndrome (MECP2 mutation) and MS misdiagnosed as movement disorders and disclosed after(More)
PURPOSE Some consider the "voices" of Joan of Arc to have been ecstatic epileptic auras, such as Dostoevsky's epilepsy. We performed a critical analysis of this hypothesis and suggest that the "voices" may be the expression of an epileptic syndrome recently described: idiopathic partial epilepsy with auditory features (IPEAF). METHODS Joan's symptoms were(More)
OBJECTIVES AND METHODS To perform a video-polygraphic analysis of 11 cataplectic attacks in a 39-year-old narcoleptic patient, correlating clinical manifestations with polygraphic findings. Polygraphic recordings monitored EEG, EMG activity from several cranial, trunk, upper and lower limbs muscles, eye movements, EKG, thoracic respiration. RESULTS Eleven(More)
Epileptic seizures, movement disorders and breathing disturbances may be observed in Rett syndrome, and correct diagnosis is mandatory for the management. We evaluated the usefulness of video-polygraphy in the differential diagnosis between epileptic and non-epileptic paroxysmal events in eight patients with Rett syndrome. Based on video analysis, myoclonic(More)